Hemangiomas

Question 1

An 18-month-old boy has a 25-cm pigmented lesion on his back. Which of the following is the most appropriate management?
(A) Observation with photographic mapping
(B) Intralesional injection of interferon gamma
(C) Dermabrasion
(D) Tunable dye laser ablation
(E) Excision

Answer 1

(E) Excision

This 18-month-old boy has a giant congenital nevus on the back. Congenital nevi can be classified as “giant” according to several criteria, including those lesions that are larger than 20 cm in diameter, lesions that are greater than twice the size of the patient’s palm, and those nevi for which excision and primary closure cannot be performed as a single procedure. Because of the potential for malignant transformation, surgical excision of the entire lesion is recommended. Although the actual risk for melanoma is controversial, one study reported that approximately 8% of patients with giant congenital nevi developed melanoma during the first 15 years after the initial appearance of the nevus.

Question 2

How are congenital nevi determined to be “giant”?

Answer 2

Although the actual risk for melanoma is controversial, one study reported that approximately 8% of patients with giant congenital nevi developed melanoma during the first 15 years after the initial appearance of the nevus.

Question 3

Recommended treatment for giant congenital nevus

Answer 3

Excision - Although the actual risk for melanoma is controversial, one study reported that approximately 8% of patients with giant congenital nevi developed melanoma during the first 15 years after the initial appearance of the nevus

Question 4

A 40-year-old man has a dark purple "cobblestone" lesion covering the entire right cheek. This finding is most consistent with 
(A) an arteriovenous malformation
(B) a capillary malformation
(C) a hemangioma
(D) a lymphatic malformation
(E) a venous malformation

Answer 4

(B) a capillary malformation

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, cobblestoning and progressive darkening may occur due to ectasia of the vessels within the capillary malformation.

Question 5

Where are port-wine stains usually seen

Answer 5

Abducens (VI) and facial (VII) nerves.

Question 6

What happens if capillary malformations are left untreated?

Answer 6

If left untreated, cobblestoning and progressive darkening may occur due to ectasia of the vessels within the capillary malformation.

Question 7

Arteriovenous malformation

Answer 7

An arteriovenous malformation is a high-flow lesion often characterized by a palpable thrill or bruit.

Question 8

Branham sign

Answer 8

Compression of an arteriovenous malformation results in occlusion of the associated arteriovenous fistula, 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.

Question 9

Hemangioma

Answer 9

Hemangiomas are vascular tumors that appear just after birth and rapidly enlarge during the first year of life.

Question 10

What eventually happens after recurrent scarring of a lymphatic malformation?

Answer 10

Recurrent swelling can lead to scarring, which will significantly decrease the size of the lesion

Question 11

Cystic hygroma

Answer 11

Lymphatic malformation (another word)

Question 12

Where do cystic hygromas most often occur?

Answer 12

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.

Question 13

Venous malformation

Answer 13

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.

Question 14

A 4-month-old male infant is brought to the office because of a rapidly enlarging mass in the eyebrow region. The patient’s mother says she first noticed the lesion at 1 month of age and that it was not present at birth. A photograph is shown. Treatment with administration of propranolol is planned. Which of the following adverse effects is most likely in this patient?

A) Adrenal failure
B) Decrease in height
C) Hypertension
D) Hypoglycemia
E) Spastic diplegia

Answer 14

D) Hypoglycemia

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.

Question 15

A 3-year-old boy is brought to the office because of recurrent swelling, discoloration, and occasional discomfort of the right forearm. Discoloration and swelling have been present since birth, but these features have become more pronounced with growth and are exacerbated by limb dependency. A photograph is shown. Which of the following is the most appropriate initial treatment in this patient?
A) Interferon alpha therapy
B) Propranolol therapy
C) Reassurance that the condition will resolve with time
D) Sclerotherapy
E) Systemic corticosteroid therapy

Answer 15

D) Sclerotherapy

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.

Question 16

A 7-year-old boy is evaluated because of capillary malformations in the right leg and thigh, which have been present since birth. Physical examination shows venous varicosities over the anterior leg and medial thigh. Girth of the affected lower limb is increased compared with the unaffected limb. Duplex imaging discloses no sign of arteriovenous fistulae. This patient is at greatest risk for which of the following conditions?
A) Chondrosarcoma
B) Disseminated intravascular coagulation
C) High-output cardiac failure
D) Limb-length discrepancy
E) Pathologic fracture

Answer 16

D) Limb-length discrepancy

The presence of capillary malformations, varicosities, and limb hypertrophy paired with the absence of arteriovenous (AV) fistulae is classic for Klippel-Trénaunay syndrome. Up to 67% of patients with Klippel-Trénaunay syndrome will exhibit limb-length discrepancy, most commonly with the affected limb being hypertrophic.

