Vascular Tumors And Malformations - Vascular Injury Hand & Lower Extremities Flashcards

Question

An 18-year-old man comes to the office for follow-up examination because he has a four-month history of tripping with numbness and tingling of the right leg and foot. He underwent arthroscopic anterior cruciate ligament (ACL) grafting five months ago after he dislocated the right knee and ruptured the ACL during a football game. Physical examination shows weakness of dorsiflexion of the foot. Decreased sensibility to light touch over the lateral aspect of the right leg is noted. Which of the following is the most likely cause of his symptoms? A) Compartment syndrome B) Compression of the common peroneal nerve C) Laceration of the deep peroneal nerve D) Laceration of the superficial peroneal nerve E) Neurapraxia of the tibial nerve

Answer 1

Correct answer is option B. Compression of the common peroneal nerve is the third most common nerve compression syndrome (carpal tunnel syndrome is first and cubital tunnel syndrome is second) and the most common nerve compression syndrome in the lower extremity. Knee dislocations are a common cause of common peroneal nerve compression. The patient described has a chronic injury that has not caused complete paralysis or loss of sensibility. The initial injuries would likely cause scar tissue around the knee but are not sharp in nature. Compression of the common peroneal nerve would result in weakness in the anterior compartment muscles caused by slowing of conduction in the deep peroneal nerve and paresthesias to the superolateral foot caused by slowing of conduction in the superficial peroneal nerve.

Answer 2

The correct response is E ## Footnote Because the arteriovenousmalformation in this patient is confined to the skin and subcutaneous tissues, with no involvement of the underlying tendons, muscle units, or bone, the most appropriate management is direct wide surgical excision and skin graft reconstruction. Amputation is contraindicated until all lesser techniques of control have been exhausted. Preoperative embolization is indicated for soft-tissue arteriovenousmalformations of the head and neck region when underlying structures are involved. Laser ablation is unlikely to be successful, because laser penetration would reach no further than the upper layers of the skin, leaving the underlying subcutaneous vessels untreated. Local excision is not recommended because the skin lacks the elasticity required for direct approximation. In addition, local excision of this type of vascular tumor is associated with a high incidence of recurrence, as occurred in this patient.

Answer 3

The correct response is Option A. Infantile hemangioma is the most common tumor of infancy. These lesions typically present between 2 weeks and 2 months of life and have a predictable clinical course, including rapid proliferation during the first 9 months of life (proliferative phase), followed by gradual involution until 3.5 years of age. Infantile hemangiomas most commonly occur in the skin, but can also occur in other sites, with the liver being the most common extracutaneous site. For cutaneous lesions, most are located in the head and neck, followed by the trunk, and then by the extremities. \>95% of infantile hemangiomas are diagnosed by history and physical examination; \<5% require imaging or biopsy for diagnosis. The onset of a red lesion at 2 weeks of age followed by rapid growth is pathognomonic for infantile hemangioma. The tumor affects both sexes, but is 3 to 4 times more common in females. Infantile hemangioma is also more frequent in premature infants and twins who are low birth weight. Some infantile hemangiomas can ulcerate during the proliferating phase.

Answer 4

The correct response is Option B. The saphenous artery supplies vascularity to the saphenous flap, a fasciocutaneous flap that provides only limited coverage of small defects in the region of the knee. The saphenous artery is a branch of the descending genicular artery. The genicular artery arises from the superficial femoral artery. The descending branch of the lateral femoral circumflex artery arises from the profunda femoris and continues distally in the leg as the superior lateral genicular artery. The profunda femoris artery gives off its major branches more proximally in the leg. The superficial femoral artery becomes the popliteal artery at the popliteal fossa. Reproduced with permission of Moore KL, Dalley AF, eds. Clinically Oriented Anatomy. 4th ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 1999:574.

Answer 5

The correct response is D ## Footnote Kasabach-Merritt syndrome accompanies kaposiformhemangioendothelioma. Hemangiomastypically appear shortly after birth and then rapidly enlarge. This is followed by a longer period of slow involution. Vascular malformations, by contrast, are present at birth and then grow with the child. There is no involution. Arteriovenousmalformations are subclassifiedas fast flow in nature, such as arteriovenousfistulae, or as slow-flow malformations, which are primarily venous in nature. Venous anomalies that are extremely large may cause a low-grade bleeding disorder, but this is a localized process and not defined as Kasabach-Merritt syndrome. Angiosarcomasare uncommon malignant neoplasms characterized by rapidly proliferating, extensively infiltrating anaplastic cells derived from blood vessels and lining irregular, blood-filled spaces. Angiosarcomasare aggressive and tend to recur locally, spread widely, and have a high rate of lymph node and systemic metastases. The rate of tumor-related death is high.

Answer 6

Correct answer is option D. Ulnar artery thrombosis and/or superficial arch thrombosis are the most commonly encountered non-traumatic occlusive lesions in the hand. False aneurysms are usually due to penetrating trauma. Allen’s testing should have flow within 3 seconds. Raynaud’s phenomenon affects 5%–10% of the general population. Digital brachial index <0.7 is abnormal and requires further work up.

Answer 7

The correct response is Option A. Beta-adrenergic blockers, including oral propranolol (Hemangeol), would be the best first-line treatment due to the size and location of the hemangioma. Such medications can act to limit growth, decrease bulk, and prevent complications of the hemangioma in a time-sensitive manner. Embolization is often employed in treatment of arteriovenous and lymphatic malformations. It is typically the treatment of choice for complications of larger vessels, rather than the capillaries comprising a hemangioma. Laser therapy is beneficial in the treatment of port-wine stains and telangiectasia post involution of hemangiomas. The hemangioma described in the vignette is clearly in the proliferative phase, making it too large, thick, and deep to respond well to laser therapy alone. Surgical excision is not a viable option in this case due to the extensive size and location of the hemangioma. The treatment is not emergent, since the patient described remains stable, and more conservative options should be exhausted before resorting to resection. Topical corticosteroid is not preferred in this case due to the large size and location of the hemangioma. Topicals should not be applied to the intraoral mucosa, and corticosteroids would be too slow-acting to improve significant symptoms such as bleeding and poor feeding.

Answer 8

The correct response is B Capillary malformations are slow-flow vascular malformations that classically were referred to as "port-wine stains." Facial capillary malformations often occur in a dermatomaldistribution. In Sturge-Weber syndrome, the capillary malformation may involve V1, V2, and/or V3 dermatomes, with ipsilateralocular and leptomeningealvascular anomalies. Arteriovenousmalformations are a combination of arterial and venous channels. MRI best documents the extent of involvement. The mainstay of management is embolization and surgical excision. Hemangiomasare vascular tumors, not malformations. They grow rapidly, often within the first year of life, and regress slowly, often involutingby 5 years of age. Hemangiomastypically are observed and not treated unless they cause visual interference, auditory canal obstruction, airway blockage, or ulceration and bleeding. Treatment regimens include corticosteroids or interferon. Lymphatic malformations are characterized as microcystic, macrocystic, or combined. Lymphatic malformations most commonly occur in the cervicofacialregion, axilla/chest, mediastinum,retroperitoneum, buttock, and perineum, and were classically referred to as "cystic hygromas." Lymphatic malformations in the floor of the mouth and tongue present asmacroglossia, vesicles, and intermittent swelling. Lymphatic malformations may enlarge with infection. Venous malformations are the most common type of vascular malformation. They are present at birth, slowly increase in size as the child grows, and are prone to thrombosis. Maffuccisyndrome includes exophyticcutaneous venous malformations, bony exostoses, and enchondromas. There is also a risk for malignant transformation. The two major categories of vascular anomalies include tumors and malformations. Vascular tumors exhibit rapid growth with endothelial proliferation followed by regression. Vascular malformations have a normal rate of endothelial cell turnover, are present at birth, and grow proportionately with the child.

Answer 9

The correct response is Option A. Beta-adrenergic blockers, including oral propranolol (Hemangeol), would be the best first-line treatment due to the size and location of the hemangioma. Such medications can act to limit growth, decrease bulk, and prevent complications of the hemangioma in a time-sensitive manner. Embolization is often employed in treatment of arteriovenous and lymphatic malformations. It is typically the treatment of choice for complications of larger vessels, rather than the capillaries comprising a hemangioma. Laser therapy is beneficial in the treatment of port-wine stains and telangiectasia post involution of hemangiomas. The hemangioma described in the vignette is clearly in the proliferative phase, making it too large, thick, and deep to respond well to laser therapy alone. Surgical excision is not a viable option in this case due to the extensive size and location of the hemangioma. The treatment is not emergent, since the patient described remains stable, and more conservative options should be exhausted before resorting to resection. Topical corticosteroid is not preferred in this case due to the large size and location of the hemangioma. Topicals should not be applied to the intraoral mucosa, and corticosteroids would be too slow-acting to improve significant symptoms such as bleeding and poor feeding.

Answer 10

Correct answer is option C. Spontaneous radial artery thrombosis is not as common as ulnar artery thrombosis. Unlike ulnar artery thrombosis, it is not usually associated with acute or repetitive trauma, or with embolic disease. It has been associated with Berger’s disease, but the majority of cases are idiopathic. The symptoms are pain, numbness, and discoloration of the tips of the radial-sided digits—usually the index finger and thumb. The occluded portion of the radial artery is almost always located beneath the tendon(s) of the 3rd or 1st dorsal compartment. Streptokinase infusion is usually ineffective. Resection of the thrombosed segment with direct end-to-end anastomosis or repair with a vein graft is necessary in most cases to restore distal blood flow. Digital sympathectomy is indicated for vasospastic disorders with Raynaud’s symptoms and not indicated for this vascular problem. The anterior interosseous artery is not a dominant arterial supply to the deep arch. The symptoms of acute cephalic venous thrombosis are diffuse, generalized swelling, edema, and duskiness of the forearm, arm, and digits.

Answer 11

The correct response is D ## Footnote Rendu-Osler-Weber syndrome, or hereditary hemorrhagic telangiectasia, is an autosomal dominant disorder characterized by malformed vessels in the skin, mucous membranes, lungs, and abdominal viscera. The vessels often become apparent after puberty and increase in severity with age. They may be accompanied by melena, hematuria, hematemesis, epistaxis, or bleeding into the brain or spinal cord. In Klippel-Trénaunaysyndrome, malformation of the capillary lymphatic veins typically affects the extremities and sometimes the trunk. Associated signs and symptoms usually include enlargement of the affected extremity and geographic port-wine stain in that area. Maffuccisyndrome (or Kastsyndrome) is characterized by multiple cutaneous or visceral hemangiomasand enchondromatosis(hemartomatousproliferation of cartilage cells, causing growth distortion, especially exostosesof the fingers and toes) and may lead to malignant degeneration. Parkes-Weber syndrome is similar to Klippel-Trénaunaysyndrome but has an arteriovenousanomaly as its primary vascular lesion instead of a venous anomaly. Sturge-Weber syndrome consists of vascular anomalies of the upper face, choroid plexus, and ipsilateralleptomeninges.

