ABSTRACT
Complete congenital arhinia is a rare defect of embryogenesis leading to the absence of the external nose and airway. We report our novel multistaged reconstructive approach and literature review. Nasal methyl methacrylate prosthesis was created from a stereolithographic model for use as a temporary prosthesis and tissue expander. Lefort 1 with cannulization was utilized for midface advancement and airway formation. External framework was reconstructed with bilateral conchal bowl cartilage and rib osteocartilagenous grafts. Patient was pleased with the aesthetics and had safe decannulation with the ability to breathe through the nose and airway.
Subject(s)
Dental Implants , Rhinoplasty , Congenital Abnormalities , Esthetics, Dental , Humans , Nose/abnormalities , Nose/diagnostic imaging , Nose/surgeryABSTRACT
ABSTRACT: Noonan syndrome is a rare, autosomal dominant disorder encompassing multiple congenital defects, as well as association with solid tumor and lesion development. The authors present a 26-year-old female with known Noonan syndrome and ongoing complaint of worsening unilateral vision, progressing to vision loss due to lesion mass effect. Decompressive surgery was performed, restoring patient's vision to baseline immediately postoperative. The lesion was confirmed to be giant cell granuloma. In this paper we discuss the unique presentation of vision loss due to orbital giant cell granuloma in Noonan syndrome with postoperative return of vision; the importance of a multi-disciplinary team evaluation, thorough preoperative clinical and image-based work up, intraoperative findings, postoperative outcome, and complexity of definitive management.
Subject(s)
Granuloma, Giant Cell , Noonan Syndrome , Orbital Diseases , Adult , Blindness/etiology , Decompression, Surgical , Female , Granuloma, Giant Cell/complications , Granuloma, Giant Cell/diagnostic imaging , Granuloma, Giant Cell/surgery , Humans , Noonan Syndrome/complications , Noonan Syndrome/surgery , Orbital Diseases/surgeryABSTRACT
CASE: We discuss our reconstructive approach to avoid an above-knee amputation in a 33-year-old man presenting after lower extremity crush injury. We used a vascularized tibial bone flap and a foot fillet flap to restore length and joint functionality to the residual limb. The patient ambulates with good prosthetic fit on durable heel pad skin and 100° active knee motion. CONCLUSION: This pairing of intramedullary nail with vascularized bone flap and fillet flap to address soft-tissue coverage and retain limb length is a useful tool in traumatic lower extremity injury management, providing an alternative technique for tibial bone graft stabilization with robust, sensate tissue coverage.