Page 11 Flashcards
syndrome of multiple hamartomas including HAMARTOMATOUS polyposis of the GIT, with goiter and thyroid adenomas and increased risk of breast ca and TCC of the urinary tract, and with mucocutaneous lesions with facial papules, oral papillomas, and palmoplantar keratoses
Cowden disease
phakomatosis associated with colonic polyps, cutaneous tumors, meningioma, glioma, as well as thyroid and breast neoplasms; half of patients with dysplastic cerebellar gangliocytoma has this syndrome
Cowden sidease
inherited ADENOMATOUS polposis coli / FAP syndrome which includes a few polyps in the small bowel, with associated bone and skin abnormalities
including cortical thickening of the ribs and long bones, multiple osteomas of the skull, supernumerary teeth, exostoses of the mandible, and dermal fibromas; desmoids; and epidermal inclusion cysts
Gardner syndrome
aka hepatorenal fossa
Morison’s pouch
familial ADENOMATOUS polyposis syndrome with associated tumors of the CNS
Turcot syndrome
pathologic ring-like stricture at the level of the B ring, caused by reflux esophagitis
Schatzki ring
normal variant, elongated inferior tip of right lobe of liver, >women
Riedel lobe
association of cervical esophageal webs, IDA, and pharyngeal or esophageal ca
Plummer-Vinson syndrome or Paterson-Kelly syndrome
consists of 1) dlated small bowel loops, 2) air in biliary tree or gallbladder, and 3) calcified gallstones in ectopic location
Rigler triad
occur in the lower abdominal wall lateral to the rectus abdominis and inferior to the umbilicus through a defect in the aponeurosis of the transversus abdominis and internal oblique muscles
Spigelian hernias
systemic disorder affecting the GIT, joints, CNS, and LNs, which may present with arthritis, neurologic symptoms, or steatorrhea
Whipple disease
entrap only a portion of the bowel wall without compromising viability
Richter hernias
most common subtype (type IA) of glycogen storage disease to affect the liver
von Gierke’s disease
Bile duct hamartomas
von Meyenburg complexes
copper deposition
Wilson disease
acrocephalosyndactyly consisting of suture closure, midfacial hypoplasia, and symmetrical syndactyly of the hands and feet. Characteristic skull findings at birth are closed coronal suture and midline calvarial defect from glabella to the posterior fontanelle, producing a wide metopic suture area. Cerebral anomalies are common. Cervical spine fusion is common and almost always involves C5-C6 (vs C2-C3 in Crouzon’s syndrome)
Apert’s syndrome
caused by gastrin-secreting islet cell tumor (gastrinoma), resulting in unusually severe and refractory ulcer disease, accompanied by diarrhea
Zollinger-Ellison syndrome
craniofacial dysostosis; characterized by craniosynostosis, exophthalmos, and midface retrosuion. Other findings include prominent digital markings in the skull (>90%), stylohyoid ligament calcification, and nasal septal deviation. Maxilla is hypoplastic. Spinal anomalies usually consist of C2-C3 fusion.
Crouzon’s syndrome
acrocephalosyndactyly type 1 - craniosynostosis with hypertelorism, midface hypoplasia, and cervical spine anomalies; severe symmetric hand and foot syndactyly
Apert syndrome
mandibulofacial dysostosis; antimongoloid slant to the eyes, flat cheekbones, small mandible, dysplastic ears, deafness, coloboma, and deficient lashes in the lower eyelids. Radiographic findings: marked hypoplasia of the zygomatic arches, maxilla, and paranasal sinuses. Egg-shaped orbits. Sometimes, with external and middle ear involvement.
Treacher Collins syndrome
Enthesophyte that arises from the posteroinferior portion of the glenoid rim, often seen in baseball pitchers and probably arising at the site of insertion of the posterior band of the inferior glenohumeral ligament complex
Bennett’s lesion
fracture at the base of the thumb into the carpometacarpal joint
Bennett fracture
fracture of the base of the thumb that does not involve the joint
psuedo-Benett fracture
comminuted fracture of the base of the thumb that extends into the joint
Rolando fracture
normal anatomic landmark that if is narower than 20 deg is indicative of a compression/fracture of the calcaneus
Bohler angle
Fracture of the dorsal rim of the radius with intra-articular extension
Barton’s fracture
Fracture of the styloid process of the radius
Hutchinson’s / chauffeur’s fracture
Fracture of the distal radius with dorsal displacement
Colle’s / Pouteau’s fracture
variation of seatbelt injury demonstrating fracture through the spinous process
Chance fracture
Fracture of the distal radius with palmar / volar displacement; reverse Colle’s
Smith’s / Smith fracture
variation of seatbelt injury demonstrating fracture of the posterior body
Smith fracture
Combination of ulnar fracture and superior radial / radial head dislocation (GRIMUS)
Monteggia’s fracture-dislocation
Combination of radial fracture and inferior radioulnar dislocation (GRIMUS)
Galeazzi’s fracture-dislocation
condensed linear shadow on the lateral radiograph representing tangential bone in the intercondylar fossa
Blumensaat’s line
absent anterosuperior labrum in association with a thickened “cord-like” middle glenohumeral ligament
Buford complex
neuropathic joint
Charcot joint
