Alice Cherqui1, Wale A R Sulaiman, David G Kline. 1. Department of Neurosurgery, Louisiana State University Health Science Center, New Orleans, Louisiana 70112, USA. acherq@lsuhsc.edu
Abstract
OBJECTIVE AND IMPORTANCE: Our patient's symptomatology, history, physical examination, diagnosis, management, and functional outcome 1 year after surgical repair is presented and discussed in light of the current literature on lipofibrohamartomas. CLINICAL PRESENTATION: A 3-year-old boy presented to the Louisiana State University Nerve Clinic for evaluation and management because he was experiencing progressive symptoms of left hand swelling, dysesthesia, and impaired motor function. Physical examination demonstrated median nerve distribution motor impairment. Electromyographic/nerve conduction velocity studies also showed severely reduced conduction and amplitude of the median nerve response, and the magnetic resonance imaging findings were highly suggestive of lipofibrohamartoma. Hence, the presumed diagnosis was lipofibrohamartoma on the basis of imaging characteristics, location, and patient's age. INTERVENTION: The patient was brought to the operating room with the objectives of carpal tunnel release and biopsy. However, routine intraoperative nerve action potential recordings showed no or very poor responses, consistent with significant loss of median nerve function. On the basis of the intraoperative nerve action potentials, we opted to resect the tumor back to healthy median nerve fascicles and to perform graft repairs. Surgery proceeded uneventfully, without any complications. Pathology confirmed the diagnosis of lipofibrohamartoma. CONCLUSION: At 18 months postoperatively, the patient had excellent left hand function. On the basis of our experience with this patient, we believe that intraoperative nerve action potentials and the availability of usable proximal and distal nerve fascicles (which may be discernible on diagnostic imaging) are key factors in deciding whether a lipofibrohamartoma needs to be repaired or decompressed/biopsied. As illustrated by our case, we believe that resection and graft repair may be the best treatment option for some of these patients, and perhaps more so for pediatric patients.
OBJECTIVE AND IMPORTANCE: Our patient's symptomatology, history, physical examination, diagnosis, management, and functional outcome 1 year after surgical repair is presented and discussed in light of the current literature on lipofibrohamartomas. CLINICAL PRESENTATION: A 3-year-old boy presented to the Louisiana State University Nerve Clinic for evaluation and management because he was experiencing progressive symptoms of left hand swelling, dysesthesia, and impaired motor function. Physical examination demonstrated median nerve distribution motor impairment. Electromyographic/nerve conduction velocity studies also showed severely reduced conduction and amplitude of the median nerve response, and the magnetic resonance imaging findings were highly suggestive of lipofibrohamartoma. Hence, the presumed diagnosis was lipofibrohamartoma on the basis of imaging characteristics, location, and patient's age. INTERVENTION: The patient was brought to the operating room with the objectives of carpal tunnel release and biopsy. However, routine intraoperative nerve action potential recordings showed no or very poor responses, consistent with significant loss of median nerve function. On the basis of the intraoperative nerve action potentials, we opted to resect the tumor back to healthy median nerve fascicles and to perform graft repairs. Surgery proceeded uneventfully, without any complications. Pathology confirmed the diagnosis of lipofibrohamartoma. CONCLUSION: At 18 months postoperatively, the patient had excellent left hand function. On the basis of our experience with this patient, we believe that intraoperative nerve action potentials and the availability of usable proximal and distal nerve fascicles (which may be discernible on diagnostic imaging) are key factors in deciding whether a lipofibrohamartoma needs to be repaired or decompressed/biopsied. As illustrated by our case, we believe that resection and graft repair may be the best treatment option for some of these patients, and perhaps more so for pediatric patients.