Answer 12

Correct answer is option C. The most appropriate management is immediate coverage of the exposed tibia using a fillet-of-foot free flap. Free tissue from the amputated foot can be transferred to provide appropriate soft-tissue coverage without additional donor site morbidity. The dorsalis pedis artery and/or posterior tibial artery supply vascularity to the flap, and the tibial nerve provides sensory innervation. Above-knee amputation should be considered only as the last possible option. The energy required for ambulation in a patient with an above-knee amputation is much greater than in a patient with below-knee amputation. The reverse rectus femoris flap is not based on a distal pedicle; thus, it should not be used in a patient in whom knee extension must be preserved. A latissimus dorsi flap and split-thickness skin graft could be used for coverage if the amputated part were injured and unavailable as a donor site; however, in this patient there is no evidence of trauma distal to the lower third of the leg. Microvascular replantation cannot be performed in a patient who has sustained an avulsion amputation. In addition, bone shortening of greater than 10 cm would ultimately result in an abnormal gait.

Answer 13

The correct response is Option E. Hemangiomas are the most common tumors found in infancy. These vascular tumors are characterized by rapid growth and increased cellular proliferation. Hemangiomas typically present between 2 weeks old and 2 months old. Vascular malformations, on the other hand, are present at birth and grow slowly. The clinical course of a hemangioma is predictable and includes rapid proliferation during the first 9 months of life, followed by gradual involution until 3.5 years of age. Most cutaneous lesions are located in the head and neck, followed by the trunk, and then the extremities. Extracutaneous sites most commonly involve the parotid glands or liver. Reports of nervous system involvement are rare. Most hemangiomas can be diagnosed by history and physical examination alone. About 5% require biopsy or radiologic imaging. The tumor may involve both sexes but is five times more common in females. The onset of a red lesion followed by rapid growth is the sine qua non for infantile hemangioma. In 30 to 50% of cases, the hemangioma is preceded by a telangiectasia or bruise-like patch known as a herald patch. Some hemangiomas may ulcerate during their proliferative growth phase.

Answer 14

The correct response is Option C. Vascular anomalies are a common source of pediatric morbidity, potentially resulting in cosmetic and functional abnormalities. Two main categories of lesions are hemangiomas and vascular malformations. Vascular malformations are generally named after the vessel types that are involved and are further subdivided into low-flow and high-flow lesions. Low-flow lesions include capillary, lymphatic, venous, and mixed lesions. High-flow lesions include arteriovenous malformations. Both high- and low-flow vascular malformations are almost always present at birth and either grow commensurately with the child or slowly enlarge over a period of years. Treatment is guided by the degree of functional impairment, and many require surgical therapy. Hemangiomas are rarely present at birth. They tend to appear between 2 and 8 weeks of life and grow rapidly. These lesions comprise of rapidly proliferating endothelial cells and follow a predictable clinical course. They usually undergo an aggressive proliferative phase that lasts several months before reaching a plateau phase, when they grow very little. Finally, at about 1 year of life, hemangiomas begin a process of spontaneous involution which may last for up to 4 years. Completion of involution does not mean complete involution, and many hemangiomas may leave cosmetically unacceptable residua. Hemangiomas found in inconspicuous areas can be observed. Those noted in cosmetically or functionally sensitive areas require more aggressive treatment. Treatment options have included topical, intralesional, and systemic corticosteroids, laser treatment, interferon-alfa, and surgical resection. However, each of these treatments carries marked risks and still does not provide consistent, reliable success. In June 2008, a French group reported rapid resolution of hemangiomas in children treated with propranolol for pediatric cardiomyopathy. This serendipitous finding led to a landmark paper and has resulted in a paradigm shift in the clinical care of these patients. Many groups have now confirmed their findings with very promising results. The use of propranolol for hemangiomas remains off-label, but there are centers of excellence with institutional review board protocols in place to further study its clinical effects. Recommended doses vary but frequently start at 0.5 mg/kg/day divided three times daily and slowly titrated over 1 to 2 weeks to 2 mg/kg/day. Most centers recommend obtaining pretreatment electrocardiography (ECG); if normal, then therapy can be initiated on an outpatient basis. If the ECG is abnormal, then pretreatment cardiology evaluation is warranted. Dose titration, blood pressure, and heart rate are checked regularly. The most common adverse effect is lethargy. Other adverse effects include hypoglycemia, hypotension, and bradycardia. All children drool, and this has not been reported as a complication of propranolol administration. Hypoglycemia can be a potentially life-threatening complication that can also cause seizures. Parents are counseled to give food with medication. Many centers regularly check blood glucose. An image of a child with upper lip hemangioma treated with propranolol for 14 months is shown.

Answer 15

The correct response is A ## Footnote Hemangiomasare true benign neoplasms that have plump, rapidly dividing endothelial cells and an increased quantity of mast cells on histopathologicexamination. These mast cells produce heparin, which is believed to be the primarily stimulus for migration of capillary endothelial cells. The number of mast cells begins to decrease as the hemangiomainvolutes. During the proliferation phase of a hemangioma, the levels of circulating 17-beta estradiol are increased. In addition, the number of binding sites for 17-beta estradiol is actually increased, not decreased. Collagenase activity is increased in all enlarging hemangiomasas the endothelial cells destroy the basement membrane to allow for the formation of new capillary tubules. A multilaminatebasement membrane is also observed. In contrast, vascular malformations exhibit flattening of the endothelium, thinning of the basement membrane, and a normal quantity of mast cells.

Answer 16

Correct answer is option a. This patient has an injury of the terminal branch of the deep peroneal nerve, which innervates the adjacent side of the great and second toes. The deep perineal nerve arises from the bifurcation of the common perineal nerve between the fibula and peroneus longus in the lateral compartment, then continues deep to the extensor digitorum longus to the anterior surface of the interosseus membrane. It meets the anterior tibial artery in the proximal third of the leg and continues with it distally, passing deep to the extensor retinaculum and medial to the dorsalis pedis artery. It enters the dorsum of the foot, where it divides into terminal, medial, and lateral branches. The superficial peroneal nerve divides into medial and lateral branches. The medial branch innervates the medial aspect of the great toe and adjacent side of the second and third toes. The lateral branch innervates the contiguous sides of the third through fifth toes. The lateral plantar nerve supplies sensation to the fifth and lateral half of the fourth toe. It is a terminal branch of the tibial nerve. The saphenous nerve innervates the medial aspect of the foot, while the sural nerve innervates the dorsolateral aspect.

Answer 17

The correct response is E The most appropriate action at this time is close observation with frequent vision and refractive examination by an ophthalmologist. Hemangiomasare the most common tumor of infancy, with a predilection for the head and neck and the upper eyelid. They are more common in females, usually noted in the weeks following birth, and are characterized by a rapid proliferative phase of 6 to 12 months. This phase is followed by a period of quiescence, then slow involution beginning the second year of life. Most hemangiomasare initially managed by observation. When a hemangiomainterferes with the visual axis or airway, medical or surgical intervention may be indicated. Periocularhemangiomasmay permanently affect vision by causing ptosis of the eyelids, resulting in visual field obstruction, strabismus, or anisometropia(a condition in which the two eyes have unequal refractive power). Each of these may, in turn, result in astigmatism, amblyopia (impairment of vision without an organic cause within the eye, in this case, caused by deprivation of visual stimuli centrally), or blindness. As this particular lesion is not interfering with the child's vision and, based on her age, is likely to enter a quiescent phase, observation is the most reasonable course of action. Cosmetic and functional results of spontaneous involution, particularly for smaller lesions, are often superior to those obtained by invasive treatment. Systemic corticosteroids can have multiple side effects, and intralesionalcorticosteroids carry the risk of blindness secondary to central retinal artery occlusion. Patching of the contralateral eye is usually performed after debulkingof a hemangiomato treat amblyopia.

Answer 18

The correct response is Option E. Observation is the most appropriate management of the child with the nonulcerating, nonobstructive hemangioma described. Hemangiomas are a vascular tumor of infancy. They are typically not present at birth but appear within the first few months of life. They undergo a proliferating phase of rapid growth in the first 3 to 6 months of life, followed by an involuting phase that can last up to 12 years of age but is completed most commonly by age 5 to 7 years. Finally, an involuted phase is reached with complete regression of the hemangioma, leaving some scar or discoloration in 50% of patients. Diagnosis is often made by a complete history and physical examination. Hemangiomas should not be confused with venous malformations that are often present at birth. These malformations can maintain a consistent color and grow with the child. Doppler ultrasonography, CT scan, and MRI can be useful in diagnosis. Given that most hemangiomas ultimately regress completely, most are managed appropriately with observation and reassurance of the family. Intervention is indicated to avoid morbidity and mortality from alarming hemangiomas. These include ulcerating hemangiomas (bleeding and infection), obstructing hemangiomas (sight, hearing, airway), and hemangiomas compromising hemodynamic stability. Local or systemic corticosteroids, interferon, the pulsed-dye laser, and surgical excision are treatment modalities in the armamentarium to treat hemangiomas.

Answer 19

The correct response is Option E. The most likely diagnosis is venous malformation. Venous malformation is a congenital lesion that enlarges slowly over time. Treatment involves either sclerotherapy or resection. Congenital hemangioma is fully grown at birth. There are two types: rapidly involuting congenital hemangioma (RICH) and noninvoluting congenital hemangioma (NICH). RICH rapidly involutes postnatally and is usually fully regressed by age 12 months. Treatment is rarely necessary. NICH does not undergo involution; it remains the same size over the course of the patient’s lifetime and would not be enlarging at age 2 years. NICH is rarely problematic, but may be resected if it is causing a significant deformity. Infantile hemangioma is usually noted 2 weeks after birth and enlarges rapidly over the first few months of life. By age 1 year, the tumor begins to regress and would be decreasing in size at age 2 years. Problematic lesions are treated with intralesional corticosteroid, oral prednisolone, or resection. Kaposiform hemangioendothelioma is present at birth and does not increase in size. It is often large, superficial, and diffuse. It typically involves the trunk and extremities. Patients commonly have Kasabach-Merritt phenomenon (thrombocytopenia, bruising, and bleeding) as well. First-line treatment is administration of vincristine. Pyogenic granuloma usually appears in early childhood as a rapidly growing, red lesion. Lesions are commonly small (less than 1 cm) and complicated by bleeding. Definitive treatment is excision.

