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  • Author or Editor: Daniel M. Prevedello x
  • Journal of Neurosurgery: Pediatrics x
  • By Author: Prevedello, Daniel M. x
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John A. Jane Jr., Daniel M. Prevedello, Tord D. Alden and Edward R. Laws Jr.

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.

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Hilal A. Kanaan, Paul A. Gardner, Gabrielle Yeaney, Daniel M. Prevedello, Edward A. Monaco III, Geoffrey Murdoch, Ian F. Pollack and Amin B. Kassam

Olfactory schwannomas are rare tumors of the anterior skull base that are possibly derived from ectopic Schwann cells, perivascular neural tissue, or sensory nerves of the meninges. The authors report the case of a 14-year-old boy with an olfactory schwannoma that extended inferiorly through the cranial base and superiorly into the frontal lobe. Because of the growth characteristics of the tumor and the significant overlying frontal lobe edema, the lesion was approached via an endonasal endoscopic route, as a strategy to minimize brain retraction. This tumor was characterized radiographically as contrast-enhancing with cystic areas and erosion into bone. The tumor showed immunoreactivity for S100 protein and leukocyte antigen 7 (CD57) but not epithelial membrane antigen, supporting the diagnosis of olfactory schwannoma. A gross-total resection was achieved. This approach represents a novel application of endoscopic endonasal surgery to the pediatric neurosurgical context, as well as a favorable outcome in an extremely unusual tumor type, that should be applicable to other appropriately selected pediatric brain tumors.