Embryology of the Upper limb and congenital hand anomalies Flashcards

Question

Failure of formation: Apert hand What is the definition, classification and treatment

Answer 1

Def: complex synctalyly pathognomonic for Aperts =\>fusion of D2,3,4 into central mass and D1/5 may be invovled. Thumb, if separate, radially deviated Classification: Upton * Accoding to 1st and 4th web and central mass * Type 1 * 1st&4th web: incomplete simple syndactyly * Central mass - flat, good MCPs, IP symphalangism, no synonychia * Type 2 * 1st&4th web: complete simple syndactyly * Central mass - concave, synonychia with distal fusion of DPs and proximal splaying of MC * Type 3 * 1st&4th web: complete complex syndactyly * Central mass - thumb incorporated, tight cupping, synonychia, D2 ray skeletal abnormalities TREATMENT according to Upton's Type * Type 1-2, stage * Stage 1 - thumb corrected position w ostetomy and release 2nd and 4th web to create 3 digits * Stage 2 - release remaining digits * Type 3 * separate synonychia first (multiple infx risk) * then stage as above

Answer 2

MCPs and D5 DIP

Answer 3

* Def: congenital PIPj flexion contracture * Etiology * unknown - Macfarlane hypothesized lumbrical anomalous insertion on D5 PP to act as flexor not extensor of IP * Clinical presentation * 2/3 bilateral, * AD w variable penetrance * may be congenital or acquired * Classification * Reducible or not * congenital or acquired * Type 1 - congenital - present at birth, isoalted, usually just D5 * Type 2 - acquired - presents in preadolescent * Type 3 - severe - associated w syndrome * DDX * marfans, juvenile palmar fibromatosis * arthrogryposis, symphalangism, trigger finger * Beals syndrome (congenital contractural arachnodactyly * Treatment * Non-op - most * splinting, serial casting. Usual \<30' can be corrected non-op * Operative - if \>90 flexed, failed conservative * FDS trasnfer to extersor apparatus * lumbrical release * arthrodesis

Answer 4

LATERAL XRAY * flat dorsal head of PP * exaggerated recess neck of PP * narrowed PIP joint space * volar base beak on MP

Answer 5

* Definition; congenital stiffness of IPjts, most commonly PIP * Clinical presentation * AD * ulnar digits most common D5\>D4\>D3\>D2 * due to poor formation of joint surfaces and resulting ankylosis - usually stiff/fused in extension * poorly formed MP * Classification - Flatt * type 1 - true symphalangism - stiff w normal Phalangeal length * type 2 - symbrachydactyly - most common - stiff w shortened length * type 3 - symphalangism w assocaited abnoralities (clinodactyly, hearing defect) * Treatment * non-op - not effective * Operative; fusion in correct position

Answer 6

* Def: Angular deformity due to abnormal phalageal growth (longitudinal bracket epiphysis) * Etiology * epiphysis abnomrally present along diaphysis (C- J- shaped) leading to abnormal growth (delta phalanx) * Presentation * AD, inherited forms usually bilateral * D5 MP and D1 PP most common location * most comomn radial deviation D5 MP * Rarely in isolation * Assocaited with symphalangism, polydactyly, syndactyly, Polands, CLEFT foot * Associated with syndromes Apert/Pfeiffer (=\>thumb delta phalanx), Downs (D5 delta phalanx) * Classification - **Cooney** * ****type 1 - simple - bone \<45'angulation * type 2 - simple complicated - bone \>45' * type 3 - complex - bone and ST deformity \<45' & syndactyly * type 4- coplex complicated - bone & ST deformity \>45' & polydactyly /gigantism * Treatment * non-op not effetcive * OPeratie - only if \>45' and functional/cosmetic defomrity * Osteotomies - closing/opening wedge, rotation, reversed

Answer 7

* Def: hypersegmentation anomaly - 3rd phalanx * Features * abnormal phalanx * web contracture * thenar hypoplasia * Associated with * Holt Oram, polydactyly, blood dyscrasias, cleft foot * Treatment * at 12mths * resect phalanx, deepend 1st web, opponenplasty if required later

