Synostosis of the Ring-Small Finger Metacarpal in Apert Acrosyndactyly Hands: Incidence and Treatment

• Published in: Journal of pediatric orthopaedics Vol. 21; no. 4; pp. 502 - 507
• Main Authors: Dao, Khiem D, Shin, Alexander Y, Kelley, Steve, Wood, Virchel E
• Format: Journal Article
• Language: English
• Published: Hagerstown, MD Lippincott Williams & Wilkins, Inc 01-07-2001; Lippincott
• Subjects: Acrocephalosyndactylia - complications; Adolescent; Adult; Biological and medical sciences; Bone Wires; Casts, Surgical; Child; Child, Preschool; Diseases of the osteoarticular system; Female; Follow-Up Studies; Hand Strength; Humans; Incidence; Male; Malformations and congenital and or hereditary diseases involving bones. Joint deformations; Medical sciences; Metacarpus; Osteotomy - instrumentation; Osteotomy - methods; Radiography; Silicones; Synostosis - classification; Synostosis - diagnostic imaging; Synostosis - etiology; Synostosis - physiopathology; Synostosis - surgery; Treatment Outcome; Human; Prognosis; Diseases of the osteoarticular system; Finger; Synostosis; Dysostosis; Congenital disease; Epidemiology; Incidence; Metacarpal bone; Apert syndrome; Association; Treatment; Disease of the hand; Malformation; Skull disease; Upper limb; Child; Public health
• ISSN: 0271-6798; 1539-2570
• DOI: 10.1097/01241398-200107000-00016

This study reports on the incidence of a synostosis between the ring-small metacarpal bases in patients with Apert syndrome and describes a technique to resect the synostosis and insert silicone sheets to improve hand function. Records of 9 patients (18 hands) were evaluated. Average age at follow-up was 9.5 years. Three relationships between the ring-small metacarpals were observed. Type I hands (22%) had no abnormal interconnections; type II hands (33%) possessed a synostosis since birth; type III hands (44%) did not possess a synostosis at birth, but one later developed at average age of 79 months. Total incidence of synostosis was 77%. Five patients underwent excision of the synostosis. At an average of 16 postoperative months, 4 of these patients experienced improvement in hand prehension. In patients with Apert syndrome, resection of abnormal interconnections between the ring-small metacarpals is highly recommended to improve hand prehension based on the high incidence of a synostosis in this region.