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  • Author or Editor: Elizabeth C. Gardner x
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Maria Koutourousiou, Paul A. Gardner, Juan C. Fernandez-Miranda, Elizabeth C. Tyler-Kabara, Eric W. Wang and Carl H. Snyderman

Object

The proximity of craniopharyngiomas to vital neurovascular structures and their high recurrence rates make them one of the most challenging and controversial management dilemmas in neurosurgery. Endoscopic endonasal surgery (EES) has recently been introduced as a treatment option for both pediatric and adult craniopharyngiomas. The object of the present study was to present the results of EES and analyze outcome in both the pediatric and the adult age groups.

Methods

The authors retrospectively reviewed the records of patients with craniopharyngioma who had undergone EES in the period from June 1999 to April 2011.

Results

Sixty-four patients, 47 adults and 17 children, were eligible for this study. Forty-seven patients had presented with primary craniopharyngiomas and 17 with recurrent tumors. The mean age in the adult group was 51 years (range 28–82 years); in the pediatric group, 9 years (range 4–18 years). Overall, the gross-total resection rate was 37.5% (24 patients); near-total resection (> 95% of tumor removed) was 34.4% (22 patients); subtotal resection (≥ 80% of tumor removed) 21.9% (14 patients); and partial resection (< 80% of tumor removed) 6.2% (4 patients). In 9 patients, EES had been combined with radiation therapy (with radiosurgery in 6 cases) as the initial treatment. Among the 40 patients (62.5%) who had presented with pituitary insufficiency, pituitary function remained unchanged in 19 (47.5%), improved or normalized in 8 (20%), and worsened in 13 (32.5%). In the 24 patients who had presented with normal pituitary function, new pituitary deficit occurred in 14 (58.3%). Nineteen patients (29.7%) suffered from diabetes insipidus at presentation, and the condition developed in 21 patients (46.7%) after treatment. Forty-four patients (68.8%) had presented with impaired vision. In 38 (86.4%) of them, vision improved or even normalized after surgery; in 5, it remained unchanged; and in 1, it temporarily worsened. One patient without preoperative visual problems showed temporary visual deterioration after treatment. Permanent visual deterioration occurred in no one after surgery. The mean follow-up was 38 months (range 1–135 months). Tumor recurrence after EES was discovered in 22 patients (34.4%) and was treated with repeat surgery (6 patients), radiosurgery (1 patient), combined repeat surgery and radiation therapy (8 patients), interferon (1 patient), or observation (6 patients). Surgical complications included 15 cases (23.4%) with CSF leakage that was treated with surgical reexploration (13 patients) and/or lumbar drain placement (9 patients). This leak rate was decreased to 10.6% in recent years after the introduction of the vascularized nasoseptal flap. Five cases (7.8%) of meningitis were found and treated with antibiotics without further complications. Postoperative hydrocephalus occurred in 7 patients (12.7%) and was treated with ventriculoperitoneal shunt placement. Five patients experienced transient cranial nerve palsies. There was no operative mortality.

Conclusions

With the goal of gross-total or maximum possible safe resection, EES can be used for the treatment of every craniopharyngioma, regardless of its location, size, and extension (excluding purely intraventricular tumors), and can provide acceptable results comparable to those for traditional craniotomies. Endoscopic endonasal surgery is not limited to adults and actually shows higher resection rates in the pediatric population.

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Srinivas Chivukula, Maria Koutourousiou, Carl H. Snyderman, Juan C. Fernandez-Miranda, Paul A. Gardner and Elizabeth C. Tyler-Kabara

Object

The use of endoscopic endonasal surgery (EES) for skull base pathologies in the pediatric population presents unique challenges and has not been well described. The authors reviewed their experience with endoscopic endonasal approaches in pediatric skull base surgery to assess surgical outcomes and complications in the context of presenting patient demographics and pathologies.

Methods

A retrospective review of 133 pediatric patients who underwent EES at our institution from July 1999 to May 2011 was performed.

Results

A total of 171 EESs were performed for skull base tumors in 112 patients and bony lesions in 21. Eighty-five patients (63.9%) were male, and the mean age at the time of surgery was 12.7 years (range 2.3–18.0 years). Skull base tumors included angiofibromas (n = 24), craniopharyngiomas (n = 16), Rathke cleft cysts (n = 12), pituitary adenomas (n = 11), chordomas/chondrosarcomas (n = 10), dermoid/epidermoid tumors (n = 9), and 30 other pathologies. In total, 19 tumors were malignant (17.0%). Among patients with follow-up data, gross-total resection was achieved in 16 cases of angiofibromas (76.2%), 9 of craniopharyngiomas (56.2%), 8 of Rathke cleft cysts (72.7%), 7 of pituitary adenomas (70%), 5 of chordomas/chondrosarcomas (50%), 6 of dermoid/epidermoid tumors (85.7%), and 9 cases of other pathologies (31%). Fourteen patients received adjuvant radiotherapy, and 5 received chemotherapy. Sixteen patients (15.4%) showed tumor recurrence and underwent reoperation. Bony abnormalities included skull base defects (n = 12), basilar invagination (n = 4), optic nerve compression (n = 3) and trauma (n = 2); preexisting neurological dysfunction resolved in 12 patients (57.1%), improved in 7 (33.3%), and remained unchanged in 2 (9.5%). Overall, complications included CSF leak in 14 cases (10.5%), meningitis in 5 (3.8%), transient diabetes insipidus in 8 patients (6.0%), and permanent diabetes insipidus in 12 (9.0%). Five patients (3.8%) had transient and 3 (2.3%) had permanent cranial nerve palsies. The mean follow-up time was 22.7 months (range 1–122 months); 5 patients were lost to follow-up.