Answer 8

Stelling ## Footnote Type I - extra soft tissue mass only Type 2 - extra whole digit articulating with MC/P Type 3- extra whole digit articulating w EXTRA MC Crossed polydactyly - invovles hand and feet Mixed polydactyly - invovles both ulnar/radial

Answer 9

* duplicate central digit, most ofeten assocaited with SYNDACTYLY * most common ulnar digits * frequent bilateral +/- feet * Treatment * carry out normal syndactyly release, recon DTML

Answer 10

* Def: duplication of little finger * Epidemiology * cited as most common congenital hand anomaly * most common polydactyly * frequently bilateral * 8x more common in africans * can be isolated, AD or syndromic, AR * Classification - McKusick, Stilling, Temtamy * TYpe A - well developed digit * Type B - rudimentary digit (no bone articulation) * Treatment * after 6months * Goals: remove extra digit, rebalance ST/jt/tendons * Type A: excise ulnar-most digit, reattach UCL/hypothenar muscles

Answer 11

* Def: charcaterized by absent radius and two ulnae, 7-8 digits and no thumb * Clinical presentation * usually soradic, unilateral * if inherited, bilateral +/- associated with fibular dimelia * stiff elbow, flexed wrist, poor pron/sup * duplicated ulnar carpal bones * stiff IP joints * bifucated flexor tendons shared by digits * 2 ulnar arteries joined by one superficial palmar arch * Treatment goals * correct elbow stiffness * improve pronation/supination * improve wrist stiffness * create thumb and webspace * remove multiple digits * Treatment steps * preaxial ulna resected subperiosteally at elbow joint * tendon lengtheing of flexors and transfer to extensors * pollicization of pre-axial digit * amputation of other excess digits

Answer 12

* Definition: overgrowth of all structures in digit * Clinical presentation * usually single digit or 2-3 digits adjacent to one another * may be static (large at birth) or progressive (more commonly, faster gorwth rate compared to other digits) * Unilateral, right * most common index, long * DDx * macrodactyly, hemihypertrophy, Vascular malformation, lymphatic malformation, neurofibromatosis, proteus * Associated syndromes * neurofibromatosis, lipomatosis * Maffucci, Ollier (high maignant potential) * Klippel trenaunay * Proteus (hamartomatous dysplasia, hemihypertrophy, feet/hand macrodactyly, parotid/ovarian tumor, CLVM) * Treatment * Ray amputation (preferred if non-fx) * debulking (not effective)

Answer 13

* Etiology * external - amnion band constricting limb * Clinical presentation * all sporadic * most commonly afftect index/long fingers * proximal tissues form the band are normal * Assocaited with Oligohydramnios, prematurity * Classification: **Patterson** * Type 1 - shallow band * Type 2- deep band with distal lymhedema * Type 3 - distal fusion of structures (acrosyndactyly) * A- fusion of tips * B- fusion of tips and incomplete web formation * C- just fenestration in web * Type 4 - intrauterine amputation * Type 5 - mixed * Treatment * Type A: for cosmesis - excise and release band w zplasty. Need to monitor for acute edema/impedending tissue loss * Type B : ass. w edema, neuro deficit; release band w zplasty, consider staged doing 1/2 at a time * Type 3 - treat as acrosyndactyly, earile than 12m

Answer 14

* Def: congenital abnormal groth of radius physis at the volar ulnar aspect of the distal bone * = dorsal subluxation of ulna * = volar ulnar subluxation of carpus * Presentation * usually in adolescence with wrist pain/stiffness, limited supination, extension * females, sporadic, bilateral * P/E: carpus volar ulanr, ulna dorsally subluxed, limited supination, limited finger extension/EDC rupture, +/- CTS * Xray: Radial volar angulation of radius and ulna dorsal subluxation * Assocaited w: * kienbock, achondroplasia, ollier, MPS * Treatment * tx at skeletal maturity if possible adn symptomatic * jt leveling procedure * partial/total radiocarpal arthrodesis * radioulnar arthrodesis

Embryology of the Upper limb and congenital hand anomalies Flashcards

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