severe arthropathy of CPPD mimicking a neuropathic or Charcot joint
pseudo-Charcot joint
to the upper outer acetabular margin consists of rheumatoid arthritis, splenomegaly, and leukopenia
Felty’s syndrome
indication of acetabular depth; angle formed by a perpendicular line through the midportion of the femoral head and a line from the femoral head center
center-edge angle of Wiberg
bony irregularity or fragment off the inferior glenoid (same mechanism with Hill-Sachs)
Bankart deformity
triad consisting of keratoconjunctivitis sicca, xerostomia, and connective tissue disease. Last feature may be identical to RA
Sjogren’s syndrome
AI disorder characterized by sicca syndrome (dry eyes/keratoconjunctivitis sicca, dry mouth/xerostomia, and dry nose/xerorhinia) which results from
Sjogren syndrome
Garre’s sclerosing osteomyelitis
indentation on the posterosuperior portion of the humeral head; caused by the impaction of the humeral head on the inferior lip of the glenoid during a shoulder dislocation
Hill-Sachs deformity/lesion
atlantoaxial subluxation in association with an inflammatory condition of the head and neck
Grisel’s syndrome
burst fracture of C1
Jefferson’s fracture
aka dancer’s fracture; avulsion of the base of the 5th metatarsal from a pull by the peroneus brevis tendon
Jones fracture
erosive osteoarthritis; same distribution with primary osteoarthritis but with erosions and osteoporosis
Kellgren arthritis
avascular necrosis of the carpal lunate
Kienbock malacia
avascular necrosis of the tarsal navicular
Kohler disease
avascular necrosis of the metatarsal heads
Freiberg infraction
avascular necrosis of the ring of the epiphyses / apophyseal ring of the spine
Scheuermann disease
avascular necrosis of the femoral head
Legg-Perthes disease
fracture through large osteoid seams; pathognomonic for osteomalacia
Looser fracture / Looser’s zones
avascular necrosis of the tibial tubercle (aka surfur knees)
Osgood-Schlatter disease
delayed further collapse of a wedge compression fracture with severe neurologic deficits 1-2 weeks after trauma
Kummell disease
meniscofemoral ligament that extends from the medial femoral condyle, passes in front of the PCL, and attaches to the posterior horn of the lateral
ligament of Humphrey
meniscofemoral ligament that extends from the medial femoral condyle, passes behind the PCL, and attaches to the posterior horn of the lateral meniscus
ligament of Wrisberg
fracture-dislocation of the tarsometatarsals
Lisfranc fracture
triad: polyostotic fibrous dysplasia associated with cafe au lait spots on the skin and precocious puberty; assoc with pituitary adenomas
McCune Albright syndrome
bilateral nodular adrenal hyperplasia
McCune-Albright syndrome
mucopolysaccaridose characterized by abnormal storage and excretion in the urine of keratin sulfate
Morquio syndrome
mucopolysaccaridose characterized by abnormal storage and excretion in the urine of heparan sulfate
Hurler syndrome
multiple enchondromas or enchondromatosis
Ollier disease
multiple enchondromas associated with soft tissue hemangiomas
Maffucci syndrome
neurogenic edema is found in muscle groups that correspond to a particular nerve (ie, supra/infra=suprascapular nerve; teres minor/deltoid=axillary nerve)
Parsonage-Turner syndrome
Triad of radiographic findings characteristic of TB arthritis
Phemister’s triad
joint affliction occuring after minor trauma to an extremity, demonstrating severe patchy osteoporosis and ST swelling
Sudeck atrophy
reactive arthritis
Reiter syndrome
Salter epiphyseal fracture
small, bony fragment pulled off the posterior lateral tibial joint line by an avulsion of the lateral joint capsule
Segond fracture
(+) space between navicular/scaphoid and lunate
Terry Thomas sign
another name for pyknodysostosis
Toulouse-Lautrec syndrome
Voorhoeve disease
aka osteopathia striata which manifests multiple linear bands of sclerosis parallel to the long axis of the bone
partial sacral agenesis, anorectal stenosis, and presacral mass
Currarino triad
thymic aplasia, absence of the parathyroid glands, and cardiovascular anomalies; caused by faulty development of the 3rd and 4th pharyngeal pouches
DiGeorge syndrome
one of the syndromes associated with short ribs and small thoracic cage
Ellis-van Creveld syndrome
hepatoportoenterostomy
Kasai procedure
vasculitis of unknown etiology that affects the skin, GIT, joint, and kidneys; abdominal pain and intestinal bleeding (50%)
Henoch-Schoenlein purpura
hemangioendothelioma associated with thrombocytopenia due to sequestration of platelets within the tumor
Kasabach-Merritt syndrome
mucocutaneous lymph node syndrome
Kawasaki disease
peritoneal bands
Ladd bands
utilized to determine the level at which the blind pouch ends on lateral radiographs. Line is drawn perpendicular to the long axis of the ischia on lateral view and passes through the junction between the middle and lower third of the ischia
M line of Cremin
associated with bilateral renal agenesis, congenital renal cystic disease, or obstructive uropathy (posterior urethral valves, prune belly syndrome) resulting
in hypoplastic lungs
Potter syndrome
triad of necrotizing granulomatous lesions in both the upper and lower respiratory tract, as well as glomerulonephritis
Wegener polyangitis
