Summary Radial Clubhand is a rare birth defect characterized by a deformity of hand, with perpendicular relationship between forearm and wrist, and an absent thumb. Diagnosis is made on physical exam. Treatment may be observation or surgical reconstruction when the child is around 6 months of age. EPIDEMIOLOGY Incidence rare 1 per 100,000 anatomic location bilateral in 50-72% thumb usually deficient as well Etiology Genetics likely related to sonic hedgehog gene Associated with TAR autosomal recessive condition with thrombocytopenia and absent radius different in that thumb is typically present Fanconi's anemia autosomal recessive condition with aplastic anemia Fanconi screen and chromosomal breakage test to screen treatment is bone marrow transplant Holt-Oram syndrome autosomal dominant condition characterized by cardiac defects VACTERL Syndrome vertebral anomalies, anal atresia, cardiac abnormalities, tracheoesophageal fistula, renal agenesis, and limb defects) VATER Syndrome vertebral anomalies, anal atresia, tracheoesophageal fistula, esophageal atresia, renal agenesis) Classification Bayne and Klug Classification Type I Deficient distal radial epiphysis Type II Deficient distal and proximal radial epiphyses Type III Present proximally (partial aplasia) Type IV Completely absent (total aplasia - most common) Presentation Physical exam deformity of hand with perpendicular relationship between forearm and wrist absent thumb perform careful elbow examination Imaging Radiographs entire radius and often thumb is absent Laboratory must order CBC, renal ultrasound, and echocardiogram to screen for associated conditions Treatment Nonoperative passive stretching target tight radial-sided structures observation indicated if absent elbow motion or biceps deficiency hand deformity allows for extra reach to mouth in presence of a stiff elbow Operative hand centralization indications good elbow motion and biceps function intact done at 6-12 months of age followed by tendon transfers contraindications older patient with good function patients with elbow extension contracture who rely on radial deviation proximate terminal condition technique involves resection of varying amount of carpus, shortening of ECU, and, if needed, an angular osteotomy of the ulna (be sure to spare ulnar distal physis) may do as two stage procedure in combination with a distraction external fixator if thumb deformity then combine with thumb reconstruction at 18 months of age
QUESTIONS 1 of 9 1 2 3 4 5 6 7 8 9 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 Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (OBQ11.143) A child is seen in the pediatric orthopedic hand clinic for evaluation of a congenital deformity. A clinical photograph and radiograph are seen in Figures A and B. What is the next best step in this child's evaluation to rule out an associated autosomal-recessive lethal condition? QID: 3566 FIGURES: A B Type & Select Correct Answer 1 Cardiac ultrasound and renal ultrasound 30% (1082/3610) 2 Cardiac ultrasound, barium swallow and MRI 3% (117/3610) 3 LFTs, CBC and cardiac ultrasound 8% (287/3610) 4 Cardiac ultrasound, peripheral blood smear and MRI 9% (323/3610) 5 CBC, peripheral blood smear and chromosomal breakage analysis 49% (1782/3610) L 4 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 5 Review Tested Concept Review Full Topic (OBQ11.149) A 7-month-old boy has radial longitudinal deficiency. The initial work-up is negative for any cardiac, hematopoetic or renal abnormalities. He has good active elbow flexion and no other deformities. A clinical image and radiograph are seen in Figures A and B. Surgical management should include which of the following? QID: 3572 FIGURES: A B Type & Select Correct Answer 1 Definitive splinting and stretching 8% (343/4344) 2 Pollicization of the index finger 3% (121/4344) 3 ECRB and ECRL transfers to the ulna 3% (111/4344) 4 Free fibula transfer to the forearm 4% (154/4344) 5 Ulna centralization and possible tendon transfers 82% (3578/4344) L 2 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 5 Review Tested Concept Review Full Topic (OBQ09.152) A 6-year-old boy presents to your office with a forearm deformity and the radiographs shown in Figure A. What additional tests must be ordered for this patient? QID: 2965 FIGURES: A Type & Select Correct Answer 1 CBC 2% (106/5007) 2 CBC and renal ultrasound only 4% (217/5007) 3 CBC, renal ultrasound, and echocardiogram 91% (4577/5007) 4 CBC and MRI 1% (33/5007) 5 No additional testing needed 1% (48/5007) L 1 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review Tested Concept Review Full Topic (OBQ08.19) A 2-year-old boy has the upper limb deformity seen in Figures A and B. All of the following are associated with this condition EXCEPT? QID: 405 FIGURES: A B Type & Select Correct Answer 1 Fanconi's Anemia 5% (143/2947) 2 Holt-Oram syndrome 4% (118/2947) 3 VATER syndrome 3% (87/2947) 4 VACTERL syndrome 3% (97/2947) 5 Osteogenesis Imperfecta 84% (2490/2947) L 1 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 5 Review Tested Concept Review Full Topic This is an AAOS Self Assessment Exam (SAE) question. Orthobullets was not involved in the editorial process and does not have the ability to alter the question. If you prefer to hide SAE questions, simply turn them off in your Learning Goals. (SAE07PE.82) A 4-year-old child was born with bilateral congenital radial clubhands. Which of the following associated conditions is a contraindication to centralization of the hands on the ulna? QID: 6142 Type & Select Correct Answer 1 Congenital scoliosis 0% (3/1071) 2 Hypoplastic thumb 22% (237/1071) 3 Tracheoesophageal fistula 1% (14/1071) 4 Imperforate anus 1% (10/1071) 5 Lack of elbow flexion 75% (802/1071) L 2 Question Complexity E Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 5 Review Tested Concept Review Full Topic (SBQ04PE.69) Your partner, a pediatric hand surgeon, sees a child with a congenital upper extremity deformity. A radiograph is shown in Figure A. What is a relative contraindication to performing a hand centralization procedure in this patient? QID: 2254 FIGURES: A Type & Select Correct Answer 1 Diminished grip strength 4% (74/1899) 2 Unstable carpus 27% (516/1899) 3 Cardiac defects 5% (100/1899) 4 Limited terminal elbow extension 30% (564/1899) 5 Current patient age 34% (640/1899) L 5 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 5 Review Tested Concept Review Full Topic
All Videos (2) Podcasts (1) Login to View Community Videos Login to View Community Videos Cleveland Combined Hand Fellowship Lecture Series 2019-2020 Congenital Wrist and Forearm Deformities: Radial Longitudinal Deficiencies - Anil Akoon, MD Anil Akoon Hand - Radial Clubhand (radial deficiency) D 9/15/2020 83 views 5.0 (1) Login to View Community Videos Login to View Community Videos Frontiers in Upper Extremity Surgery - 2016 Radial Deficient Hand - Terry R. Light, MD (Frontiers #15, 2016) Terry Light Hand - Radial Clubhand (radial deficiency) A 2/17/2017 694 views 4.8 (6) Hand⎪ Radial Clubhand (radial deficiency) Hand - Radial Clubhand (radial deficiency) Listen Now 13:16 min 9/14/2020 305 plays 5.0 (1)