Answer 20

The correct response is Option E. Hemangiomas are proliferative lesions that occur most often in the first weeks of life and then proceed into a proliferative phase. Involution and spontaneous regression occur for most lesions. The lesions frequently occur on the face, scalp, chest, and back but can occur on the extremities as well. The indications for treatment are related to symptoms or secondary effects such as: bleeding/ulceration, visual obstruction, and impingement on the mouth prohibiting proper feeding. Small lesions without symptoms should be observed and resolution is anticipated. However, if treatment is required, the options include intralesional or systemic steroids, systemic propranolol therapy, and surgical resection. If propranolol is used, it must be initiated cautiously with inpatient observation due to risks of hypoglycemia and bradycardia. Sirolimus has been found to be helpful for complex lymphatic and vascular malformations. It is not indicated for hemangioma treatment.

Answer 21

The correct response is D The target chromophorefor this capillary malformation isoxyhemoglobinin the ectaticvessels within the dermis. The three primary absorption peaks are 418 nm, 542 nm, and 577 nm. The flashlamp-pumped, pulsed-dye laser was specifically designed for vascular lesions with a wavelength of 585 nm. It provides selective photothermolysiswithout unwanted thermal damage and can be used in patients with light and dark skin tones. Additional treatments may be required especially in the centrofacialarea. Disadvantages include postoperative purpuraand transient dyspigmentation. Vesiculation, crusting, textural change, and scarring occur rarely. The KTP laser has a wavelength of 532 nm. It does work well with telangiectasias; however, it has decreased tissue penetration and is not as effective as the pulsed-dye laser in treatment of port wine lesions. Also, the 532-nm wavelength is absorbed by melanin, which limits its use in patients with darker skin tones. The erbium:YAG(2490-nm) and Q-switched ruby (694-nm) lasers are not targeting the oxyhemoglobinchromophoreand are used in other cutaneous applications. The argon-pumped tunable dye laser can be used to treat capillary malformations. However, it causes nonspecific thermal damage with higher incidences of hypertrophic scarring, textural changes, and dyspigmentation.

Answer 22

The correct response is E This patient has an arteriovenousmalformation, or AVM, which is characterized by rapid, turbulent blood flow through multiple feeding vessels from the high-pressure arterial system to the low-pressure venous system. These lesions are often pulsatile; a thrill or bruit will be heard on auscultation. MRI is most helpful in delineating the anatomy of the AVM and its flow characteristics. Because treatment can be associated with the same life-threatening complications as the existence of the lesion itself, appropriate management typically requires multi-modality therapy, including preoperative correction of any underlying consumptive coagulopathy, angiographic embolization of feeding vessels, complete surgical excision of the malformation, and soft-tissue reconstruction. Radiation therapy and laser ablation are not appropriate management options for a patient with a congenital AVM. Ligation of feeding vessels or angiographic embolization alone will only worsen this patient's condition.

Answer 23

The correct response is Option D. First-line treatment of a small problematic infantile hemangioma is intralesional corticosteroid (triamcinolone 3 mg/kg). If the tumor is too large to inject, then oral corticosteroid (prednisolone 3 mg/kg daily) or propranolol is initiated. Interferon is no longer used to treat infantile hemangioma because it may cause spastic diplegia when administered to infants. The common side effects of propranolol include gastrointestinal effects (vomiting, diarrhea, and constipation), rash, fatigue, and hypersomnia. Severe side effects may include bradycardia, hypotension, chest pain, shortness of breath, bleeding, bronchospasm, and glaucoma. In pediatric patients, propranolol has been associated with hypoglycemia that may occur without the characteristic jitteriness. Proliferating hemangiomas should not be treated with pulsed-dye laser therapy because accelerated involution does not occur, and patients are at risk for ulceration, pain, bleeding, hypopigmentation, and scarring. The pulsed-dye laser is indicated, however, to treat residual telangiectasias after the tumor has involuted. Corticosteroid complications (e.g., adverse neurodevelopment, aseptic necrosis of the femoral head, diabetes mellitus, osteoporosis, adrenal insufficiency, cataracts, glaucoma, infection, gastric irritation) have not been observed in patients treated with corticosteroid for infantile hemangioma. Although increased blood pressure has been observed, the clinical significance of this is unclear, and no adverse effects have been reported. Twenty percent of infants develop a temporary cushingoid appearance that disappears during tapering of the medication. Approximately one third of infants exhibit decreased gain in height but return to their growth curve by 24 months of age.

Answer 24

The correct response is Option B. GLUT-1 is a specific marker for infantile hemangioma and is often used by pathologists to confirm the diagnosis. VEGF and TGF-B are incorrect, because while these markers may often be present in hemangiomas, they are not as specific as GLUT-1 in confirming the diagnosis of hemangioma. Staining for FGFR-3 is not routinely used in confirming the diagnosis of hemangioma. FGFR-3 mutations are implicated in a number of syndromes, including Muenke syndrome, epidermal nevus, and achondroplasia. Staining for TNF-1 is also not routinely used in confirming the diagnosis of hemangioma.

Answer 25

The correct response is Option E. Infantile hemangiomas are one of the most common infant tumors. Hemangiomas of the parotid gland often surround the facial nerve and are difficult to resect completely. Luckily many parotid hemangiomas resolve on their own without the need for resection. Beta-adrenergic blockers can be effective in decreasing the size for symptomatic or large hemangiomas with minimal side effects. The parotid hemangiomas are too deep for significant improvement with laser treatment. After involution, a small portion of children with a history of infantile parotid gland hemangioma have excess skin that requires surgical reduction. Percutaneous sclerotherapy has a role in the management of venous malformations, but not hemangiomas.

Answer 26

The correct response is B ## Footnote This vascular lesion is a facial capillary malformation (port-wine stain) involving the V1 (ophthalmic) dermatome. It is part of Sturge-Weber syndrome and is associated with ipsilateralabnormalities of the pialand ocular vessels. It may be accompanied by gyriformcalcifications on the outer layers of the cerebral cortex, which increase the child’s risk of seizures, contralateral hemiplegia, and developmental delays. The child also is at risk for retinal detachment, glaucoma, and blindness. Therefore, she should be seen regularly by an ophthalmologist in the first few years of life. Similar to arteriovenousand lymphatic malformations, capillary malformations are types of vascular malformations that result from errors in embryologic development and are composed of dysplastic vessels. These lesions are present at birth, grow as the child grows, and do not regress. They may be associated with underlying soft tissue and bony overgrowth. Hemangiomasgrow rapidly during the first year of life, then slowly regress over childhood and never appear in adulthood.

Answer 27

The correct response is B ## Footnote This 40-year-old man has a capillary malformation, or port-wine stain. These lesions are often seen in the distribution of the abducens(VI) and facial (VII) nerves. If left untreated, cobblestoningand progressive darkening may occur due to ectasiaof the vessels within the capillary malformation. An arteriovenousmalformation is a high-flow lesion often characterized by a palpable thrill or bruit. Compression of the lesion results in occlusion of the associated arteriovenousfistula, causing a baroreceptor response and an increase in blood pressure. This leads to a decrease in heart rate and is known as the Branham sign. Hemangiomasare vascular tumors that appear just after birth and rapidly enlarge during the first year of life. Because spontaneous regression is common, conservative management is suggested for non-life-threatening lesions that do not obstruct the airway or visual axis. Lymphatic malformations, also known as cystic hygromas, occur most frequently in the head and neck region and often enlarge in response to an adjacent infection. Recurrent swelling can lead to scarring, which will significantly decrease the size of the lesion. Venous malformations are low-flow lesions composed of dilated venous channels. These lesions are compressible and have a propensity to fill with blood when the patient changes body positions. When the malformation is associated with thrombosis, pain may result.

Answer 28

The correct response is C ## Footnote The lesion is a proliferative hemangioma, which should be observed closely at this point. Administration of interferon alfa-2a is beneficial but is not a first-line treatment for this lesion. Intralesionalinjection of a corticosteroid also may be of benefit but probably is not indicated in this area in a lesion that is not bleeding, ulcerating, or causing other problems. Yellow pulsed-dye laser therapy is effective when used in the early proliferative phase but is unlikely to offer significant benefits by this stage in a lesion this large. Surgical excision is inappropriate for such a young patient and would leave a large scar in a visible location.

Answer 29

The correct response is Option E. Hemangiomas are vascular tumors characterized by increased cellular proliferation. Classically, they exhibit rapid growth and slow regression. Vascular malformations are present at birth and grow slowly. Hemangiomas are the most common tumor of infancy. They occur at an incidence that is five times more common in girls and are more common in premature infants. Vascular malformations have an equal sex ratio. Multiple cutaneous hemangiomas may be present with extracutaneous sites, most commonly involving the parotid glands or the liver; nervous system involvement is rare. In 30 to 50% of cases, hemangiomas are preceded by a premonitory mark, such as a telangiectasis or a bruise-like patch (herald patch).

Answer 30

The correct response is B Lymphatic malformations, previously known as cystic hygromasand lymphangiomas, are lesions composed of lymph-filled dysplastic vessels that occur in infancy; 90% of these lesions are noted by age 2 years. Males and females are affected equally. These malformations enlarge commensurately with growth of the child; cellulitis and respiratory infection are most likely to cause rapid growth of the lesion. Large lymphatic anomalies of the face can result in airway compromise, feeding difficulties, bleeding, and infection. Approximately 80% of patients with large cervicofacialmalformations have hypertrophy and distortion of the facial bones, typically resulting in an open bite, prognathism, and/or malocclusion. The mandible is affected most frequently, and bony overgrowth often persists despite soft-tissue resection. Examination of hypertrophied bone has shown the presence of lymphatic channels, providing a link between the malformations and involvement of osseous structures. Although orthognathicprocedures are generally not performed until bony growth has been completed, patients with gross facial disharmony may require urgent treatment. Unlike hemangiomas, lymphatic malformations do not have proliferative capacity, but enlarge in response to fluid accumulation, inadequate drainage, or anomalous lymphatic channels. Bruits are not associated, and mediastinalinvolvement occurs in only 2% of affected patients. Although the lesions can shrink spontaneously, this is not considered involution or regression but is more likely caused by lymphaticovenousshunting.

Answer 31

Correct answer is option E. The clinical and angiographic findings are most consistent with hypothenar hammer syndrome, which is thrombosis of the ulnar artery and proximal superficial arch. This syndrome is the most common type of post-traumatic upper extremity arterial occlusion and results from repetitive injury to the hypothenar region of the hand. Symptoms of pain, cold sensitivity, and numbness are common after thrombosis of the artery within Guyon’s canal. The syndrome occurs most commonly in male laborers who usually use the palm of the hand as a hammer, resulting in injury to the ulnar artery as it is crushed between the roof of Guyon’s canal and the hook of the hamate and pisiform. Patients with this condition are often smokers. Treatment consists of resection of the thrombosed segment followed by vein grafting. Previous treatments include ligation of the artery alone. Smoking cessation, calcium channel blocker therapy, and biofeedback have all been used to treat vasospastic conditions of the hand and would not be effective in this situation.

