Introduction: The 4th-5th metacarpal synostosis, present in over 80% of Apert hands, flattens the metacarpal arch, restricts metacarpal descent, may prevent opposition of border rays, and combined with symphalangism, negates any functional flexion. Ironically, the fifth ray including the digit is the most intact finger within the hand. Restoration of both position and mobility with arthroplasty changes the cardinal plane of flexion and enables both pinch and grip in these compromised hands. This report summarizes the evolution of our technique over five decades.
Methods: In a cohort of 184 Apert patients (368 hands) the presence, anatomy, and level of the metacarpal synostosis with a classification was determined. The present technique consists of incision along ulnar border of hand, wide excision of the skeletal coalition, release of dorsal structures, soft tissue interposition (with cadaveric fascia lata graft) with the fifth metacarpal flexed and supinated. During the past two decades the fascia was also wrapped around the fifth metacarpal in a subperiosteal plane. No fixation is needed. The arthroplasty was commonly combined with a thumb lengthening procedure, such as opening wedge osteotomy plus autogenous bone graft at 2-6 years or distraction lengthening and bone graft at 11-13 years old. Silicone blocks, silicone sheeting, and autogenous tendon were also used as interpositions early in our experience were not included. Data was generated over a 46 period and consisted of clinical and operative records, serial molds, serial X-rays, and OT records. Follow-up ranged from 3 to 44 years.
Results: 80% of the hands (N=147 patients) had bilateral 4-5th metacarpal synostoses, the extent of which correlated with the Apert hand classification3. In 72 patients (144 hands) the synostosis was resected and cadaveric fascia interposed. The 7 hands that had silicone blocks, silicone sheets, or tendon interposition were not included in the final results. Synostosis refusion occurred in 38 hands, all of which were performed early in the series and under the age of 6 years. Despite refusion, the position of the 5th digit was improved and the flat transverse arch tin a more curved or cupped posture. Eight of refused synostoses were re-released, usually in conjunction with thumb distraction lengthening. Distance between the opposing border rays was always improved and a new grip and pinch mechanism created.
Conclusion: Aggressive ostectomy of synostosis and fascial interposition places the ulnar side of the hand in a much more functional position. In conjunction with thumb lengthening, opposition between the thumb and fifth finger becomes a clinical reality in Apert children who are born with diminutive thumbs and minimal interphalangeal joint motion.
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http://dx.doi.org/10.1097/PRS.0000000000011671 | DOI Listing |
Bones develop to structurally balance strength and mobility. Bone developmental dynamics are influenced by whether an animal is ambulatory at birth ( precocial). Precocial species, such as goats, develop advanced skeletal maturity in utero, making them useful models for studying the dynamics of bone formation under mechanical load.
View Article and Find Full Text PDFPlast Reconstr Surg
August 2024
MGB Harvard Plastic Surgery.
Introduction: The 4th-5th metacarpal synostosis, present in over 80% of Apert hands, flattens the metacarpal arch, restricts metacarpal descent, may prevent opposition of border rays, and combined with symphalangism, negates any functional flexion. Ironically, the fifth ray including the digit is the most intact finger within the hand. Restoration of both position and mobility with arthroplasty changes the cardinal plane of flexion and enables both pinch and grip in these compromised hands.
View Article and Find Full Text PDFBone Res
April 2024
McKusick-Zhang Center for Genetic Medicine, State Key Laboratory of Complex Severe and Rare Diseases, Department of Medical Genetics, Institute of Basic Medical Sciences Chinese Academy of Medical Sciences, School of Basic Medicine Peking Union Medical College, Beijing, 100005, China.
Syndactyly type V (SDTY5) is an autosomal dominant extremity malformation characterized by fusion of the fourth and fifth metacarpals. In the previous publication, we first identified a heterozygous missense mutation Q50R in homeobox domain (HD) of HOXD13 in a large Chinese family with SDTY5. In order to substantiate the pathogenicity of the variant and elucidate the underlying pathogenic mechanism causing limb malformation, transcription-activator-like effector nucleases (TALEN) was employed to generate a Hoxd13Q50R mutant mouse.
View Article and Find Full Text PDFJ Hand Surg Am
January 2024
Department of Orthopaedic Surgery, Seoul National University Hospital, College of Medicine, Seoul, Republic of Korea; Yeson Orthopaedic Hospital, Bucheon-si, Republic of Korea. Electronic address:
Purpose: We attempted a technique for patients with congenital ring-little finger metacarpal synostosis involving simultaneous interpositional allograft bone after split osteotomy of the synostosis site and distraction lengthening of the fifth metacarpal along with correction of the metacarpal joint abduction contracture. The purpose of this study was to describe the surgical technique and its outcomes.
Methods: We reviewed the medical records of children with congenital ring-little finger metacarpal synostosis treated surgically at our institute.
Mol Genet Genomic Med
January 2024
Medical Genetic Center, The Affiliated Yancheng Maternity & Child Health Hospital of Yangzhou University Medical School, Yancheng, China.
Background: Cenani-Lenzsyndactyly syndrome (CLSS; OMIM 212780) is a rare autosomal recessive acral deformity, which is mainly manifested in the fusion of fingers or toes, disordered phalangeal structure, shortening or fusion of the radius and ulna, and renal hypoplasia.
Case Presentation: Our report described an individual with mild phenotypes from China. His parents were not consanguineous.
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