Index A dermatological anomalies , 182 Abductor digiti minimi (ADM) transfer , 102–103 skeletal abnormalities , 182 Abductor pollicis brevis (APB) , 186–187 upper extremity anomalies , 182, 183 ABS. See Amniotic band syndrome (ABS) visceral anomalies , 182 Achondroplasia defi nition of , 179 classifi cation/characterization , 338 description , 32, 33 defi nition of , 337–338 epidemiology of , 181 genetics , 338 genetics and embryology management , 338 molecular etiology , 180 Acrocephalosyndactyly syndrome , 32, 33, 179 prenatal diagnosis , 180–181 Acrosyndactyly repair, ABS , 300–302 molecular basis of , 180 Adactylic group IV symbrachydactyly , 129, 131 postoperative care and complications , 187 Al-Awadi syndrome , 154 treatment Amniotic band syndrome (ABS) APB release , 186–187 acrosyndactyly repair , 300–302 border digits syndactylies , 184 anesthesia concerns of fi rst web space release , 186 induction and maintenance of anesthesia , 43 patient age , 183 postoperative concerns , 43 secondary revisions , 187 preoperative preparation , 42–43 symphalangism , 184 classifi cation , 298 syndactyly ( see Syndactyly) clinical presentation thumb radial clinodactyly , 186–187 acrosyndactyly , 297–298 type II apert hand , 187–188 digital malformation , 297 Apical ectodermal ridge (AER) , 3–5 distal skeletal bones tapering , 297–298 Arthrogryposis , 210 hand deformity , 297 classic arthrogryposis , 305–306, 308 complications , 302 classifi cation , 305, 306 constriction band release defi nition of , 229, 230, 305 Upton’s technique , 299, 301 de-rotation osteotomy, shoulder , 308, 309 z-plasty , 299–300 distal , 230–231 ( see Distal arthrogryposis) diagnosis of , 296 elbow treatment digital hypoplasia reconstruction , 302 muscle transfers , 310–311 etiology of , 295–296 nonoperative management , 308 preoperative considerations , 299 posterior elbow capsular release , 309 treatment , 299 radial head dislocations , 311 Amniotic constriction band syndrome. See Amniotic splint use , 309, 310 band syndrome (ABS) etiology , 307–308 Amputation hand treatment , 313 ray , 287, 289 syndromic , 231, 306 ulnar dimelia , 270 types of , 305, 306 Amyoplasia , 230, 305–306, 308 wrist treatment Angiogenesis , 11 dorsal carpal wedge osteotomy , 312–313 Anterior–posterior/radial–ulnar patterning (A–P/R–U) , 6–7 hand orthosis , 311 APB. See Abductor pollicis brevis (APB) stiff upper limbs , 311 Apert syndrome Arthrogryposis multiplex congenita (AMC) , 222 acrocephalosyndactyly syndrome , 179 description , 44 advantages of , 179–180 induction and maintenance of anesthesia , 45 anesthesia concerns , 43–44 postoperative concerns , 45 clinical features preoperative evaluation , 44–45 CNS abnormalities , 181–182 Arthroplasty , 244 craniofacial anomalies , 181 Assisting Hand Assessment (AHA) , 59 D.R. Laub Jr. (ed.), Congenital Anomalies of the Upper Extremity: Etiology and Management, 349 DOI 10.1007/978-1-4899-7504-1, © Springer Science+Business Media New York 2015 350 Index B hand-foot-genital syndrome , 221 Bardet–Biedl 6 (BBS6) syndrome , 30, 31 symphalangism , 221–222 Bent fi nger. See Camptodactyly Cenani–Lenz syndrome , 164, 166–167 Bifurcation, EPL tendon , 247 Central cleft hand , 199 Bilateral thumb deformity , 216 Central defi ciency of the hand. See Cleft hand Bilateral transradial prosthesis , 55, 56 Centralization procedure , 89–90 Bilhaut-Cloquet procedure (BC procedure) Chondrodysplasia punctata , 343 slight volar axial rotation , 251–252 Chondroectodermal dysplasia. See Ellis–van Creveld syndrome Wassel type 2 , 252 Chondrogenesis , 11–13 bony union , 253–254 Ciliopathies , 30, 31 distal phalangeal epiphysis , 253 Cilium-centrosome complex (CCC) , 30 nail contour and grew , 253–254 Classic arthrogryposis. See Amyoplasia polydactylic thumbs nail , 253 Claw fi nger deformity , 201 transverse K-wire insertion , 253 Cleary and Omer classifi cation, congenital radioulnar Wassel type 3 , 252 synostosis , 118–119 Wassel type 4 , 252 Cleft hand bony growth and alignment , 255 abnormal induction, digital rays , 199–200 IP and MP joints, range of motion , 255, 256 clinical characteristics , 197–198 lateral views , 255, 256 defi nition of , 191 polydactylic thumbs , 254 EEC syndrome , 201 postoperative radiographs , 255 foot deformities , 201 reconstruction , 255, 256 fourth web space , 199, 200 Bizarre forearm synostosis , 86 IFSSH classifi cation Blauth classifi cation, thumb hypoplasia , 95–97 brachysyndactyly , 195 Blisters , 325–327, 331 E. Nail dysplasia , 197 Body-powered terminal devices , 53 hand modifi cation , 195–196 Bone distraction osteogenesis , 138–143 polydactyly and syndactyly , 196–197 advantages , 138 incidence and genetics , 191 digital lengthening , 138 vs. longitudinal defi ciency distraction osteogenesis , 141, 142 abnormal cleft