Answer 32

The correct response is B

Answer 33

The correct response is E ## Footnote In this infant who has a hemangiomathat is obstructing the visual axis, the most appropriate management is immediate surgical resection to preserve vision. Studies have shown that children younger than age 1 year are susceptible to the development of amblyopia if visual obstruction is present for a period of time as short as one week. Once the hemangiomahas been carefully excised, primary closure can be attempted; larger wounds should be covered with a skin graft or flap because of the risk for lagophthalmosand corneal exposure. Because systemic administration of corticosteroids has been shown to result in significant regression of lesions, it is the recommended course of therapy in patients with hemangiomasthat do not obstruct the visual axis or airway. In these patients, doses of prednisone 2 mg/kg/daily to 4 mg/kg/daily are prescribed for two to three weeks and then tapered to 1 mg/kg/daily before the prednisone is discontinued. Although success rates of as high as 60% have been reported, associated complications in infants can include adrenal suppression, immunosuppression, growth arrest, and the development of cushingoidfacies. Intralesionalcorticosteroids such as betamethasone, dexamethasone, and triamcinolone can be extremely effective when injected at multiple sites under sedation or general anesthesia. As many as three separate injections can be administered every four to eight weeks, and marked involution has been reported in a majority of patients. Nevertheless, this course of treatment is rarely recommended because patients are at risk for significant complications, including perforation of the globe, infection, renal artery embolism, blindness, eyelid necrosis, and atrophy of soft tissues. Interferon alfaÐ2a is a newly recognized treatment of hemangiomas. This agent is thought to inhibit the migration and proliferation of endothelial cells through a blockade of basic fibroblast growth factor. Complications, which are infrequent, can include low-grade fever, a transient increase in liver enzymes, and rebound tumor growth, which can be prevented by prolonging the course of therapy. However, investigational studies of the use of interferon alfa-2a for the treatment of hemangiomashave been limited to those patients in whom corticosteroid therapy has been unsuccessful. Radiation therapy is effective for treatment of proliferative hemangiomasbut is often associated with significant adverse effects, especially in infants. These include the potential for injury to the globe and optic nerve, as well as the risk for subsequent induction of malignancy, such as thyroid carcinoma. Therefore, this treatment is limited to patients who have high risk lesions that have not responded to corticosteroid therapy.

Answer 34

The correct response is Option E. The most likely diagnosis is kaposiform hemangioendothelioma, which is a vascular tumor that is usually present at birth. The lesion can cause Kasabach-Merritt phenomenon: extreme thrombocytopenia (\<25,000/mm3) from platelet trapping within the tumor which leads to spontaneous bleeding and bruising. Treatment is either vincristine or rapamycin. Infantile hemangioma, congenital hemangioma, capillary malformation, and arteriovenous malformation do not cause Kasabach-Merritt phenomenon or any other type of coagulopathy.

Answer 35

The correct response is Option D. The lesion shown is a venous malformation. Unlike infantile hemangioma, these lesions are present at birth and grow proportionately with the child, and therefore, cannot be expected to resolve over time. Venous malformations can become more symptomatic with growth and swelling or symptoms are exacerbated by dependency. In addition, rapid swelling and pain can result from phlebothrombosis (clotting) in the anomalous venous channels. The most effective treatment for symptomatic lesions is sclerotherapy. Operative excision is generally reserved for focal lesions or those with significant functional effects; because the lesions involve critical tissues and are poorly circumscribed, operative excision is rarely curative. The other options presented have been used to treat infantile hemangioma, but have not demonstrated effectiveness for venous, arteriovenous, or lymphatic malformations.

Answer 36

The correct response is Option C. Systemic propanolol therapy has quickly become a safe and effective treatment for hemangioma and is well tolerated with few side effects. It is considered the first line medical treatment of infantile hemangiomas at many medical centers. Surgical treatment is generally reserved for lesions that are refractory to medical management which present a functional impairment such as affecting or obstructing vision. Laser ablation and intralesional corticosteroid injection are less effective in several meta-analyses and some randomized controlled trials. Observation is reasonable for small, stable lesions in cosmetically non-sensitive areas.

Answer 37

The correct answer is option D. In 1982, Mulliken and Glowacki proposed a binary classification system for vascular anomalies based on pathologic features. This system divides vascular anomalies into two primary biological categories: 1) vasoproliferative or vascular neoplasms and 2) vascular malformations. Vasoproliferative neoplasms, such as hemangioma and Kaposiform hemangioendothelioma, have increased endothelial cell turnover (i.e., they proliferate and undergo mitosis) because they are neoplasms. Vascular malformations do not have increased endothelial cell turnover. Instead, vascular malformations are structural abnormalities of the capillary, venous, lymphatic, and arterial systems that grow in proportion to the child. The lesion shown in the photographs grew in proportion to the child; therefore, it is a malformation, not a vasoproliferative neoplasm. Given the absence of a bruit or thrill, it is unlikely to be an arteriovenous malformation; therefore, a slow-flow lesion such as a lymphatic malformation is the most likely diagnosis. Although a port-wine stain is a type of venous malformation, it is characterized by its very superficial location, which causes the skin to be discolored red (hence the name). Such findings are not present in the photographs shown.

Answer 38

The correct response is Option D. Non-involuting congenital hemangioma (NICH) is a rare form of hemangioma that is present at birth, is stable in size over time (ie, does not involute), and often has a white-grey rim. It is histologically and radiographically similar to infantile hemangioma except that it stains negative for glucose transporter protein 1 (GLUT1). Venous malformation and lymphatic malformation are low-flow and do not fit the clinical description: they are typically darker in color, amorphous in form, and are compressible. Dermatofibrosarcoma protuberans is rare in children, begins more like a flat scar, and grows over time. It is not a high-flow lesion.

Answer 39

The correct response is E ## Footnote Lymphatic malformations develop early in embryogenesis and result in deforming, enlarged lymphatic chambers that can occur throughout the face, trunk, or extremities. They can be associated with bony overgrowth. The overlying skin may be normal or contain multiple small, fluid-filled vesicles. Diagnosis of large lesions can often be made during prenatal ultrasonography. Lymphatic malformations of the neck have previously been called "cystic hygromas." "Lymphangioma" is another misused term because it implies a tumor proliferation, whereas the pathology involves dilated, aberrantly formed lymphatic channels. Although some lesions are noted early in life, others may present in later childhood or early adulthood. Cervicofaciallesions can cause early airway obstruction and occasionally necessitate tracheotomy. Treatment focuses on symptom management, contour, and function improvement. Malformations are prone to infection, and cellulitis can rapidly progress to sepsis. Early antibiotic administration is essential, and parents should have prescriptions on hand to start treatment at the first sign of symptoms. Surgical resection is a mainstay of treatment, although dissection of the malformation from vital structures can be difficult. When complete clearance of the mass is not feasible, some degree of recurrence is likely. Complete removal can result in dramatic contour and functional benefit. Surgery should be deferred until after infancy, if possible, to facilitate dissection around delicate neurovascular structures. Diffuse or large lesions can be removed in stages. Postoperative compression and drainage are important for managing fluid accumulation. Limb amputation is not indicated in the patient described. Compression therapy alone will have no long-term effect on this malformation and will not prevent future infection. Sclerotherapywith hypertonic saline, sugar, and oil solutions have not been successful. Intralesionaltreatment with bleomycinor OK-432 has been shown to shrink isolated masses and may be useful in recurrent lesions. Laser treatment has limited temporizing benefit for oral mucosal lesions, although it has no effect on the deeper portion of the mass.

Answer 40

The correct response is D Approximately 80% of hemangiomasare noted in the first month of life. Hemangiomas are the most common tumors of infancy; their incidence at birth ranges from 1% to 3%, and nearly one-third are noted before the neonate is discharged from the nursery. Boys are reportedly three times more likely than girls to be affected. Approximately 60% of hemangiomasoccur in the head and neck region.

Answer 41

The correct response is Option A. The patient described has focal hemangioma. Medical therapy is the first line of treatment in this case when there are minimal ophthalmologic findings. There is a subset of periorbital hemangiomas for which surgical resection is indicated: well-localized hemangioma amenable to resection, greater than 2 diopters of astigmatism, visual obstruction, or nonresponsive to medical therapy. In a study by Arneja and Mulliken, the best ophthalmologic improvement occurred when the hemangioma was resected at less than age 3 months. Laser therapy can treat discoloration only and will not decrease the bulk of the lesion. Surgical debulking is indicated if the hemangioma does not clinically respond (softer, lightening in color, no improvement in/worsening of astigmatism).

Answer 42

The correct response is Option B. GLUT-1 immunostain positivity is the defining feature in distinguishing infantile hemangioma from other vascular anomalies. HER2/neu is a marker that is used in breast cancer pathological studies. HER2 is a tyrosine kinase receptor that is part of the human epidermal growth factor family. Overexpression is found in approximately 30% of breast cancers and is associated with a more aggressive clinical course with higher recurrence. VEGFR-3 marker positivity is observed in lymphatic malformations. PROX-1 marker positivity is observed in lymphatic malformations, as well as tufted angioma and Kaposiform hemangioendothelioma. D2-40, like PROX-1, is a marker for lymphatic origin, and would not be expected to be broadly positive within an infantile hemangioma.

Answer 43

The correct response is D Hemangiomasare vascular lesions that enlarge through a process of rapid cellular proliferation known as hyperplasia. These lesions have an increased rate of cellular turnover uncommon to all other vascular malformations. Instead, vascular malformations, which can be classified as arterial, capillary, lymphatic, or venous, have structural anomalies not present in hemangiomas. Aplasia describes the lack of development of an organ or tissue; dysplasia refers to an abnormality in the developmental process. Fibroplasia is the formation of fibrous tissue. Metaplasia describes a change in the type of adult cells within a tissue to another form of cells not normal for that tissue.

Answer 44

The correct response is Option B. GLUT-1 immunostain positivity is the defining feature in distinguishing infantile hemangioma from other vascular anomalies. HER2/neu is a marker that is used in breast cancer pathological studies. HER2 is a tyrosine kinase receptor that is part of the human epidermal growth factor family. Overexpression is found in approximately 30% of breast cancers and is associated with a more aggressive clinical course with higher recurrence. VEGFR-3 marker positivity is observed in lymphatic malformations. PROX-1 marker positivity is observed in lymphatic malformations, as well as tufted angioma and Kaposiform hemangioendothelioma. D2-40, like PROX-1, is a marker for lymphatic origin, and would not be expected to be broadly positive within an infantile hemangioma.

