Surgery for cystic lymphangioma in Gorlin-Goltz syndrome

Introduction We present a case of three major lymphomesenteric cysts in a female patient with known basal cell nevus syndrome (BCNS or Gorlin-Goltz syndrome). Discussion Although those cysts have been reported to appear in this syndrome, the exact prevalence is not known. In this case, the patient suffered from severe and recurrent abdominal pain due to bowel obstruction. Conclusion By causing severe symptoms, cystic lymphangiomas are an important complication of the BCNS despite of their seldom occurrence. Surgeons confronted with intraabdominal masses in a patient with BCNS should always be aware of this manifestation of the disorder and plan interdisciplinary operations.