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 ( see Syndactyly) clinical presentation thumb radial , 186–187 acrosyndactyly , 297–298 type II apert hand , 187–188 digital malformation , 297 Apical ectodermal ridge (AER) , 3–5 distal skeletal tapering , 297–298 , 210 , 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, , 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 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 , 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 , 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 distraction osteogenesis , 138–143 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 /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 , 32–36 physiolysis , 215, 216 , 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 , 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 morphological overview , 32, 33, 181 limb initiation , 3–4 signaling centers , 4 OMT vs. Swanson’s classifi cation , 15–16 D pattern formation Deformations , 18, 20 anterior–posterior/radial–ulnar patterning , 6–7 Delta phalanx deformity , 213, 214 dorsal–ventral patterning , 7–8 Dermatological anomalies , 182 proximal–distal patterning , 4–6 Diastrophic dysplasia , 340 physical medicine and rehabilitation (see Physiatry) Digital hypoplasia reconstruction , 302 psychological research Distal arthrogryposis adults , 76 clinical features of , 305, 307 children and adolescents , 75–76 hand treatment , 313 parental coping and child , 76–77 simple incomplete syndactyly , 313 stigma and stigmatization , 74 thumb-in-palm deformity , 313–314 352 Index

Distal Hoxd expression , 6, 7 median nerve block , 328 Dorsal ectoderm , 7, 8 well-padded postoperative cast , 329–330 Dorsal–ventral defi ciency types of , 325–326 dorsal dimelia Epiphyseal transfer , 90 ENGRAILED-1 pathway , 151, 152 Extrinsic tendon reconstruction , 103–105 in experimental animals , 152 in humans , 152–153 management , 153 F ventral dimelia Fanconi anemia , 86 in humans and experimental animals , 154 FDP. See Flexor digitorum profundus (FDP) management , 154–155 Fibroblast growth factor 4 , 151 WNT7A pathway , 153, 154 Fibroblast growth factor 8 (FGF8) , 5 Dorsal–ventral patterning (D–V) , 7–8 Fibrodysplasia ossifi cans progressiva , 341–342 Dupuytren’s disease. See Juvenile palmar fi bromatosis Fibrous dysplasia , 281, 343–344 Dyschondrosteosis. See Leri–Weill dyschondrosteosis (LWD) Flexion adduction deformity , 227, 228 Dysplasias , 18–20 Flexor digitorum profundus (FDP) , 211 Dystrophic epidermolysis bullosa , 326 Flexor digitorum superfi cialis (FDS) , 103, 104, 233 Flexor pollicis longus (FPL) , 103–105, 235 Foot deformities , 201 E Four fl ap fi rst web-plasty , 99–100 EB simplex (EBS) , 325 Fuhrmann syndrome , 154 –ectodermal dysplasia–clefting (EEC) syndrome , 201 Edema massage , 62 G motion restriction , 63–64 Gaucher’s disease , 345 pressure , 62–63 Gel pin suspension , 55 scar , 62 Gorham disease , 344 silicone and tape , 63 Greig cephalopolysyndactyly , 30, 32 EEC syndrome. See Ectrodactyly–ectodermal dysplasia–clefting (EEC) syndrome Ehlers Danlos syndrome (EDS) , 342 H Elbow treatment, arthrogryposis Haas type polysyndactyly , 163, 166 muscle transfers , 310–311 , 280–281 nonoperative management , 308 Hand-foot-genital syndrome , 221 posterior elbow capsular release , 309 Handplate patterning radial head dislocations , 311 digit number , 8–9 splint use , 309, 310 digit-specifi c morphology , 9–10 Elephant’s trunk sign, MCP joint instability , 101, 102 interdigital cell death , 10 Ellis–van Creveld syndrome, 221, 341–342 Hedgehog pathways , 160 E. Nail dysplasia , 197 Hereditary brachial plexopathy , 45 Enchondroma , 343 Hereditary multiple exostoses , 341–342 Endochondral ossifi cation , 13 Holt–Oram syndrome (HOS) ENGRAILED-1 pathway , 151, 152 anesthesia concerns , 47 Epidermolysis bullosa (EB) , 46 radial longitudinal defi ciency , 86 cast removal , 330 Homeobox1 , 15 complications , 331–332 Humans diagnosis , 326 distal dorsal dimelia , 152–153 hand contractures , 326–327 proximal dorsal dimelia , 152 intraoperative image , 332, 333 ventral dimelia , 154 nonoperative management , 327 Hyperhidrosis , 187 operative treatment indications , 327 Hypoplasia , 227, 229 postoperative patient, contracture releases , 332 of hand , 199–200 preoperative considerations contracture releases , 332 fragile skin , 327 I intravenous line, Coban , 327–328 IFSSH classifi cation , 27–28 non-adhesive dressing , 327 iLimb Ultra , 55, 56 pseudosyndactyly , 325, 326 Illizarov technique , 120 rehabilitation , 330–331 Immobilization , 187 surgical technique Impairment , 74 antibiotic ointment and petrolatum gauze , 329–330 Impairment-based measurement tools , 60 contracture release , 329 Index fi nger fi nger pinning and skin grafts , 329 cleft hand, deviation , 206 hand degloving , 328 congenital clasped thumb intraoperative image, epidermal cocoon , 328 hypoplasia , 227, 229 Index 353

radial deviation , 227, 228 radiographic parameters of , 319 Innervation of limb , 15 treatment methods Intravenous catheters , 40–41 distal osteotomy , 322–323 Isolated brachydactyly , 32–36 multiple osteotomies , 323 physiolysis and vickers ligament release , 320, 321 radial dome osteotomy , 320–322 J Malformations , 16, 17 Junctional epidermolysis bullosa , 326 Manske classifi cation, thumb hypoplasia , 95–97 Juvenile palmar fi bromatosis , 210 classifi cation/diagnosis , 337 defi nition of , 336 K genetic cause of , 336–337 Kindler syndrome , 326 management , 337 Klippel–Trenaunay syndrome , 345 McKusick–Kaufman syndrome , 30, 31 Kniest’s dysplasia , 341 Meckel–Gruber syndrome , 30, 31 Melorheostosis , 344 Mesoaxial synostotic syndactyly , 163, 167 L Mesomelia , 318 , 345 Metacarpal bone deformities , 202–203 Leri–Weill dyschondrosteosis (LWD) , 318, 344 Metacarpal synostosis Lim homeobox 1b (LMX1B) , 7–8 classifi cation , 222–223 LMX1B , 151 ring vs. little fi nger , 222, 223 Lobster-claw deformity , 153 treatment , 223, 224 Longitudinal epiphyseal bracket , 214, 216 Metacarpophalangeal (MCP) joint instability , 100–102 Metaphyseal chondrodysplasia , 343 Microsurgical toe-to-hand transfer M indications , 143–144 Macrodactyly outcomes , 149 bones, joints, and epiphyses preoperative evaluation , 144 angulation , 287, 288 surgical technique , 144–148 length reduction , 286, 287 timing of surgery , 144 nail size and tip projection , 286–288 Mitten hand , 326–327 thumb , 287, 288 Monodactylic type III symbrachydactyly , 129, 130 width reduction , 286 Morphogenesis , 13 classifi cation Mucopolysaccharidoses , 341 chronologic development , 277–278 Muffuci syndrome , 281 digital hyperostosis , 277 Multiple congenital contractures , 307 multiple variations, upper limb , 276–277 Multiple epiphyseal dysplasia , 342 vascular malformations , 276 Multiple osteotomies , 323 defi nition of , 275 Myogenesis , 14–15 