Answer 45

The correct response is Option E. The lesion described is an infantile hemangioma. This tumor is in the involution phase in a nonproblematic area. The most appropriate next step in management for this lesion is observation. Infantile hemangiomas are benign vascular tumors that affect 5% of the population. They typically appear at 2 weeks of age in contrast to vascular malformations (e.g., venous malformations, lymphatic malformations, arteriovenous malformations) which are present at birth. They enter a proliferative phase of rapid growth, plateau, and begin involution around age 12 months. Involution is complete by age 4 years in 90% of patients. Most infantile hemangiomas are not problematic and do not require treatment. Following involution, a residual fibrofatty area can be excised if it is bothersome. Concerning hemangiomas, such as near the eyelid, can cause obstructive visual symptoms. Problematic hemangiomas can be treated to slow progression and shrink the lesion. This is most effective early in the proliferative phase. Those lesions can be treated pharmacologically with oral propranolol. Focal symptomatic lesions can be injected with corticosteroids. Topical timolol may be used for small, superficial lesions. Pulse dye laser can be used to treat telangiectasias that remain after involution. Sclerotherapy is the treatment for vascular malformations and macrocystic lymphatic malformations. Embolization followed by resection is the treatment for symptomatic arteriovenous malformations. A punch biopsy is not indicated for this lesion, which is following the classic growth pattern of a classic infantile hemangioma in the involution phase.

Answer 46

The correct response is A This 18-year-old woman has a well-developed, high-flow arteriovenousmalformation of the index finger with diffuse soft-tissue involvement. The most appropriate management of this vascular malformation is preoperative embolization (as performed in patients with high-flow malformations of the head and neck). Preoperative embolization can facilitate excision, because thrombosis of the vessels can help define the true extent of the lesion. To this extent, preoperative embolization can help direct surgical excision intraoperativelyas well as limit blood loss. Embolization alone is palliative but not curative. It may at best temporarily alleviate pain and increased temperature. Proximal ligation of the arterial vessels is ineffective and will result in a rebound increase in vascularity of the arteriovenousmalformation. Ray amputation alone is possible but is not the best functional alternative in this patient. Because of the diffuse involvement of the soft tissues, adequate excision of this lesion would require deglovingor near deglovingof the index finger soft tissues. A great toe wraparound flap will not deliver sufficient soft tissue, and the functional result of any soft-tissue reconstruction of a deglovedindex finger will be functionally far inferior to that of ray amputation.

Answer 47

The correct response is C Port-wine stains, or capillary malformations, are slow-flowing congenital vascular anomalies characterized by ectaticvessels located at various levels within the dermis. Histologic analysis has identified decreased numbers of neural structures associated with the ectaticvasculature, which may indicate an etiology related to the neural modulation of vascular flow. Unlike hemangiomas, capillary malformations exhibit normal endothelial cell turnover. The treatment of choice for capillary malformation is pulsed dye laser therapy, which typically uses a wavelength that targets oxyhemoglobin(577, 585, and 595 nm) and results in intravascular coagulation. The response to treatment is variable and most patients do not achieve complete clearing. Lesions located on the central face and limbs tend to be less responsive than lesions found on other parts of the face, neck, and trunk. Biopsy studies and noninvasive assessments have shown that deeper and smaller vessels within a capillary malformation tend to remain untreated after the application of pulsed dye laser treatment.

Answer 48

The correct response is B ## Footnote This patient has bilateral proliferating parotid gland hemangiomas. Hemangiomasare the most common tumor of infancy and the most common tumor of the parotid gland in childhood. They tend to be more common in females and may be bilateral. Administration of a systemic corticosteroid would be the most appropriate initial treatment. Observation is acceptable for smaller lesions and when functional compromise is not present. In this case, compression of the external auditory canal may result in speech problems due to impaired hearing. Although systemic corticosteroids may have adverse effects, they tend to be well tolerated in patients without contraindications. Patients who do not respond to corticosteroids may still respond to interferon. Interferon is usually not the first line of treatment because it has more toxic adverse effects. Surgical excision is warranted if medical management is unsuccessful or if there are residual skin and soft-tissue changes after the lesion has involuted.

Answer 49

The correct response is Option E. According to the Children’s Hospital of Boston’s experience with arteriovenous malformations, the Schobinger stage of the lesion is most important when considering the individual’s recurrence risk for these lesions. In their experience, lesion recurrence was more associated with advanced Schobinger staging than with quiescent lesions, whether these were treated with embolization alone or embolization followed by resection. Enlargement of the malformation describes a Schobinger Stage I lesion, which has the lowest risk for recurrence in the Boston Children’s series. The presence of these lesions, while concerning to parents, typically has a recurrence risk of 80% with embolization alone and 21% with embolization followed by resection. Patient sex, age, and lesion location did not correlate with lesion recurrence in the Boston Children’s series. Quiescence of the malformation represents a Schobinger Stage I lesion, which is the lowest Schobinger stage description among the options listed. This stage lesion has a recurrence risk of 99% with embolization alone and 81% with embolization followed by resection.

Answer 50

The correct response is A

Answer 51

The correct response is Option C. The most appropriate treatment for this lesion is radical resection, with the goal of eradicating the lesion. Although the lesion is prone to recurrence, this approach maximizes the chances for a cure. This patient has recurrent bleeding and is at risk for death due to exsanguination. Observation is not acceptable in this otherwise healthy 68-year-old. Laser therapy is futile as it will only affect superficial dermal structures, and this lesion clearly involves large vessels and deeper tissues. Corticosteroid therapy is appropriate for management of infantile hemangioma but has no role in the treatment of arteriovenous malformations. Sclerotherapy is not indicated in this patient.

Answer 52

The correct response is D The 3-month-old infant has a vascular malformation, which is comprised of anomalous vascular structures. Vascular malformations are present at birth and do not regress; they grow commensurately with the child. Skeletal deformities are associated. Treatment is often difficult, and recurrence rates are high. Surgical excision of the entire lesion is required to remove all residual tissue, decreasing the risk for recurrence. The 2-year-old boy has a pyogenic granuloma, a rapidly growing, pedunculatedlesion in the central face that often bleeds easily. Pyogenic granulomas can be differentiated from hemangiomasbecause they break down spontaneously with bleeding and do not typically develop during the first year of life. Curettage or excision is appropriate. Hemangiomasare true neoplasms that develop by age 1 year, grow rapidly for the next four to eight months, and then regress spontaneously. Less than 5% of hemangiomasbreak down spontaneously with bleeding. Skeletal deformities are rarely associated. Lymphatic malformations can be superficial or deep. Superficial lymphatic malformations are comprised of anomalous lymphatic remnants that manifest as clear vesicles. Deep lymphatic malformations appear similar to hemangiomasbut are soft and compressible on palpation. Bony overgrowth is associated.

Answer 53

The correct response is A ## Footnote The clinical history and MRI findings strongly suggest that the condition described is an arteriovenousmalformation. When there is cutaneous involvement, these anomalies can be observed at birth as a faint area of pink discoloration. Over time, there is a tendency for gradual expansion of the malformation to involve adjacent tissues and to become warm and painful. They are considered "high flow" lesions, implying a significant arterial component to the mass. Most vascular anomalies can be diagnosed clinically. Gadolinium-enhanced MRI can be useful to determine the extent and nature of the vascular components in the lesion. In spite of significant advances in the understanding and classification of vascular anomalies, many malformations are still mislabeled as hemangioma. This type of malapropism is not inconsequential, as the natural history and recommended interventions for these distinct lesions are very different. Infantile hemangiomais a vascular tumor that typically appears shortly after birth, grows aggressively over the first year of life (proliferation), and subsequently regresses (involution). Intervention is rarely indicated, except when there is functional impairment or to facilitate aesthetic improvement after involution. In contrast, vascular malformations are present at birth, tend to enlarge gradually over time, and do not regress. Treatment of these lesions depends on the type of malformation and includes sclerotherapywith or without resection for lymphatic and venous malformations, as well as selective embolization with or without resection in arteriovenousmalformation. Infantile hemangiomais incorrect for the reasons described above. Lymphatic and venous malformations are present at birth and enlarge with growth, but they do not have significant arterial components, and they appear as "low flow" lesions on gadolinium-enhanced MRI. Sturge-Weber syndrome is the associated findings of capillary malformation in the V1 distribution of the trigeminal nerve, leptomeningealvascular malformations, glaucoma, and seizure disorder.

Answer 54

The correct response is E ## Footnote Venous malformations are the most common type of vascular malformation. They are present at birth, slowly increase in size as the child grows, and are prone to thrombosis. Maffuccisyndrome includes exophyticcutaneous venous malformations, bony exostoses, and enchondromas. There is also a risk for malignant transformation. Arteriovenousmalformations are a combination of arterial and venous channels. MRI best documents the extent of involvement. The mainstay of management is embolization and surgical excision. Capillary malformations are slow-flow vascular malformations that classically were referred to as "port-wine stains." Facial capillary malformations often occur in a dermatomaldistribution. In Sturge-Weber syndrome, the capillary malformation may involve V1, V2, and/or V3 dermatomes, with ipsilateralocular and leptomeningealvascular anomalies. Hemangiomasare vascular tumors, not malformations. They grow rapidly, often within the first year of life, and regress slowly, often involutingby 5 years of age. Hemangiomastypically are observed and not treated unless they cause visual interference, auditory canal obstruction, airway blockage, or ulceration and bleeding. Treatment regimens include corticosteroids or interferon. Lymphatic malformations are characterized as microcystic, macrocystic, or combined. Lymphatic malformations most commonly occur in the cervicofacialregion, axilla/chest, mediastinum,retroperitoneum, buttocks, and perineum, and were classically referred to as "cystic hygromas." Lymphatic malformations in the floor of the mouth and tongue present asmacroglossia, vesicles, and intermittent swelling. Lymphatic malformations may enlarge with infection.

Answer 55

The correct response is Option E. The most appropriate first step in management is sclerotherapy. First-line intervention for a large symptomatic venous malformation is sclerotherapy. Sclerotherapy is the injection of an inflammatory substance into a lesion which causes endothelial damage, fibrosis, and shrinkage of the malformation. Sclerotherapy is more effective and less morbid than resection. Propranolol and corticosteroids are treatment options for a problematic infantile hemangioma, but have no efficacy for vascular malformations. Embolization is first-line intervention for an arteriovenous malformation, and is not a treatment option for venous malformation. Resection is second-line therapy for a large problematic venous malformation. Extirpation can cause significant morbidity (i.e., bleeding, nerve injury, infection, wound breakdown). In addition, excision leaves a cutaneous scar and recurrence is common because a venous malformation can rarely be completely removed.