Pathologic fractures may occur through enchondromas found in Maffucci syndrome. Since the lesions have been present since birth and the child is young, presentation is less consistent with Maffucci syndrome, which presents in adolescence in roughly 80% of patients. These enchondromas also hold a 15 to 30% risk for malignant transformation to chondrosarcoma.

High-output cardiac failure is a feature of vascular malformations that include AV fistulae; the classic example is Parkes-Weber syndrome.

Disseminated intravascular coagulation can result in life-threatening thrombocytopenia. Referred to as Kasabach-Merritt syndrome, this finding is more common in abnormalities such as kaposiform hemangioendothelioma.

Question 17

An otherwise healthy 4-year-old boy is brought to the office for evaluation of a large scalp hemangioma. A photograph is shown. The family has requested removal of the hemangioma before he enters school. Which of the following is the most appropriate management in this patient?

A) Deferral of surgery for 2 years
B) Excision and skin grafting
C) Single-stage excision and closure
D) Staged excision and closure
E) Tissue expansion

Answer 17

C) Single-stage excision and closure

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.

Question 18

A 22-year-old woman comes to the office because of oral bleeding and a 20-year history of a radiologically defined arteriovenous malformation. Embolization and resection of the tumor are planned. Which of the following factors is most likely to be associated with an increased risk for recurrence?
A) Enlargement of the malformation
B) Patient age
C) Patient gender
D) Quiescence of the malformation
E) Ulceration

Answer 18

E) Ulceration

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 III lesion, which is the highest 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.

Question 19

A 12-year-old girl has a 7 × 7-cm venous malformation of the thigh that is slowly enlarging and causing pain. The lesion involves the skin, subcutaneous tissue, and muscle. Which of the following is the most appropriate first step in management?
A) Corticosteroid therapy
B) Embolization of the lesion
C) Propranolol therapy
D) Resection of the lesion
E) Sclerotherapy

Answer 19

E) Sclerotherapy

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.

Question 20

An 8-month-old male infant is evaluated for a lip mass. A photograph is shown. Treatment with propranolol is initiated. Which of the following adverse effects is most important to monitor?
A) Drooling
B) Hypertension
C) Hypoglycemia
D) Lethargy
E) Tachycardia

Answer 20

C) Hypoglycemia

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.

Question 21

A male newborn has a large macrocystic lymphatic malformation of the neck. Which of the following is the most appropriate first step in management?
A) Embolization of the lesion
B) Prednisolone therapy
C) Propranolol therapy
D) Resection of the lesion
E) Sclerotherapy

Answer 21

E) Sclerotherapy

The most appropriate first step in management is sclerotherapy. Lymphatic malformation is a type of vascular anomaly that results from aberrant formation of lymphatic vessels. Lymphatic malformation most commonly affects the neck and axilla. There are two major types of lymphatic malformations: macrocystic and microcystic. Macrocystic lesions have cysts large enough to be cannulated by a needle and treated with sclerotherapy. Microcystic lesions have cysts that are too small for treatment with sclerotherapy. First-line management of macrocystic lymphatic malformations is sclerotherapy, which is the injection of an inflammatory substance into the lesion that causes scarring of the cyst walls together and shrinkage of the malformation. The most commonly used sclerosants are doxycycline, sodium tetradecyl sulfate, and ethanol.

Prednisolone and propranolol are drugs used to treat problematic proliferating infantile hemangioma, and have no efficacy for vascular malformations. Embolization is used to treat arteriovenous malformations and involves delivering a substance through an artery to occlude blood flow.

Resection is second-line therapy for a problematic macrocystic lymphatic malformation. Extirpation can cause marked morbidity (i.e., bleeding, nerve injury, infection, wound breakdown). In addition, excision leaves a cutaneous scar and recurrence is likely because a lymphatic malformation can rarely be completely removed. Resection is considered if a lesion remains symptomatic following sclerotherapy, or for microcystic lymphatic malformations that cannot be sclerosed.

