Metachronous Microvascular Free Flaps In Patients With Fanconi Anemia
Matthew A. Wright1, Ishani Premaratne1, Mariam Gadjiko1, Gwendolyn S. Reeve2, David I. Kutler3, Jason A. Spector1.
1Weill Cornell Medicine, Division of Plastic Surgery, New York, NY, USA, 2Weill Cornell Medicine, Division of Oral and Maxillofacial Surgery, New York, NY, USA, 3Weill Cornell Medicine, Department of Otolaryngology, New York, NY, USA.
PURPOSE: Fanconi anemia (FA) is a genetic disorder characterized by bone marrow failure, chromosomal instability, bone defects, congenital anomalies, and a high incidence of solid and leukemic malignancy. Nearly 30% of patients develop a solid malignancy by age 40, with a large proportion of these being head and neck tumors with an incidence of relapse approaching 50%. Consequently, there is a need for reconstruction in this cohort, with free flaps often representing the most reasonable approach. Surgeons are often hesitant to perform such reconstructions on these patients, however, given the significant comorbidities associated with FA, and there are little published data regarding the safety and outcomes of such cases. We present a small case series performing metachronous head and neck free flaps in two patients with FA and show that such procedures can be performed safely and with acceptable functional and cosmetic outcomes not only once, but also in cases where metachronous free flaps are necessary. METHODS: Patients were identified, and charts were retrospectively reviewed for pertinent pre- and perioperative details in addition to functional and cosmetic outcomes. RESULTS: Case 1 A 43-year-old female with FA presented with squamous cell carcinoma (SCC) of the left mandible alveolar mucosa. This was resected and immediately reconstructed with a right fibular osteomyocutaneous free flap, with good functional (tolerating softs by four months) and cosmetic outcomes. Eighteen months later, she was found to have SCC of the hypopharynx. After strict hematologic optimization including transfusion to a hemoglobin above 7 g/dL and a platelet count greater than 50,000 cells/mL, she underwent total laryngopharyngectomy followed by immediate reconstruction of her neopharynx with a tubed anterolateral thigh flap. The postoperative course was complicated by a small pharyngocutaneous fistula which was managed conservatively and resolved by six weeks. The patient had a good cosmetic and functional outcome, though she died nine months postoperatively due to complications unrelated to her prior surgeries. Case 2 A 19-year-old male with severe FA and a history of bone marrow transplantation presented with extensive SCC of the mobile tongue requiring subtotal (~90%) glossectomy. His defect was immediately reconstructed with a right anterolateral thigh chimeric free flap, with the vastus lateralis used to recreate the floor of the mouth and the fasciocutaneous tissue providing the bulk of the tongue. Despite an excellent initial outcome, however, he had a recurrence six months later and required resection of the floor of the mouth (including the anterior portion of his prior reconstruction), segmental mandible, and neck with a left osteomyocutaneous free flap. Although he was tolerating PO initially after the second flap, he required tube feeds after undergoing radiotherapy, and seventeen months postoperatively he has a satisfactory cosmetic outcome. CONCLUSIONS: Patients with FA will often require reconstruction of a second devastating head and neck SCC. This small case series is the first to demonstrate that patients with FA are not only potential candidates for microvascular free flap reconstruction but that they may undergo multiple such procedures with acceptable functional and cosmetic outcomes with the appropriate perioperative management.
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