Answer 56

The correct response is D ## Footnote Spider angioma, or arteriolar malformation, is a small vascular malformation that contains a central blanching arteriole vessel. Spider angiomasoccur on the face in both children and adults. Obliteration of the central feeding arteriole is the goal of treatment; otherwise, the lesion will continue to enlarge. Laser therapy or electrocauteryis recommended. Macular stains are typically seen on the skin of neonates and are not true vascular nevi. Port-wine stains are capillary vascular malformations of the face that are seen at birth and grow commensurately with the child. Pyogenic granulomas are reparative vascular lesions commonly seen on the head and neck in children. Because these lesions receive their blood supply primarily from capillaries, they are not true vascular malformations. A strawberryhemangiomais a venous malformation that is typically seen at birth and enlarges during the first few months of life. These lesions do not grow commensurately with the child.

Answer 57

The correct response is C ## Footnote The most likely diagnosis is pseudoaneurysm, which has occurred secondary to trauma in the area of the radial artery. This patient sustained a stab wound to the forearm that immediately began to bleed. Although he controlled the bleeding at the time of injury, a pulsatile mass developed subsequently. Because obtaining a biopsy specimen can result in significant hemorrhage in these patients, it is important to carefully review preceding history, which is consistent with pseudoaneurysmin this situation. This patient’s history is not consistent with a true aneurysm, and examination findings rule out an arteriovenousfistula. Ulnar artery thrombosis is also known as hypothenarhammer syndrome because it occurs secondary to localized trauma in construction workers or other persons who strike objects with their hands. Typical symptoms include pain, paresthesia, decreased temperature, color changes, pallor, and numbness of the ulnarmostdigits. Vascular malformations are usually present at birth, but may develop and enlarge over time. A thrill or bruit is frequently associated, and color changes may occur.

Answer 58

The correct response is B ## Footnote This 3-year-old boy has findings most consistent with Parkes-Weber syndrome, an anomaly characterized by skeletal hypertrophy of one upper extremity with associated port-wine stains. Deep soft-tissue involvement, including capillary, lymphatic, and venous malformations, is also seen. Klippel-Trenaunaysyndrome is similar to Parkes-Weber syndrome but instead involves the lower extremities. Patients with Maffucci'ssyndrome have multiple enchondromas, most frequently affecting the hand, and venous malformations. The limbs are foreshortened. Approximately 20% of patients with Maffucci'ssyndrome will develop chondrosarcoma. Proteus syndrome manifests as partial gigantism of the extremities. Hemifacialhemihypertrophy, macrocephaly, macrodactyly, localized exostoses, subcutaneous lipomas, and vascular malformations are also associated. Patients with Rendu-Osler-Weber syndrome have multiple bright red telangiectasiasaffecting the face, fingertips, and mucosal surfaces of the gastrointestinal tract, bladder, and bronchial lining. Brain involvement can lead to the onset of seizures. Sturge-Weber syndrome is associated with a large vascular malformation, usually a port-wine stain, in the distribution of the first and second branches of the trigeminal nerve (V1 and V2). Other findings include vascular anomalies of the choroid plexus and leptomeninges, focal motor seizures, and visual field defects, especially glaucoma. Mental retardation may also be seen.

Answer 59

Correct answer is option D. The arteriogram in Figure 8 shows vascular occlusion in the thumb, index, ring and small. Considering the patient’s history of soft tissue induration, i.e., calcifications and telangiectasis, the patient has a diagnosis of CREST scleroderma. Although Raynaud’s phenomenon is a component of CREST, this patient does not have the typical Raynaud’s disease that affects young females and does not have the soft tissue calcifications. Buerger’s disease, or thromboangiitis obliterans, is associated with a history of heavy smoking. Hypothenar hammer syndrome is due to repetitive trauma to the ulnar artery at the wrist resulting in well defined filling defects in the superficial palmar arch of the hand. Although not well visualized in this patient, the superficial arch is narrowed showing no evidence of aneurismal dilatation.

Answer 60

The correct response is A ## Footnote Well-localized cutaneous hemangiomasrespond well to intralesionalinjections of corticosteroids. Large or endangering hemangiomasmay require a course of oral corticosteroids for four to six weeks with tapering over several months. Although there is a high success rate with this treatment, the most common complication is the short-term development of cushingoidfacies. One study found that 71% of patients developed this feature. Other short-term complications include personality changes (29%), gastric irritation (21%), oral or perinealfungal infections (6%), and a reversible diminished gain of height (35%) and weight (42%). Bacterial infections did not appear to be more common in this group of patients. Systemic corticosteroids can be given safely at doses of 2 to 3 mg/kg per day to treat appropriate hemangiomas, but minor and transient complications should be expected. Serious long-term complications are rare.

Answer 61

The correct response is A ## Footnote This 8-year-old boy has findings consistent with an arteriovenousmalformation, a congenital vascular soft-tissue anomaly that has extended to cause destruction of the underlying humerus. Arteriovenousmalformations are a subtype of vascular malformation involving arterial channels; they are present at birth and thus grow commensurately with the child's growth. Associated physical findings can include increased warmth in the area of the malformation, mass effect, the presence of a thrill or bruit on auscultation, ulceration, and distal ischemia, which is related to the "steal" phenomenon (ie, diversion of normal blood flow). Skeletal changes, especially intraosseousextension and lytic changes, occur in 34% of affected patients. Hemangiomasare also classified as vascular anomalies but instead appear shortly after birth and are characterized by a period of rapid growth (typically six to nine months) followed by slow involution. Rapid endothelial proliferation is characteristic, but skeletal changes are not, occurring in only 1% of patients. Lytic lesions are also not seen. Although hemangiomasare often unpleasant looking, they are typically only removed surgically if they begin to obstruct the airway or visual fields. Hereditary hemorrhagic telangiectasia, or Osler-Weber-Rendusyndrome, is an autosomal dominant disorder that first manifests following puberty and is characterized by lesions with ectaticvessels involving the skin, mucous membranes, and viscera that worsen over time. Epistaxis, hematuria, and hematemesis are frequently associated. Neurologic symptoms may occur in patients who have involvement of the central nervous system. Skeletal malformations are not associated. In patients with lymphatic malformations, skeletal hypertrophy and distortion are frequent. Venous malformations, especially those affecting the extremities, can cause hypoplasia and bone demineralization.

Answer 62

The correct response is A ## Footnote This 40-year-old man has findings most consistent with Klippel-Trenaunaysyndrome, characterized by a port-wine stain (typically involving one extremity) overlying venous and lymphatic malformations. Skeletal hypertrophy may also be seen; the lower extremity is most frequently involved. Patients with venous malformations may experience pain, ulceration, and ectasia; those with lymphatic malformations, such as this patient, typically report soft-tissue swelling and hypertrophy. This patient also has involvement of the trunk, which is rare. Parkes-Weber syndrome is similar to Klippel-Trenaunaysyndrome but is distinguished by the presence of arteriovenousfistulas. Rendu-Osler-Webersyndrome, or hereditary hemorrhagic telangiectasia, is an autosomal dominant disorder characterized by multiple ectaticvessels involving the skin, mucous membranes, and visceral organs. Epistaxis, hematuria, hematemesis, and melena are frequently associated. Sturge-Weber syndrome is associated with a large vascular malformation, usually a port-wine stain, in the distribution of the first and second branches of the trigeminal nerve (V1 and V2). Other findings include focal motor seizures, hemiparesis, and visual field defects, especially glaucoma. Mental retardation may result from leptomeningealvenous malformations. Patients with von Hippel-Lindaudisease have hemangiomasaffecting the retina, as well as hemangioblastomasof the cerebellum and visceral organs. Seizures and mental retardation may also be associated.

Answer 63

The correct response is E ## Footnote This patient most likely has a venous malformation, which is characterized by compressibility and a propensity to fill with blood when the patient changes body positions. It is usually larger and extends deeper than its superficial structure indicates, and it frequently intertwines with neurovascular components, which may affect the function of the involved extremity. When a venous malformation is associated with thrombosis, pain may result. Arteriovenousmalformations are high-flow lesions characterized by rapid blood flow through multiple feeding vessels. Pulsations and temperature changes in the affected area are associated, but dermal involvement is minimal. A bruit or thrill is heard on auscultation. Capillary malformations, or port-wine stains, have abnormalities limited to the capillaries in the dermis. These lesions are present at birth and do not regress spontaneously. If left untreated, cobblestoning, ectasia, and progressive darkening may occur. Patients with facial port-wine stains involving the first (ophthalmic) or second (maxillary) divisions of the trigeminal nerve (V1 and V2) are at significantly greater risk for ocular or nervous system involvement. Hemangiomastypically appear shortly after birth and proliferate for several months before spontaneously regressing. They are characterized by proliferative primitive endothelial cell nests in syncytial masses, with and without vessel lumens. Lymphatic malformations can be superficial or deep. Superficial lymphatic malformations are comprised of anomalous lymphatic remnants that manifest as clear vesicles. Deep lymphatic malformations appear similar to hemangiomasbut are soft and compressible on palpation. Bony overgrowth is associated.

Answer 64

The correct response is Option E. The lip is a favorable area for early surgical intervention for infantile hemangiomas and vascular malformations. The amount of lip tissue that can safely be resected allows for primary closure in the majority of cases. The tumors often distort the local tissues and are “additive” to the amount of available tissue, so resection typically allows for safe linear closure along the vertical resting tension lines. Reconstruction of other facial areas, such as the cheek and eyelids, is usually more complicated, and nonoperative measures may be a better first line of treatment. Surgical treatment is further warranted in this case by the functional impact of the lesion. Pulsed-dye lasers are effective treatment for thin vascular lesions. Multiple treatments are needed, they can be painful, and some residual scarring is usually left after resolution. Intralesional bleomycin is another option for local control, though multiple treatments, often with anesthesia, are required. Propranolol therapy has recently come to the forefront of hemangioma treatment. Oral treatment is preferred at 1 mg/kg three times daily. Dramatic responses are seen within 24 hours of starting treatment, and significant resolution occurs over a course of weeks. Contraindications may include bronchospasm, significant cardiac abnormalities, or cerebrovascular abnormalities. Pretreatment MRI and pediatric medical management may help determine suitability for treatment. Propranolol is most effective for early lesions that are in a proliferative growth phase. In this case, by 2 years, the lesion is likely done with growth and in a stable to involuting phase. Oral prednisolone is also effective at treating hemangiomas and is a common treatment option. Hyperglycemia and cushingoid features are temporary drawbacks. Given the symptoms in this case and the relative ease of surgical resection, medical therapies such as steroids and beta blockers would be secondary choices. In asymptomatic patients with minimal disfigurement, observation alone may be the preferred first line of treatment.

