Sequential Second Free Flap for Head and Neck Reconstruction in a Patient with Fanconi Anemia and Metachronous Squamous Cell Carcinoma

Abstract

Sir: Fanconi anemia is a rare genetic disease characterized by chromosomal instability, bone marrow failure, congenital malformations, and increased susceptibility to both solid and leukemic malignancies. The incidence of solid malignancies approaches 28 percent by 40 years of age, with a high proportion of these being head and neck tumors.1 In a series of 754 patients with Fanconi anemia, Kutler et al. reported that 3 percent developed head and neck squamous cell carcinoma, with a 50 percent relapse rate by the age of 40.2 Although Fanconi anemia patients may develop multiple tumors over a lifetime, repeated operations/ reconstructions are rarely seen because of their shortened life expectancies. Furthermore, surgeons may be hesitant to treat patients with Fanconi anemia because of an increased risk of complications, particularly if treatment involves extensive resection or possible free tissue transfer. We have previously described a successful mandibular reconstruction with a free fibula free flap in a patient with Fanconi anemia and squamous cell carcinoma of the mandible.3 Unfortunately, the patient developed squamous cell carcinoma of the pharynx 18 months later. We now report subsequent pharyngeal reconstruction with a metachronous free flap. Initially, a 43-year-old woman with Fanconi anemia was referred to our institution with an erosive floor-of-mouth mass ultimately diagnosed as squamous cell carcinoma. She underwent hemimandibulectomy and bilateral neck dissection followed by immediate reconstruction with a right fibular osteomyocutaneous flap. The patient recovered from surgery without complications and underwent adjuvant radiation therapy. Her postoperative result was satisfactory. Eighteen months later, she was found to have squamous cell carcinoma of the hypopharynx. She then underwent total laryngopharyngectomy and construction of a neopharynx using a tubed anterolateral thigh flap. The soft tissues of the right neck were resurfaced with a separate skin paddle based off of a second perforator; the left lateral neck received a split-thickness skin graft. The patient recovered without any hematologic complications but did suffer partial skin graft loss and a small pharyngocutaneous fistula. A modified barium swallow performed 6 weeks postoperatively demonstrated a small leak that resolved 1 month later, resulting in satisfactory cosmetic and functional outcomes. As recommended by our hematologists, during both hospitalizations, the patient's perioperative hemoglobin level and platelet count were maintained above 7 g/dl and 50,000 cells/ml, respectively, by means of hematopoiesis-stimulating agents and blood product transfusions. A total of 42 units of single-donor platelets and 13 units of packed red blood cells were administered while the patient was hospitalized after the second free flap. In conclusion, patients with Fanconi anemia are at increased risk for perioperative hematologic complications. There are two published reports that describe successful free tissue transfer in Fanconi anemia patients.3,4 To our knowledge, this is the first reported case of a patient with Fanconi anemia to undergo a second sequential free flap operation for metachronous head and neck cancer reconstruction. As this case demonstrates, such large-scale reconstructions can be successfully undertaken with careful perioperative management of patients with this disease. Karly A. Kaplan, M.D. Alyssa J. Reiffel, M.D. David I. Kutler, M.D. Christine H. Rohde, M.D. Jason A. Spector, M.D.