Transsphenoidal microsurgery for pediatric craniopharyngioma: special considerations regarding indications and method

Abstract

Although the transsphenoidal approach offers a narrow operative field and poor visual control through a small sellar opening, this approach has the advantage of being associated with minimal surgical trauma, especially in children. We share our experience of good surgical outcome achieved by the transsphenoidal approach in selected craniopharyngiomas in childhood, and report on our pediatric series of transsphenoidal complete removals of six relatively large craniopharyngiomas and one Rathke's cleft cyst. The most common presenting symptom was visual dysfunction (6/7). All tumors were 'prechiasmatic' cystic masses with moderate to marked suprasellar extensions and elevated diaphragm sellae. The tumors measured from 25 to 37 mm in maximum diameter in the midsagittal plane (median 33 mm). In 5 of the 7 tumors, the suprasellar portion (range 11-27 mm) was larger than the intrasellar portion (range 8-14 mm). Gross total resection was achieved in all patients. Special attention should be paid to multicystic craniopharyngiomas to prevent the possibility of incomplete tumor resection. The 'bone in a fat pocket' method was useful for preventing postoperative cerebrospinal fluid leakage. Vision was improved in all six patients who had preoperative visual disturbances. Hypopituitarism was provoked by radical tumor removal in all patients and managed by hormonal supplementation therapy. Transsphenoidal surgery is an appropriate approach for the radical excision of intrasellar-suprasellar 'prechiasmatic' craniopharyngiomas, even in children, and even if the tumor has a relatively large suprasellar component.