Introduction A congenital disorder characterized by unilateral chest wall hypoplasia due to absence of sternocostal head of pectoralis major hypoplasia of the hand and forearm symbrachydactyly and shortening of middle fingers result of absence or shortening of the middle phalanx simple complete syndactyly of the short digits Epidemiology 1 in 32,000 live births occurs in 10% of syndactyly cases Etiology thought to be linked to subclavian artery hypoplasia Presentation Physical exam extent of hand and chest involvement varies chest deformities hypoplasia or absence of the pectoralis major, pectoralis minor, deltoid, serratus anterior, external oblique, and latissimus dorsi Sprengel’s deformity scoliosis dextrocardia absence or underdevelopment of the breast hand deformities syndactyly hypoplasia or absence of metacarpals or phalanges absence of extensors or flexor tendons carpal coalition or hypoplasia radioulnar synostosis nail agenesis Imaging CT scan will show absent perctoralis major Treatment Operative syndactyly release indications performed in most patients technique complete syndactyly release produces skin deficiency that requires skin grafting perform only one side of the digit at a time to avoid vascular complications local flap is created for commisure reconstruction followed by interdigitating zigzag dorsal and palmar flaps along the medial and lateral aspect of the digit Complications Skin graft failure Excessive tension Improper flap planning Digital artery injury Web creep Nail deformity
QUESTIONS 1 of 4 1 2 3 4 Previous Next Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (OBQ12.191) Which of following malformations is most commonly associated with Poland's syndrome? Review Topic QID: 4551 FIGURES: A B C D E 1 Figure A 32% (1341/4213) 2 Figure B 4% (187/4213) 3 Figure C 17% (701/4213) 4 Figure D 37% (1565/4213) 5 Figure E 9% (362/4213) ML 5 Select Answer to see Preferred Response PREFERRED RESPONSE 4 (OBQ04.152) Syndactyly is associated with all of the following conditions EXCEPT? Review Topic QID: 1257 1 Apert syndrome 3% (87/2901) 2 Poland's syndrome 5% (154/2901) 3 Holt-Oram syndrome 9% (259/2901) 4 Carpenter syndrome 8% (229/2901) 5 Tay-Sach's Disease 74% (2158/2901) ML 3 Select Answer to see Preferred Response PREFERRED RESPONSE 5