Surgery for cystic lymphangioma in Gorlin-Goltz syndrome

被引:3
|
作者
Kayser, C.
Kayser, G.
Baier, P.
Hopt, U. T.
Eggstein, S.
机构
[1] Freiburg Univ Hosp, Dept Gen Surg, D-79106 Freiburg, Germany
[2] Freiburg Univ Hosp, Dept Pathol, D-79106 Freiburg, Germany
[3] Helios Hosp Titisee Neustadt, Dept Surg, Titisee Neustadt, Germany
关键词
Gorlin-Goltz syndrome; BCNS; mesenteric cysts; surgery; lymphangioma; PTCH; hedgehog;
D O I
10.1007/s00423-006-0126-5
中图分类号
R61 [外科手术学];
学科分类号
摘要
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.
引用
收藏
页码:203 / 207
页数:5
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