ePoster
Presentation Description
Institution: Queensland Children's Hospital - Queensland, Australia
Background
Apert Syndrome is a congenital condition characterised by primary craniosynostosis, midface deformities and symmetrical malformations of the hands and feet. Apert hands demonstrate a spectrum of differences involving soft tissue and bony structures. Reconstruction is complex and multiple stages are required. The scarcity of local reconstructive options in infants augments the challenge.
Case Report
We present an 11-month-old infant girl with Apert syndrome with Upton Type III (Rosebud) bilateral hand deformities. Additional malformations in this infant included multi-suture craniosynostosis, midface hypoplasia and Veau-I cleft palate. The first stage of hand reconstruction involved the separation of bilateral thumbs. Triangular dorsal and volar flaps were raised. Osteotomy of the distal phalanx was performed to separate it from the remaining phalanges, and thumbs were separated. Following the inset of flaps, defects remained on the ulnar surface of the thumbs and the radial surface of each remaining rosebud. Novosorb® Biodegradable Temporizing Matrix (BTM) was inset into the defects and dressed with Mepitel®, gauze, wool and crepe. The surgical site was reviewed and re-dressed weekly as an outpatient. Four weeks following initial surgery, BTM was delaminated following complete integration, and the site was split skin grafted from a thigh donor site. Photography and imaging are presented. No complications have been noted at twelve months of follow up.
Conclusion
Surgical management of the Apert hand is a complex reconstructive challenge. BTM use in combination with split-thickness skin grafting is a novel and effective option to counter the relative scarcity of full-thickness skin graft donor sites in these affected infants.
Speakers
Authors
Authors
Dr Arunan Jeyakumar - , Dr Ricky Bilbao - , Dr Stuart Bade -