Transnasal surgery for infradiaphragmatic craniopharyngiomas in pediatric patients

Abstract

Objective: Transnasal surgery has been performed in only a small number of cases of pediatric craniopharyngiomas, and its efficacy is still a matter of debate.

Methods: We analyzed the results of transnasal surgery performed in 11 pediatric patients (6 male and 5 female patients) with infradiaphragmatic craniopharyngiomas who were younger than 15 years at the time of surgery (age range, 7.7-14.9 yr) and who were treated between 1985 and 1996, when more refined diagnostic and surgical techniques were available. Two of the patients had undergone primary surgery elsewhere. The transnasal approach was chosen because of sellar enlargement and the presence of an infradiaphragmatic tumor. The duration of the clinical follow-up period was at least 1 year.

Results: Five patients required drilling of incompletely pneumatized sphenoid sinuses to reach the sella, but in no cases were the anatomic differences related to patient age or size thought to be a limiting factor in the transnasal procedure. The normal pituitary gland was incised to expose a dorsally located tumor in each of eight patients (72.7%). This surgical procedure has never provoked any major functional damage to the anterior pituitary gland. Complete tumor resection was achieved in three patients (27.3%) and subtotal removal in eight (72.7%). Twenty-two (95.7%) of 23 normal functions were preserved after subtotal tumor removal. In contrast, only one of four normal functions was maintained after complete tumor resection. Persistent diabetes insipidus occurred after total tumor resection in one patient. Tumor regrowth occurred in two patients. No tumor recurrence was observed during the follow-up period.

Conclusion: Based on the present findings, transnasal surgery seems to be indicated for most infradiaphragmatic craniopharyngiomas occurring in pediatric patients. Transnasal surgery is as safe to perform in pediatric patients as it is in adult patients. The concept of subtotal tumor removal with preservation of pituitary function, avoiding damage to hypothalamic structures and excessive cerebrospinal fluid leakage, seems to be justified in pediatric patients.