The transsphenoidal resection of pediatric craniopharyngiomas: a case series

Clinical article

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  • 1 Department of Neurosurgery, University of Virginia Health System, Charlottesville, Virginia;
  • 2 Department of Neurological Surgery, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania;
  • 3 Department of Neurological Surgery, Children's Memorial Hospital, Feinberg School of Medicine, Northwestern University, Chicago, Illinois; and
  • 4 Department of Neurosurgery, Brigham and Women's Hospital, Boston, Massachusetts
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Object

The majority of pediatric craniopharyngiomas are treated using a transcranial approach. Although there is an increasing acceptance of transsphenoidal resection in adults, there are few reports describing this approach in the pediatric population. The purpose of this study is to report the outcomes after transsphenoidal surgery in a consecutive series of pediatric patients with craniopharyngiomas treated at a single institution with the goal of gross-total resection (GTR).

Methods

Twenty-three patients with pathologically proven craniopharyngiomas were identified who were 18 years of age or less at the time of surgery. The medical records and imaging studies of the patients were retrospectively reviewed. One patient who was lost to follow-up after surgery was excluded.

Results

Among the 22 patients included in the study, 11 underwent transsphenoidal surgery as the primary procedure and 11 underwent transsphenoidal surgery as a secondary procedure after a previous procedure. All patients had at least some sellar component to their tumor and all had either anterior or posterior pituitary dysfunction at presentation. In the entire cohort, a GTR was achieved in 15 (68%) of 22 patients, a radical subtotal resection in 4 (18%) of 22 patients, a subtotal resection in 1 patient, and a partial resection in 2 patients. The degree of resection was higher in the primary transsphenoidal group. After a mean follow-up of 82 months, 4 patients (18%) experienced recurrence. Recurrence occurred in 13% after GTR compared with 28.5% after all other degrees of resection. Tumor recurred in 9% of the primary transsphenoidal group and in 30% of patients who had undergone other therapies prior to the transsphenoidal operation. No patient who had panhypopituitarism experienced a gain of function postoperatively, 67% developed new panhypopituitarism, and 56% experienced new diabetes insipidus. Vision improved or normalized in 9 (64%) of 14 patients presenting with visual loss. Complications included 1 death 3 weeks postoperatively, 2 CSF leaks, and new obesity in 37%.

Conclusions

Transsphenoidal resection of pediatric craniopharyngiomas results in a high rate of both visual improvement and GTR with a low associated risk of recurrence. The transsphenoidal approach should be considered in selected pediatric patients with craniopharyngioma, especially those with infradiaphragmatic origin.

Abbreviations used in this paper: DI = diabetes insipidus; GKS = Gamma Knife surgery; GTR = gross-total resection; STR = subtotal resection; VPS = ventriculoperitoneal shunt.

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Contributor Notes

Address correspondence to: John A. Jane Jr., M.D., Department of Neurosurgery, University of Virginia Health System, Charlottesville, Virginia 22908. email: johnjanejr@virginia.edu.
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