Answer 65

Correct answer is option B. Because of the presence of life-threatening injuries, the further stabilization of this patient is imperative before any operative procedure is performed. The patient has been intubated, is being mechanically ventilated and is receiving intravenous fluids for resuscitation. Examination of the foot shows no pulse and no reflexes, indication nerve damage; the foot is cold and the vasculart supply has been compromised. According to the Gustillo classification, s severe open fracture of the tibia with associated neurologic injury is classified as grade IIIC. Once the patient has been completely stabilized, a below-knee amputation can be performed. Revascularization and reconstruction using a free flap is appropriate for patients with grade IIIC fractures without associated nerve injuries or life-threatening complications.

Answer 66

The correct response is E ## Footnote The patient described has a hemangioma. The most appropriate management of a hemangiomaon the cheek is observation. After a rapid growth phase, most of these lesions proceed to involution and disappear completely by puberty. Complications may occur such as ulceration, bleeding, or infection. Lesions that obstruct vision or the airway may require more aggressive treatment, such as injection or oral administration of a corticosteroid. Laser therapy is not effective in the treatment of hemangiomas. Incisional biopsy is only necessary if a malignancy is suspected.

Answer 67

The correct response is C The patient described has an acquired arteriovenousfistula after penetrating trauma, which represents an abnormal connection between an artery and vein. The affected area is usually enlarged, warm, and has distended and often pulsatile superficial veins. A thrill can be palpated over the fistula, and a bruit may be present. In severe cases, altered hemodynamics may cause heart failure if a significant portion of the cardiac output is diverted through the fistula. The Branham sign indicates slowing of the heart rate upon compression proximal to the arteriovenousmalformation. The next appropriate step is a magnetic resonance angiogram of the affected wrist to delineate the nature and extent of the fistula. It also helps rule out traumatic pseudoaneurysmand helps plan for the subsequent surgical procedure. Comparative plethysmographywith the contralateral limb is typically used to quantify flow in an entire limb, but assessing blood flow through localized areas is difficult. Ligation of feeding vessels without surgical resection will result in rapid recruitment of collateral vessels and is contraindicated. Percutaneous biopsy is contraindicated in pulsatile lesions. Observation in a case of an enlarging pulsatile lesion is not appropriate.

Answer 68

The Correct response is E ## Footnote Sturge-Weber syndrome is characterized by capillary malformations in the distribution of the ophthalmic or the maxillary division of the trigeminal nerve. It may be unilateral or bilateral. Often there is also gradual enlargement and hypertrophy of the cheek, lip, and maxilla, and occasionally there is gradual enlargement and hypertrophy of the mandible. MRI may show additional vascular anomalies of the leptomeningesand choroid plexus. Cobb syndrome consists of a capillary malformation in the midline scalp region overlying an encephaloceleor in the skin posterior to an area of dysraphismin the cervical or lumbosacral spine. Fibrous dysplasia is an overgrowth syndrome of bones that is caused by abnormal proliferation of bone-forming mesenchyme. It does not involve soft-tissue hypertrophy nor is it associated with vascular malformations. Albright syndrome is a specific variety of polyostoticfibrous dysplasia and includes endocrine abnormalities and cafe-au-laitspots. Klippel-Trenaunaysyndrome is a capillary-lymphatic-venous malformation typically involving hypertrophy of the extremities and sometimes the thorax of one side of the body. It does not involve the head and neck. The skin surface shows deep red staining with hemolymphaticvesicles. A pathognomonic feature of this condition is the presence of the embryonallateral vein of Servellein the lower extremity. Parkes-Weber syndrome is similar to Klippel-Trenaunaybut is confined to an upper or lower extremity. Overgrowth of the extremity is characteristic along with microscopic arteriovenous fistulas, and unlike Klippel-Trenaunay, lymphatic anomalies are rare. It does not involve the head or neck. Nevus flammeusneonatorumis an entity that behaves more like a hemangiomaand not a vascular malformation in that it is usually gone or faded by 1 year of age. It is typically located on the upper face and posterior neck but is not associated with soft-tissue or bony hypertrophy.

Answer 69

The correct response is E ## Footnote Although conservative management is usually proposed for hemangiomasoccurring in infancy, the presence of these tumors on the face may result in severe complications and may provide an indication for treatment. Hemangiomasthat cause functional disturbances or serious psychological distress deserve surgical excision before the age of expected spontaneous regression; surgery can provide active treatment with excellent results and minimal morbidity. Severe complications can be encountered, including visual occlusion, repeated bleeding, and distortion of adjacent structures. Early surgery, often before 2 years of age, is recommended in the management of large periocularhemangiomas, to prevent secondary amblyopia, and in the management of proliferative labial tumors, which are prone to bleeding and to cause difficulty while eating. Early surgical management is also recommended for nasal-tip hemangiomas, which regress very slowly and may result in severe distortion of the cartilaginous framework. The operative technique depends on the location and size of the lesion and focuses on resection of the tumor and reconstruction of the adjacent structures when necessary. Postoperative outcomes can be very satisfactory. In this patient, oral corticosteroids would not be recommended, and both pulsed-dye laser therapy and sclerotherapywould not be efficacious treatment modalities for a lesion of this magnitude.

Answer 70

The correct response is Option C. Although the hemangioma shown is sizable, judicious mobilization of the scalp and galeal scoring allow the resultant defect to be easily closed without resorting to delayed rotational flaps, tissue expanders, or skin grafting. Single-staged excision and linear closure is the simplest, most predictable manner of closing small to intermediate-sized scalp defects such as the one in the patient described. The maximum defect using this technique is not well defined, but at least one series has demonstrated successful primary closure of defects in infants of up to 7 cm without using the other alternatives listed or tissue expansion. Delaying the resection for another 2 years might be productive in a younger child, but further involution is unlikely in a 4-year-old. Staged excision is possible, but unnecessary for this lesion; moreover, repair along the edge of the friable hemangioma can lead to wound problems.

Answer 71

Correct answer is option D. The plantaris muscle and tendon of the lower extremity comprise a vestigial muscle tendon complex that is analogous to the Palmaris muscle and tendon of the upper extremity. The plantaris muscle may be harvested easily through the use of a tendon stripper inserted at the medial ankle. A tendon of approximately 25 to 35 centimeters may be harvested. The plantaris muscle belly is found in the superficial posterior muscle compartment along with the gastrocnemius and soleus muscles. The plantaris muscle rises from the lower lateral supracondyla line of the femur immediately above the lateral head of the gastrocnemius muscle and from the oblique popliteal ligament. It is a short (10 cm) muscle belly and the tendon runs between the gastrocnemius and soleus muscles and then along the medial border of the calcaneal tendon to insert into the calcaneous and plantar fascia.

Answer 72

The correct response is D In this 8-year-old girl who has swelling of the calf, an MRI of the leg should be obtained to delineate the extent of the lesion. The diffuse swelling indicates that the lesion is most likely an arteriovenousmalformation (AVM) and not a single arteriovenousfistula. AVMs are composed of multiple arterial feeding vessels, microarteriovenousand macroarteriovenousfistulas, and enlarged veins. Operative management of AVMs is typically undertaken only in the presence of serious signs or symptoms such as ischemic pain, recalcitrant skin ulceration, bleeding, or increased cardiac output. In patients with nonsymptomaticAVMs, surgery may be considered as long as resection can be achieved without complication. Sclerosingagents are typically injected preoperatively to decrease the size of the lesion and minimize intraoperative bleeding; however, this does not lessen the extent of the excision. Because ligation of the feeding vessels causes rapid recruitment of nearby vessels, it is contraindicated.

Answer 73

The correct response is C ## Footnote Because treatment of lymphatic malformations typically centers on relief of symptoms, including obliteration of lymphatic bullae, the erbium laser, with a wavelength of 2910 nm, is most appropriate. Lymphatic malformations are frequently larger than their skin manifestations would indicate; the intradermal and subcutaneous vessels are often extensive. The aforementioned bullae tend to drain intermittently, causing chronic infection. By eliciting an inflammatory reaction, the erbium laser causes scarring within the superficial dermis, resulting in obliteration of superficial channels and temporary relief of symptoms. The alexandrite laser (755 nm) is indicated for removal of hair, blue and green tattoo pigments, and some pigmented lesions; the diode laser (810 nm) is used similarly. The 532-nm Nd:YAGand 585-nm pulsed dye lasers are appropriate for ablation of small vascular lesions; the 1064-nm Nd:YAGlaser is recommended for removal of tattoo pigments and other dermal pigments. Because these lasers do not penetrate to the depths of the erbium laser, they are less likely to cause an inflammatory reaction and to result in obliteration of superficial channels.

Answer 74

The correct response is Option B. Infantile hemangiomas are the most common benign tumors in infancy. Their clinical course is characterized by a period of rapid growth over 6 to 12 months (proliferative phase), followed by slow involution over the subsequent 1 to 5 years. In the absence of visual, nasolaryngeal, and auditory canal obstruction, conservative/medical management is recommended. Recently, the efficacy of propranolol, a nonselective beta-adrenergic blocker, has been demonstrated in treating hemangiomas and has established itself as a first-line treatment option. Adverse effects, however, include bradycardia, hypoglycemia, and electrolyte imbalances, notably hyperkalemia. Hypercalcemia, hypermagnesemia, hypernatremia, and hyperphosphatemia have not been associated with the administration of propranolol.

Answer 75

The correct response is A ## Footnote Kasabach-Merritt syndrome is a disorder characterized by profound thrombocytopenia associated with a solitary hemangiomaor diffuse hemangiomatosis. Capillary malformations, or port-wine stains, are not seen. Systemic heparin should not be administered to patients with Kasabach-Merritt syndrome because of the associated risk for exacerbation of bleeding diatheses. Instead, appropriate management includes embolization or compression of lesions, interferon therapy, radiation therapy, and systemic administration of corticosteroids. All of the other syndromes are associated with port-wine stains. Klippel-Trenaunaysyndrome is characterized by a port-wine stain, typically involving one extremity, overlying venous and lymphatic malformations. Skeletal hypertrophy may also be seen. Although patients with Parkes-Weber syndrome have findings similar to Klippel-Trenaunaysyndrome, arteriovenousfistulas are a distinguishing feature. Port-wine stains are most frequently seen in conjunction with Sturge-Weber syndrome. The malformation usually occurs in the distribution of the first and second branches of the trigeminal nerve (V1 and V2), and is associated with focal motor seizures, hemiparesis, and visual field defects, especially glaucoma. Mental retardation may result from leptomeningealvenous malformations.

