The clinical characteristics and surgical treatment of infradiaphragmatic craniopharyngioma in children

Objective To analyze the clinical characteristics and surgical treatment strategy of infradiaphragmatic craniopharyngiomas in children. Methods A retrospective analysis was conducted on 68 cases of childhood-onset infradiaphragmatic craniopharyngiomas who underwent surgical treatment at Department of Neurosurgery, Nanfang Hospital of Southern Medical University from December 2000 to January 2015. Among them, 19 patients received transsphenoidal surgery, 49 cases underwent craniotomy including 12 through lateral approach and 37 through anterior interhemispheric fissure approach. All patients were clinically followed up post operation. Analysis was conducted to explore the factors affecting the surgical operations, approaches and recurrence of infradiaphragmatic craniopharyngiomas. Results Infradiaphragmatic craniopharyngioma was common in childhood-onset craniopharyngiomas and accounted for 41.7%(68/163). Growth retardation and impaired vision were two most common clinical manifestations which accounted for 86.8%(59/68) and 69.1%(47/68), respectively. There were 58.8%(40/68) of the patients who presented pre-operative panhypopituitarism. The follow-up ranged from 18 to 270 months (mean: 71.4±53.4 month), and 13 out of 68 (19.1%) patients showed recurrence during the follow-up, 20 out of 28 (71.4%) non-panhypopituitarism patients presented newly onset deficiency of pituitary hormones. Multiple factors, including the tumor size bigger than 10 cm3, ratio of suprasellar tumor diameter to intrasellar tumor diameter >2, sagittal maximal tumor diameter greater than 4 cm, tumor with suprasellar complex tumor cysts, were more likely to result in craniotomy (all P<0.05). Tumor resection not along the membrane boundary of intrasellar part and the maximal diameter larger than 2.5 cm were two independent risk factors for recurrence (all P<0.05). Conclusions Infradiaphragmatic craniopharyngioma have negative impacts upon the patients’ quality of life. Exposure and removal of the intrasellar part of tumor could reduce the risk of recurrence for either transsphenoidal operation or craniotomy. Anterior interhemispheric fissure approach seems to be helpful for the exposure of intrasellar tumor and superior to the lateral approach. Key words: Craniopharyngioma; Neoplasm recurrence, local; Infradiaphragmatic; Child; Surgical approach; Panhypopituitarism