Question 22

An otherwise healthy 68-year-old man comes to the office because of an enlarging mass over the right ear for the past 30 years. A photograph is shown. He had no prior treatment of the mass. MRI confirms the diagnosis of arteriovenous malformation. In the past 3 months, he had bleeding from the mass requiring hospital admission and blood transfusions. Bleeding is controlled after prolonged direct pressure. Which of the following is the most appropriate management for this lesion?
A) Corticosteroid therapy
B) Laser therapy
C) Resection
D) Sclerotherapy
E) Observation

Answer 22

C) Resection

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.

Question 23

A 7-month-old male infant is referred because of the lesion shown in the photograph. Ophthalmologic examination shows mild astigmatism, for which he was being treated with corrective lenses. No amblyopia is noted. Which of the following is the most appropriate next step in management?
A) Administration of propranolol 2 mg/kg/day in divided doses
B) Magnetic resonance angiography of the brain
C) Pulsed-dye laser (595 nm) therapy
D) Surgical resection
E) Observation only

Answer 23

A) Administration of propranolol 2 mg/kg/day in divided doses

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).

Question 24

A 2-year-old girl is evaluated because of a rapidly enlarging mass in the left forearm that her parents first noticed 6 months ago. Physical examination shows a 3-cm firm mass in the volar aspect of the left forearm. Passive extension of the fingers and wrist elicits pain. MRI shows a mass within the deep forearm compartment suggestive of a vascular lesion. Which of the following is the most appropriate initial step in treatment?
A ) Application of a compression garment
B ) En bloc surgical resection
C ) Oral administration of prednisone
D ) Percutaneous sclerotherapy
E ) Pulsed-dye laser therapy

Answer 24

C ) Oral administration of prednisone

This rapidly growing vascular lesion, not present at birth, most likely represents an intramuscular hemangioma. These vascular anomalies can pose diagnostic challenges because of their depth and lack of cutaneous changes characteristic of the more common infantile hemangioma. The treatment algorithm, however, parallels that of other hemangiomas. In this case, the first line of therapy would be medical treatment with either propranolol or corticosteroids.

Compression garments may be useful in managing arteriovenous or lymphatic malformations of the extremities. In this patient, with pain caused by increased intracompartmental volume, compression may exacerbate the symptoms.

En bloc surgical resection is reserved for lesions that fail to respond to medical management or that compromise function. In the case of upper extremity lesions, this would be indicated most commonly for refractory pain or nerve compression.

Percutaneous sclerotherapy can successfully treat low-flow arteriovenous malformations and lymphatic malformations. It would not be a first-line intervention for hemangioma.

Cutaneous arteriovenous malformations may be treated with pulsed-dye laser or other light-based therapies. Deep, intramuscular lesions without a cutaneous component will not be reached by these interventions.

Question 25

A 5-year-old boy is brought to the office because of a pulsatile mass in the volar forearm. His parents say the mass first appeared 10 days ago. Physical examination shows a palpable, pulsatile mass at the radial artery. CT angiography shows a true aneurysm of the radial artery. Which of the following is the most likely cause of this patient’s condition?
A ) Arterial catheterization
B ) Crush injury
C ) Infective embolism
D ) Kawasaki disease
E ) Regional block with administration of a local anesthetic agent

Answer 25

D ) Kawasaki disease

There are two types of pathologic arterial dilations: ?false,? or pseudoaneurysms, and true aneurysms.

Pseudoaneurysms typically result from some form of acute external or internal insult that causes rupture or laceration of the endothelium and bleeding. All layers of the artery can be involved, or it can be due to a dissection between layers. The false or pseudoaneurysm is caused by an acute injury to the endothelial layer of an otherwise normal artery, subsequent bleeding, clotting, and recanalization of the vessel. These arterial cul-de-sacs do have blood in them, but they do not have endothelial layers internally. Therefore, they are described as ?false? aneurysms.

Therefore, Options A, B, C, and E are not appropriate because those types of acute insults more typically cause pseudoaneurysms. A true aneurysm is an endothelial-lined widening of an artery that has all three layers of the vessel lining the aneurysm: endothelium, media, and adventitia. The true aneurysm typically has a more fusiform, dilated appearance, rather than the outpouching that typifies false aneurysms. True aneurysms can also result from trauma, but the trauma tends to be repetitive and blunt, rather than an acute, severe insult and a vessel rupture.

Kawasaki disease is a classic example of true aneurysm. In this disorder, arteriosclerosis is a common cause of true aneurysms. Hemophilia can lead to true and false aneurysms.

