RESUMO
Wolf-Hirschhorn syndrome is a rare genetic condition characterized by typical facial appearance, growth delay, psychomotor retardation and seizures, with a mosaic of other abnormalities reported in the literature. The occurrence of symptomatic wandering spleen with massive splenomegaly and with an indication for splenectomy has not been yet described for this disease. This study reports the first case in the literature of single port splenectomy for this rare condition. In a 21-year-old female patient with Wolf-Hirschhorn syndrome, with abdominal pain and the diagnosis of wandering spleen with splenomegaly (25 cm diameter) led to an indication of elective splenectomy. In supine position under general anesthesia, single port umbilical splenectomy was performed without laparoscopic assistance, splenic vessels were ligated by sutures, and the specimen was transumbilically extracted. Operative time was 85 min, with minimal bleeding, and resumed oral intake on the same day. No intraoperative or post-operative complications occurred, and the patient was discharged in 48 h. Single port access splenectomy is feasible and is evolving as an attractive alternative therapy for hematological diseases requiring splenectomy.