RESUMO
Neurofibromatosis type 1 (NF1) is the most common form of neurofibromatosis. It is associated with neurofibromas, gliomas, neurofibrosarcomas, and neuroendocrine and hematopoietic tumors. We present a case of scalp plexiform neurofibromatosis associated with intrathoracic fibrosarcoma. An 18-year-old female presented with a 15-year history of plexiform scalp mass. She had multiple café-au-lait patches on her trunk and extremities and a first-degree relative with a plexiform right shoulder mass. She was managed by a multidisciplinary team of plastic and reconstructive surgeons, neurosurgeons, cardiothoracic surgeons, otorhinolaryngologists, ophthalmologists, pulmonologists, and pathologists. The histology of the excised scalp mass was that of a malignant peripheral nerve sheath tumor (neurofibrosarcoma). She subsequently developed upper chest and back pain with associated breathlessness and was found to have an intra-thoracic tumor. She had two sessions of exploratory right thoracotomy with subtotal excision of an aggressive, highly hemorrhagic, infiltrative mucinous tumor. The histology was a fibrosarcoma. The patient died a few hours following the second thoracotomy. NF1 is associated with several tumors, among which are neurofibrosarcomas. Intra-thoracic fibrosarcoma requires aggressive surgical resection; recurrence may be delayed with radiotherapy and chemotherapy. The prognosis is however poor, and survival beyond one year is unusual. Once one tumor is found, other body systems should be evaluated for the possibility of other tumors.
RESUMO
BACKGROUND: Corrosive esophageal stricture is a major cause of morbidity among Nigerians. In most cases, this follows accidental or parasuicidal ingestion of caustic sodium hydroxide solution (NaOH) often used in the local production of soaps. Various treatment modalities have been advocated for the treatment of esophageal stricture. In this study, we review the results of our adopted technique in the past 10 years for pedicled colonic interposition. METHODS: This is a retrospective study of 21 patients who underwent substernal isoperistaltic pedicled colonic interposition graft for management of corrosive esophageal stricture. The right colon was pulled up into the neck in all the patients without resecting the strictured esophagus. RESULTS: Long segment strictures and multiple strictures were the main indications for the procedure. The mean duration of the procedure was 339.6 ± 71.1 min. The average intraoperative blood loss was 673.1 ± 398.1 mL. There were two (9.5 %) hospital mortalities. Graft infarction (9.5 %), cervical fistulae (19.0 %), and reflux neo-esophagitis (14.3 %) were the main non-fatal complications. In the mid-term, dysphagia was completely relieved in a little over 84 % (16/19) of patients, while one patient (4.8 %) still experienced reflux neo-esophagitis requiring treatment. There was no case of gross regurgitation or nocturnal aspiration in the mid-term. CONCLUSIONS: Although the use of pedicled colonic interposition graft offers a good mid-term functional outcome with relief of dysphagia, early postoperative morbidity is high. Graft infarction is the single most important factor for poor functional outcome and every effort must be made to prevent its occurrence.