RESUMEN
Holoprosencephaly is a rare disorder of embryological development that presents with midline clefting of the lip. The incidence has been estimated at approximately 1 in 15,000. Previously, infants with severe holoprosencephaly were thought to die within 1-2 years of birth and seldom to benefit from surgery. Survival has increased with improved perinatal care and support services. Parents often request complete cheiloplasty, because the presence of a columella greatly influences aesthetic outcome. The authors report two cases of simultaneous columella reconstruction with cheiloplasty.
Asunto(s)
Holoprosencefalia/cirugía , Labio/cirugía , Cartílagos Nasales/cirugía , Procedimientos de Cirugía Plástica/métodos , Labio Leporino/cirugía , Fisura del Paladar/cirugía , Femenino , Humanos , Lactante , MasculinoRESUMEN
The therapeutic effects of neo-adjuvant chemotherapy administered by two-route infusion of cisplatin and its antidote, sodium thiosulfate, and intravenous infusion of vinblastine and peplomycin were studied in 22 patients with previously untreated squamous cell carcinoma of the oral cavity or maxillary sinus. The overall response rate to chemotherapy was 90.9%, with a complete response in 12 patients (54.5%), a partial response in 8 patients (36.4%), and no change in 2 patients (9.1%). Histologic assessment showed a grade V response in 7 patients (31.8%), grade IV in 3 patients (13.6%), grade III in 5 patients (22.7%), grade II in 4 patients (18.2%), and grade I in 3 patients (13.6%). The cumulative 5-year survival rate, including 3 patients who died of another disease, was 72.2%. Leukocytopenia, an important side effect apparently induced by vinblastine, was seen, but no life-threatening complications occurred. Renal toxicity by cisplatin was minimal because of the use of sodium thiosulfate and fosfomycin. These data indicate that this mode of chemotherapy may be more effective for treating head and neck cancer than ordinary chemotherapy, from clinical and histologic standpoints. The high complete-response rate is especially noteworthy. No serious side effects or reductions in treatment period occurred. The results are preliminary and additional studies using a larger patient population are needed.