Answer 76

The correct response is D

Answer 77

The correct response is C ## Footnote Arteriovenousmalformations are structural anomalies that develop during the fourth through sixth weeks of gestation secondary to inhibited development of the capillary network. These malformations are typically present at birth but may be seen initially in persons of all ages. Hormonal changes, such as those seen in puberty and adolescence, can result in progression of the lesion; its growth is thought to be caused by increased blood flow and expansion and not by cellular proliferation. Although angiography has long been the standard for diagnosis of vascular lesions, defining their vascular anatomy and flow characteristics, this technique does not adequately delineate the involvement of the surrounding soft tissues. Thus, MRI and magnetic resonance angiography (MRA) are currently recommended as noninvasive means of determining both the extent of the lesion and its flow characteristics. Because contrast enhancement and ionizing radiation are unnecessary, both MRI and MRA can be obtained in the same setting. Angiography is still reserved for delineation of large, highly vascular lesions that require preoperative embolization. CT scan is used for bony evaluation but is now secondary to MRI as the diagnostic procedure of choice. PET scan is not effective in evaluating vascular malformations. Doppler ultrasonography may be performed in conjunction with MRI to determine the lesion's flow characteristics but is not used alone.

Answer 78

Correct Answer: D. Catheter-based arteriography allows both diagnosis and treatment of small vessel occlusions in the hand. Arterial thrombosis of the hand occurs infrequently but can lead to significant loss of hand function. Treatment options depend on the history and clinical findings. Appropriate care almost always requires coordination with interventional radiologists. Doppler ultrasonography can be used to evaluate flow all the way out to the digital arteries. The lack of a biphasic signal suggests arterial occlusion but cannot document the area of occlusion. Another limitation of ultrasonography is operator expertise. Laser Doppler can be useful for monitoring local perfusion of flaps. CT angiography is often the first imaging modality performed for patients with limb ischemia due to faster scan times; however, it cannot show much detail beyond the wrist and is rarely useful in visualizing the superficial arch or digital vessels. MR angiography (MRA) has the advantage of not requiring ionizing radiation or the use of contrast agents, but it is time-consuming and requires significant expertise to obtain quality images. Traditionally, the spatial resolution limits of MRA restrict visualization of arteries 1.0 mm or less in diameter. Nonetheless, MRA may be indicated for patients with contraindications to CT angiography or catheter-based arteriography. Catheter-based arteriography is the gold standard for vascular imaging of the smaller vessels of the hand and may allow the interventionalist to treat with catheter-directed thrombolysis or percutaneous mechanical thrombectomy. Angiography also can evaluate the entire limb to rule out proximal sources of emboli and provides the surgeon with the best road map for surgical intervention, if required. **References** 1. Shankar DS, Rybalko DA, Goldman D, Nowakowski FS, Hausman MR. Applications of vascular imaging and interventional radiology modalities in the upper extremity: a review. *J Hand Surg Am.* 2022;S0363-5023(22)00575-5. doi:10.1016/j.jhsa.2022.09.016 2. Iannuzzi NP, Higgins JP. Acute arterial thrombosis of the hand. *J Hand Surg Am.* 2015;40(10):2099-2106. doi:10.1016/j.jhsa.2015.04.015 3. Islam A, Edgerton C, Stafford JM, et al. Anatomic findings and outcomes associated with upper extremity arteriography and selective thrombolysis for acute finger ischemia. *J Vasc Surg.* 2014;60(2):410-417. doi:10.1016/j.jvs.2014.02.005

Answer 79

Correct Answer B. Amplified alpha-2 adrenoceptor activity leading to excessive sympathetic vasoconstriction. The patient presents with classic primary Raynaud findings. While relatively common and not associated with underlying systemic disease, the symptoms of primary Raynaud disease can have a detrimental effect on quality of life. Such patients have overactive smooth muscle alpha-2 adrenoceptors, leading to amplification of sympathetic vasoconstriction in response to normal norepinephrine release at decreased temperatures. Options for treatment include chemical sympathectomy with botulinum toxin type A injections. Botulinum toxin type A can prevent sympathetic vasoconstriction seen in patients with Raynaud disease. Endothelin-1 is found circulating in higher levels in patients with secondary Raynaud phenomenon, which is seen most commonly in patients with connective tissue disorders but can also be found in patients with obstructive vascular disease, certain drug exposures, viral infections, and occupational exposures. Antiphospholipid antibodies may be seen in connective tissue disorders and are associated with increased risk for thrombosis. Delta receptors, also called delta opioid receptors, are found in the brain, and their activation leads to analgesia. They are not implicated in Raynaud phenomenon. References 1. Thibaudeau S, Serebrakian AT, Gerety PA, Levin LS. An algorithmic approach to the surgical treatment of chronic ischemia of the hand: a systematic review of the literature. Plast Reconstr Surg. 2016;137(5):818e-828e. doi:10.1097/PRS.0000000000002044 2. Beek AL, Lim PK, Gear AJ, Pritzker MR. Management of vasospastic disorders with botulinum toxin. Plast Reconstr Surg. 2007;119(1):217-226. doi:10.1097/01.prs.0000244860.00674.57 3. Wigley FM, Flavahan NA. Raynaud’s phenomenon. N Engl J Med. 2016;375(6):556-565. doi:10.1056/NEJMra1507638 4. Curtiss P, Svigos K, Schwager Z, Lo Sicco K, Franks AG Jr. Part I: epidemiology, pathophysiology, and clinical considerations of primary and secondary Raynaud’s phenomenon. J Am Acad Dermatol. 2022;S0190-9622(22)02250-2. doi:10.1016/j.jaad.2022.06.1199

Answer 80

Correct answer is option D. The patient has signs and symptoms consistent with hypothenar hammer syndrome. Hypothenar hammer syndrome results from repeated trauma to the ulnar artery at the hook of hamate bone. This can lead to ulnar artery aneurysm, with possible formation of a palpable mass, thrombosis, and distal embolization to the fingers. Patients may also experience pain, cyanosis, pallor, coolness, and recurrent episodes of vasospasm. The condition is more common in men than women, typically occurs in the fourth and fifth decade of life, and is more likely to affect the dominant hand in certain professions like carpenters, builders, and other physical laborers working with hammers and vibrating tools. Angioplasty and stenting are typically not done in the hand. Catheter-directed thrombolysis can address intermittent clots in the acute setting but would not resolve the underlying problem of aneurysmal dilation. Digital sympathectomy has been suggested to alleviate vasospastic conditions, but it would not be adequate to treat the embolic consequences of ulnar artery aneurysm in hypothenar hammer syndrome. Ligation of the ulnar artery alone is a form of treatment for hypothenar hammer syndrome, but it is typically not done unless there is better blood flow (digital brachial index greater than 0.7). In this patient with a digital brachial index of 0.6, the relative ischemia would suggest restoration of ulnar artery flow with repair or graft instead of ligation alone. Mechanical compression and thrombin injection is a method of treatment for pseudoaneurysms, but it would not be indicated in the management of hypothenar hammer syndrome. References: 1. Endress RD, Johnson CH, Bishop AT, Shin AY. Hypothenar hammer syndrome: long-term results of vascular reconstruction. J Hand Surg Am. 2015;40(4):660-665.e2. doi:10.1016/j.jhsa.2015.01.014 2. Fox PM, Watt AJ. Vascular disorders of the upper extremity. In: Weiss APC, Budoff JE, Calfee RP, et al, eds. The American Society for Surgery of the Hand Textbook of Hand and Upper Extremity Surgery. 2nd ed. American Society for Surgery of the Hand; 2019:1939-1951. 3. Hui-Chou HG, McClinton MA. Current options for treatment of hypothenar hammer syndrome. Hand Clin. 2015;31(1):53-62. doi:10.1016/j.hcl.2014.09.005 4. Kitzinger HB, van Schoonhoven J, Schmitt R, Hacker S, Karle B. Hypothenar hammer syndrome: long-term results after vascular reconstruction. Ann Plast Surg. 2016;76(1):40-45. doi:10.1097/SAP.0000000000000562 5. Yuen JC, Wright E, Johnson LA, Culp WC. Hypothenar hammer syndrome: an update with algorithms for diagnosis and treatment. Ann Plast Surg. 2011;67(4):429-438. doi:10.1097/SAP.0b013e31820859e1

Answer 81

Correct Answer: C. Administer intravenous antibiotics and admit for airway monitoring. Rationale: Lymphatic malformations are at risk for infections, which present as sudden enlargement, erythema, pain, and leukocytosis (or left shift), with or without fever. Any sudden enlargement should be treated as infection unless proven otherwise. In the cervicofacial area, the sudden enlargement could put the airway at risk and, even with a tracheostomy, the patient should be admitted. Intravenous antibiotics are the treatment of choice. Decompression will result in rapid reaccumulation of fluid. There is no indication for sclerotherapy when the lesion is actively infected and may cause seeding/skin infection/breakdown. Sirolimus should be held when there is an active infection, so this is definitely not the time to start if the patient is not already on it. The patient needs to be treated with antibiotics, and pain control treatment alone may miss an infection that can progress to sepsis quickly. References: Gao Q, Chen H, Sun B, Cui J, Shen W. Intermittent administration regimen of sirolimus for refractory cervicofacial lymphatic malformation. J Craniofac Surg. 2022;33(3):850-854. doi:10.1097/SCS.0000000000008063 Colbert SD, Seager L, Haider F, Evans BT, Anand R, Brennan PA. Lymphatic malformations of the head and neck-current concepts in management. Br J Oral Maxillofac Surg. 2013;51(2):98-102. doi:10.1016/j.bjoms.2011.12.016 Tian R, Liang Y, Zhang W, et al. Effectiveness of sirolimus in the treatment of complex lymphatic malformations: single center report of 56 cases. J Pediatr Surg. 2020;55(11):2454-2458. doi:10.1016/j.jpedsurg.2019.12.021

Answer 82

The correct answer is option C. **Capillary malformations/port-wine stains** affect 0.3% of newborns and are most frequently found on the face. They can exist anywhere on the body and incidence has a female-to-male ratio of 3:1. **Klippel-Trénaunay** syndrome is associated with a patchy port-wine stain on an extremity overlying a deeper venous and lymphatic malformation with associated skeletal hypertrophy. **Parkes-Weber** syndrome is similar to Klippel-Trénaunay syndrome, but it is distinguished by the presence of an arteriovenous fistula on MRI. **Sturge-Weber** syndrome is associated with a large facial port-wine stain involving the V1 and V2 trigeminal (V) nerve distribution. This is usually associated with leptomeningeal venous malformations and frequent mental retardation. Telangiectases of the skin and mucous membranes, epistaxis, and a positive family history make up the classic triad of **Rendu-Osler-Weber** syndrome.

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Vascular Tumors And Malformations - Vascular Injury Hand & Lower Extremities Flashcards

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