of foot , 289 genetics CNP overproduction , 284 N non-syndromic and syndromic , 282–283 Nail–Patella syndrome , 154, 340 proteus syndrome , 284 Neurocutaneous syndromes. See Phakomatoses complex , 284 Niemann-Pick disease , 341 imaging , 284 Nonvascularized toe phalangeal bone grafts incidence , 279 description , 136 index and middle fi ngers , 284–285 donor site morbidity and patient/parental satisfaction , 137 nerve , 285, 286 indications and patient selection , 137 non-syndromic macrodactyly , 279, 280 limitations , 138 osteochondrodyplasias , 281 surgical technique , 137–138 overgrowth syndromes , 281–282 phakomatoses hamartomas , 280–281 O NF1 and NF2 , 279–280 Oculodentodigital dysplasia , 163, 165–166 ray amputation , 287–289 Oligodactylic type II symbrachydactyly , 129, 130 skin and soft tissue , 285 Ollier’s disease/enchondromatosis , 343–344 timing of surgery , 289 Opening wedge osteotomy , 215 Madelung deformity Oral-facial-digital (OFD) syndrome , 29–31 anatomy , 317–318 , 281 differential diagnosis (OI) Leri-Weill dyschondrosteosis , 318 classifi cation/characterization , 335–336 SHOX , 318 description of , 335, 340 , 318 genetics , 335, 340 history and epidemiology , 317 operative and non-operative/medical management , 336, 340 354 Index

Osteopathia striata , 344 etiology , 85 Osteopoikilosis , 344 Fanconi anemia , 86 Osteotomy Holt–Oram syndrome , 86 distal radius , 322–323 non-operative management , 88 multiple , 322–323 operative management radial dome , 320–322 type 0 , 88–89 Oxford Ankle-Foot Questionnaire (OAFQ) , 137 types I and II , 89 types III and IV , 89–91 orthotics , 67 P outcomes/complications , 91 Pallister–Hall syndrome , 30, 32 ROM exercise , 67 Palmar nail syndrome , 152–153 thrombocytopenia-absent radius syndrome , 86 Palmar thumb contracture thumb hypoplasia (see Thumb hypoplasia) MCP joint , 234 VACTERL association , 86 release of , 233 Radial polydactyly skin reconstruction and augmentation , 233–234 Bilhaut-Cloquet procedure Pattern formation Wassel types 2 , 3, 253–254 anterior–posterior/radial–ulnar patterning , 6–7 Wassel types 4 , 254–255 dorsal–ventral patterning , 7–8 epidemiology and genetics , 241 proximal–distal patterning , 4–6 excision and reconstruction Performance-based assessment , 61 arthroplasty , 244 Phakomatoses corrective osteotomy , 244–245 hamartomas , 280–281 ligamentoperiosteal fl ap , 243–244 NF1 and NF2 , 279–280 tendon realignment , 245 Physiatry Wassel type 1 , 245–246 goals of , 51 Wassel type 4 , 247–249 management , 52–53 Wassel type 7 , 249 prosthetic management ligation , 243 components , 54–56 on-top plasty functional measures , 53 function and appearance , 258–259 prosthetic prescription , 53–54 K-wire insertion , 258 transhumeral defi ciency , 54 radial thumb and ulnar thumb , 256–257 transradial defi ciency , 54 reconstructed thumb dorsal view , 258 psychosocial adjustment , 56–57 skin closure , 258, 259 upper limb defi cient child , 52 skin incision , 257–258 Physiolysis , 320, 321 ulnar fl oating thumb , 256, 258 clinodactyly , 215, 216 vessels location , 256, 257 Pleiotrophin (PTN) , 282 web space preservation , 258 Poland syndrome, anesthesia concerns , 47 preoperative evaluation , 242 Pollicisation , 270–271 simple excision , 243, 244 CMC joint reconstruction , 109–111 timing of surgery , 242–243 dorsal dissection , 108–109 Wassel classifi cation, polydactyly , 241–242 incisions , 105–106 Reduction defects , 36–38 palmar dissection , 106–108 Reduction theory, symbrachydactyly , 133 tendon reconstruction , 111 Rehabilitation Polydactyly and syndactyly congenital clasped thumb , 235 IFSSH classifi cation , 196–197 epidermolysis bullosa , 330–331 vs. longitudinal defi ciency , 192, 193 psychology , 51 Polysyndactyly , 192, 194 Retinoic acid (RA) , 3 Postaxial polydactyly. See Ulnar polydactyly Reverse wedge osteotomy , 215 Pouce fl outant thumb , 88 Rubinstein–Taybi syndromes , 213 Progressive diaphyseal dysplasia. See Camurati–Engelmann disease Prosthetic upper extremity functional index (PUFI) , 53 Proteus syndrome , 284 S Proximal–distal (P–D) patterning , 4–6 SHFM. See Split hand/foot malformation (SHFM) Pseudosyndactyly , 325, 326 homeobox (SHOX) gene , 318 Pyknodysostosis , 344 Single-electrode myoelectric hand opening mechanism , 54 Skeletal abnormalities, 182 Skeletogenesis , 11–13 R Skin incision,radial polydactyly Radial dome osteotomy , 320–322 on-top plasty , 257–258 Radial dysplasia , 227, 229 Wassel type 4 polydactyly , 247 Radial longitudinal defi ciency (RLD) Snow–Littler procedure , 204, 205 assistive technology , 68 Social handicap , 74 classifi cation , 86–87 Splinting , 231 clinical presentation , 88 Split hand/foot malformation (SHFM) , 191 description , 85 Spondyloepiphyseal dysplasia , 342 Index 355

Stigma , 74 classifi cation , 95–99 Stigmatization , 74 description , 95 Symbrachydactyly , 296 orthotics , 66 classifi cation , 129–133 results and complications , 112–115 clinical features , 133–135 ROM exercise , 66 defi nition , 129 scar management , 66–67 etiology , 136 surgical techniques surgical treatment extrinsic tendon reconstruction , 103–105 bone distraction osteogenesis , 138–143 fi rst web insuffi ciency , 99–100 microsurgical toe-to-hand transfer , 143–148 metacarpophalangeal joint instability , 100–101 nonvascularized toe phalangeal bone grafts , 136–138 opposition transfers , 101–103 Symphalangism , 184, 210, 221–222 pollicisation (see Pollicisation) Syndactyly , 68 post-operative management , 112 anatomical classifi cation , 160–161 reconstruction of grades 3, 4 and 5 , 111–112 anatomy and management , 168–169 Thumb patterning, molecular regulation of , 9–10 complications and outcomes , 172 Thumb radial clinodactyly , 186–187 defi nition of , 159 Thumb web space digital amputation , 186 cleft hand treatment , 203–204 environmental infl uence, limb formation , 168 dorsal index-combined fl ap technique , 235, 236 epidemiology , 159 dorsal rotation advancement fl ap technique , 234–235 full thickness skin grafting , 184, 185 release of , 233 genetic and molecular pathways , 160 skin reconstruction and augmentation , 233–234 genetic and perinatal investigation , 172 Tourniquets , 42 human limb development , 159 Transhumeral defi ciency , 54 limb growth control , 159 Transradial defi ciency , 54 longitudinal osteotomy , 184, 185 Transverse failure of formation , 134, 135 non-syndromic forms Transverse radial defi ciency , 52 autosomal dominant inheritance , 167 Trigger thumb Cenani–Lenz syndrome , 164, 166–167 orthotics , 69 Haas type polysyndactyly , 163, 166 ROM exercise , 68–69 mesoaxial synostotic syndactyly , 163, 167 Triphalangeal thumb , 200–201 metacarpal and metatarsal fusion , 163, 166 Tuberous sclerosis , 284 mitten hand , 163, 166 Turing-like patterning , 8–9 oculodentodigital dysplasia , 163, 165–166 Turner syndrome (TS) , 318 , 162, 165 zygodactyly , 162, 164–165 phenotypical classifi cation , 161–162 U rectangular dorsal advancement fl aps , 184–185 Ulnar cleft hand , 199 recurrence of , 187 Ulnar dimelia silastic sheets , 186 anatomy , 267–268 surgical technique classifi cation , 266, 267 Buck Gramcko nail fold creation , 170 complications , 271 dorsal island fl ap , 170 diagnosis , 268 dorsal pentagonal fl ap , 170 epidemiology , 266 dorsal view of Jose fl ap , 170 pathogenesis , 267 interdigital web space , 169 treatment palmar-shaped fl ap , 171 amputation , 270 volar zigzag approach , 171 elbow fl exion , 268–269 V-Y advancement fl ap , 171 metacarpals and phalanges , 270 zigzag dorsal fl ap , 171 mirror hand , 270 syndromic forms , 167–168 pollicization , 270–271 zigzag fi ngertip fl aps , 184, 185 Ulnar drift hand , 227, 229 Syndromic arthrogryposis , 307–308 Ulnar dysplasia , 123 Synpolydactyly , 162, 165 Ulnar longitudinal defi ciency (ULD) associated anomalies , 124 classifi cation , 123 T clinical picture , 124 Tachdjan classifi cation, congenital radioulnar synostosis , 118 description , 123 Tarsal vs. carpal coalitions elbow , 124 arthrogryposis multiplex congenita , 222 embryology , 123 hand-foot-genital syndrome , 221 hand , 124–125 symphalangism , 221–222 treatment Thanatophoric dwarfi sm/dysplasia , 339–340 elbow/humerus , 126 Thermoregulation , 41 forearm , 126 Thrombocytopenia-absent radius (TAR) syndrome , 86 hand , 125 Thumb hypoplasia upper and shoulder , 124 associations of , 95, 96 wrist and forearm , 124 356 Index

Ulnar polydactyly type 4 , 252 anatomy , 263–264 abductor pollicis brevis tendon detachement , 249 classifi cation , 261, 262 angular deformity , 247 complications , 266 bifurcation , 247–248 defi nition of , 261 bony growth and alignment , 255 diagnosis , 264–265 divergent-convergent , 247 epidemiology , 261–262 FPL insertion , 247–248 pathogenesis , 262–263 IP and MP joints, range of motion , 255, 256 syndromes association , 262, 263 K-wire and osteotomy site , 248, 249 treatment , 265–266 lateral views , 255, 256 Ulnocarpal arthrodesis , 91 metacarpal head portion , 248 Unilateral transradial prosthesis , 55 polydactylic thumbs , 254 Upper extremity anomalies , 182, 183 postoperative radiograph , 249, 255 Upper respiratory tract infection (URI) , 40 proximal phalanx , 248 Upton’s technique , 299, 301 reconstruction , 255, 256 skin incision , 247 type 7 V angular deformity , 249–250 VACTERL association , 86 complex reconstruction procedures , 249, 250 Vascular anomaly , 282 longitudinal K-wire insertion , 249, 251 Vasculogenesis , 11 preoperative radiograph , 249–250 Visceral anomalies , 182 radial triphalangeal thumb , 249, 250 Visible distinction , 74–75 range of motion , 249, 251 reconstructed thumb alignment , 249, 251 Wilkie classifi cation, congenital radioulnar synostosis , 118 W Wingless-type MMTV integration site family member 7A Wassel polydactyly , 252 (WNT7A), 7, 8, 153, 154 type 1 Wrist extension deformity , 227, 228 K-wire insertion , 246 Wrist treatment, arthrogryposis postoperative fi nding , 246 dorsal carpal wedge osteotomy , 312–313 right thumb , 245, 246 hand orthosis , 311 type 2 stiff upper limbs , 311 bony union , 253–254 distal phalangeal epiphysis , 253 nail contour and grew , 253–254 Z polydactylic thumbs nail , 253 Zancolli lasso procedure , 212 transverse K-wire insertion , 253 Zone of polarizing activity (ZPA) , 4, 6 type 3 , 252 Zygodactyly , 162, 164–165