formation , 195 indications and patient selection , 142 busulfan , 194–195 limitations , 143 central polydactyly and syndactyly , 192, 193 surgical technique , 142–143 polysyndactyly P-3 and P-4 types , 192, 194 Bone morphogenetic protein family member 4 (BMP4) , 11 P-0 type anomalies , 192–193 Border digit syndactylies , 184 teratogenic mechanisms , 192, 194 Boutonniere deformity , 210 surgical classifi cation Brachman de Lange syndrome , 33 central cleft hand , 199 Brachysyndactyly , 195 defective fi nger rays , 198 Busulfan , 194–195 radial cleft hand , 199 thumb web space characteristics , 198–199 ulnar cleft hand , 199 C treatment Camptodactyly claw fi nger deformity , 201, 206 classifi cation of , 209, 210 deep transverse metacarpal ligament , 203 congenital clasped thumb , 227, 228 excessive interdigital space closure , 202 diagnosis of , 210–211 index fi nger deviation , 206 generalized condition , 209 indication and timing , 201 nonsurgical treatment , 211 metacarpus and cross bone , 202–203 non-traumatic progressive fl exion deformity , 209, 210 preoperative care , 202 orthotics , 65 ring vs. little fi ngers, syndactyly separation , 204, 205 pathology of , 209–210 thumb deviation , 204, 206 PROM and orthotics , 65–66 thumb web space widening , 203–204 ROM exercises , 64–65 triphalangeal thumb , 200–201 surgical treatment typical and atypical , 191, 192 PIP joint , 211–212 Cleidocranial dysostosis/dysplasia , 341 Zancolli lasso procedure , 212 Clinodactyly Camurati–Engelmann disease , 343–344 causes and associated conditions of , 213 Carpal coalitions classifi cation and differential diagnosis , 214 classifi cation , 219–220 clinical presentation of , 213 Ellis–van Creveld syndrome , 221 congenital curvature , 217 embryology , 219 defi nition of , 213 incidence , 219 etiology of , 214 isolated carpal coalition , 220–221 nonsurgical treatment , 214 vs. tarsal surgical treatment arthrogryposis multiplex congenita , 222 bilateral thumb deformities , 216 Index 351 closing wedge osteotomy , 214 syndromes with opening wedge osteotomy , 215 brachydactyly , 32–36 physiolysis , 215, 216 oligodactyly , 36–38 reverse wedge osteotomy , 215 polydactyly , 28–32 thumb radial , 186–187 reduction defects , 36–38 Closing wedge osteotomy , 214 syndactyly , 32 CLOVES syndrome , 282 visible distinctions and stigma , 74–75 CNS abnormalities , 181–182 Congenital brachial plexus palsy , 45 Congenital anomalies of upper extremity (CAUE) Congenital clasped thumb anesthesia concerns algorithm of , 232 amniotic band syndrome ( see Amniotic band arthrogryposis , 229 syndrome (ABS), anesthesia concerns of) camptodactyly , 227, 228 antibiotic prophylaxis , 41 complex clasped thumb , 232 anxiety , 39–40 defi nition , 227 Apert syndrome , 43–44 fl exion adduction deformity , 227, 228 arthrogryposis , 44–45 index fi nger brachial plexopathies , 45–46 hypoplasia , 227, 229 epidermolysis bullosa dystrophica , 46 radial deviation , 227, 228 fasting requirements , 40 IP joint , 227, 228 Holt–Oram syndrome , 47 narrowing and skin defi ciency , 227, 228 intravenous catheters , 40–41 pathoanatomy and classifi cation , 229–230 Poland syndrome , 47 prevalence and etiology , 230–231 positioning , 41 radial dysplasia , 227, 229 postoperative care , 42 rehabilitation , 235 thermoregulation , 41 supple clasped thumb , 231–232 tourniquets , 42 surgical treatment upper extremity nerve blocks , 41–42 FPL lengthening , 235 URI , 40 MCP joint stabilization , 235 VACTERL , 47–48 restore active thumb extension , 233 deformations , 20 thumb web space and palmar thumb contracture , 233–235 diagnosis-specifi c intervention treatment evaluation , 235–237 camptodactyly , 64–66 ulnar drift hand , 227, 229 radial longitudinal defi ciency , 67–68 Congenital heart disease (CHD) , 41 syndactyly , 68 Congenital radioulnar synostosis thumb hypoplasia , 66–67 Cleary and Omer classifi cation , 118–119 trigger thumb , 68–69 complications , 120–121 differentiation process description , 117 limb innervation , 15 embryology , 117 limb myogenesis , 14–15 epidemiology and natural history , 117 limb skeletogenesis , 11–13 imaging , 118 limb vasculogenesis , 11 osteotomy , 119–120 distinction , 73 post-operative correction , 120 dysplasias , 20 presentation and clinical features , 117–118 evaluation , 59–62 surgery, indications and age for , 119 handplate patterning Tachdjan classifi cation , 118 digit number , 8–9 Wilkie classifi cation , 118 digit-specifi c morphology , 9–10 Constriction band release, ABS interdigital cell death , 10 Upton’s technique , 299, 301 impairment and disability , 74 z-plasty , 299–300 incidence , 27–28 Cornelia de Lange’s syndrome , 33, 345 interventions to address impairment , 62–64 Craniocarpotarsal dysplasia , 345 malformations , 17–19 Craniofacial anomalies , 181