Question 26

A 2-year-old boy is brought to the office because of an infantile hemangioma of the right lower lip. His parents say that he frequently drools and has difficulty in phonation. On examination, the lesion measures 1 cm in width and distorts the surrounding tissues. A photograph is shown. Which of the following is the most definitive treatment?
(hemangioma)
A) Bleomycin
B) Oral prednisolone
C) Propranolol
D) Pulsed-dye laser therapy
E) Surgical resection

Answer 26

E) Surgical resection

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.

Question 27

A 7-month-old female infant is brought to the office because of a large, rapidly growing vascular tumor of the mandible. Physical examination shows a reddish purple lesion with a centrifugally advancing rim of ecchymosis. Initial platelet count is below 10,000/mm3. Administration of which of the following is the most appropriate initial treatment?
A) Imiquimod
B) Interferon alfa-2a
C) Prednisone
D) Propranolol
E) Vincristine

Answer 27

E) Vincristine

Kaposiform endothelioma is a malignant vascular tumor which is often associated with Kasabach-Merritt phenomenon. It is generally seen in infancy. It can be seen in the trunk and extremities. The skin overlying the tumor is deep red-purple, tense, and shiny, which is pathognomonic for kaposiform endothelioma. These patients typically have profound thrombocytopenia. As this is a malignant tumor, the primary mode of therapy is chemotherapy, sometimes combined with surgical resection. Vincristine is considered first-line therapy and has supplanted interferon alfa-2a due to its efficacy and decreased risk of major side effects. Interferon alfa-2a has a high risk of spastic diplegia, which is irreversible.

Corticosteroids are not considered a first-line therapy for kaposiform endothelioma.

Propranolol and imiquimod are not yet widely studied in kaposiform endothelioma.

Question 28

An otherwise healthy 3-month-old female infant is admitted to the pediatric intensive care unit because of progressive difficulty breathing for the past 3 weeks. Examination shows mild stridor. Cultures are negative for acute viral or pulmonary illness. A photograph is shown. (infantile hemangioma) Administration of which of the following is the most appropriate treatment?
A) Imiquimod
B) Interferon alfa-2a
C) Propranolol
D) Vincristine

Answer 28

C) Propranolol

In the absence of other pulmonary disease, the stridor is most likely being caused by the hemangioma which is present in the beard distribution. Hemangiomas can be present in the airway as well as the surrounding soft tissue.

In some centers, propranolol has supplanted corticosteroids as the first-line medical therapy for complicated hemangiomas. Currently, there is no universally accepted protocol for propranolol administration. Propranolol is initially given intravenously and then converted to oral dosing. Blood pressure and heart rate monitoring must be done on an outpatient basis, generally by a pediatric cardiologist. Treatment is typically 2 to 6 months.

Systemic corticosteroids are still considered effective therapy. Corticosteroids generally require prolonged therapy to prevent rebound effect. It can take upwards of 2 years for treated patients to catch up in growth after prolonged corticosteroid therapy in infancy. Treatment is typically upwards of 9 to 12 months.

Vincristine is a first-line treatment for kaposiform hemangioendothelioma. It has supplanted interferon alfa-2a, which is avoided due to the risk of spastic diplegia.

Topical imiquimod is still considered an experimental therapy.

Question 29

Which of the following syndromes is most likely in a patient with venous malformations?
A) Kasabach-Merritt
B) Maffucci
C) Osler-Weber-Rendu
D) Parkes-Weber
E) Sturge-Weber

Answer 29

B) Maffucci

Maffucci syndrome is associated with venous malformations and multiple enchondromas. It can be associated with malignant chondrosarcomas, and intracranial tumors occur in 20% of patients.

Kasabach-Merritt syndrome is associated with a hemangioma or with diffuse hemangiomatosis. The hallmark is profound thrombocytopenia. The child can present with petechiae, ecchymosis, and bleeding.

Sturge-Weber syndrome is associated with facial capillary malformations (port-wine stain) distributed in a trigeminal nerve pattern, most often the first and second divisions of the nerve. Vascular malformations on the ipsilateral side can be found deep to the leptomeninges, and seizure disorders are common.

Parkes-Weber syndrome is a variant of Klippel-Trenaunay syndrome (patchy port-wine stains of the lower extremity with lymphatic/venous malformations and hypertrophy). However, in Parkes-Weber syndrome, there are arteriovenous fistulae present.

Osler-Weber-Rendu syndrome is also called hereditary hemorrhagic telangiectasia syndrome. It is inherited in an autosomal dominant fashion. The telangiectasia is located on the face, tongue, lips, nasal and oral mucosa, conjunctiva, and hands/nails. The lesions often emerge later in life.