Conclusions

Endoscopic endonasal surgery has proved to be a safe and feasible approach for the management of a variety of pediatric skull base pathologies. When appropriately indicated, EES may achieve optimal outcomes in the pediatric population.

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Francisco Vaz-Guimaraes, Maria Koutourousiou, John R. de Almeida, Elizabeth C. Tyler-Kabara, Juan C. Fernandez-Miranda, Eric W. Wang, Carl H. Snyderman and Paul A. Gardner

OBJECTIVE

Epidermoid and dermoid cysts may be found along the cranial base and are commonly resected via open transcranial approaches. The use of endoscopic endonasal approaches for resection of these tumors has been rarely reported.

METHODS

The authors retrospectively reviewed the medical records of 21 patients who underwent endoscopic endonasal surgery for epidermoid and dermoid cyst resection at the University of Pittsburgh Medical Center between January 2005 and June 2014. Surgical outcomes and variables that might affect the extent of resection and complications were analyzed.

RESULTS

Total resection (total removal of cyst contents and capsule) was achieved in 8 patients (38.1%), near-total resection (total removal of cyst contents, incomplete removal of cyst capsule) in 9 patients (42.9%), and subtotal resection (incomplete removal of cyst contents and capsule) in 4 patients (19%). Larger cyst volume (≥ 3 cm3) and intradural location (15 cysts) were significantly associated with nontotal resection (p = 0.008 and 0.0005, respectively). In the whole series, surgical complications were seen in 6 patients (28.6%). No complications were observed in patients with extradural cysts. Among the 15 patients with intradural cysts, the most common surgical complication was postoperative CSF leak (5 patients, 33.3%), followed by postoperative intracranial infection (4 patients, 26.7%). Larger cysts and postoperative CSF leak were associated with intracranial infection (p = 0.012 and 0.028, respectively). Subtotal resection was marginally associated with intracranial infection when compared with total resection (p = 0.091). All patients with neurological symptoms improved postoperatively with the exception of 1 patient with unchanged abducens nerve palsy.

CONCLUSIONS

Endoscopic endonasal approaches may be effectively used for resection of epidermoid and dermoid cysts in carefully selected cases. These approaches are recommended for cases in which a total or near-total resection is possible in addition to a multilayer cranial base reconstruction with vascularized tissue to minimize the risk of intracranial infection.

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Cheran Elangovan, Supriya Palwinder Singh, Paul Gardner, Carl Snyderman, Elizabeth C. Tyler-Kabara, Miguel Habeych, Donald Crammond, Jeffrey Balzer and Parthasarathy D. Thirumala

OBJECT

The aim of this study was to evaluate the value of intraoperative neurophysiological monitoring (IONM) using electromyography (EMG), brainstem auditory evoked potentials (BAEPs), and somatosensory evoked potentials (SSEPs) to predict and/or prevent postoperative neurological deficits in pediatric patients undergoing endoscopic endonasal surgery (EES) for skull base tumors.

METHODS

All consecutive pediatric patients with skull base tumors who underwent EES with at least 1 modality of IONM (BAEP, SSEP, and/or EMG) at our institution between 1999 and 2013 were retrospectively reviewed. Staged procedures and repeat procedures were identified and analyzed separately. To evaluate the diagnostic accuracy of significant free-run EMG activity, the prevalence of cranial nerve (CN) deficits and the sensitivity, specificity, and positive and negative predictive values were calculated.

RESULTS

A total of 129 patients underwent 159 procedures; 6 patients had a total of 9 CN deficits. The incidences of CN deficits based on the total number of nerves monitored in the groups with and without significant free-run EMG activity were 9% and 1.5%, respectively. The incidences of CN deficits in the groups with 1 staged and more than 1 staged EES were 1.5% and 29%, respectively. The sensitivity, specificity, and negative predictive values (with 95% confidence intervals) of significant EMG to detect CN deficits in repeat procedures were 0.55 (0.22–0.84), 0.86 (0.79–0.9), and 0.97 (0.92–0.99), respectively. Two patients had significant changes in their BAEPs that were reversible with an increase in mean arterial pressure.

CONCLUSIONS

IONM can be applied effectively and reliably during EES in children. EMG monitoring is specific for detecting CN deficits and can be an effective guide for dissecting these procedures. Triggered EMG should be elicited intraoperatively to check the integrity of the CNs during and after tumor resection. Given the anatomical complexity of pediatric EES and the unique challenges encountered, multimodal IONM can be a valuable adjunct to these procedures.

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Wendy Chen, Paul A. Gardner, Barton F. Branstetter, Shih-Dun Liu, Yue Fang Chang, Carl H. Snyderman, Jesse A. Goldstein, Elizabeth C. Tyler-Kabara and Lindsay A. Schuster

OBJECTIVE

Cranial base development plays a large role in anterior and vertical maxillary growth through 7 years of age, and the effect of early endonasal cranial base surgery on midface growth is unknown. The authors present their experience with pediatric endoscopic endonasal surgery (EES) and long-term midface growth.

METHODS

This is a retrospective review of cases where EES was performed from 2000 to 2016. Patients who underwent their first EES of the skull base before age 7 (prior to cranial suture fusion) and had a complete set of pre- and postoperative imaging studies (CT or MRI) with at least 1 year of follow-up were included. A radiologist performed measurements (sella-nasion [S-N] distance and angles between the sella, nasion, and the most concave points of the anterior maxilla [A point] or anterior mandibular synthesis [B point], the SNA, SNB, and ANB angles), which were compared to age- and sex-matched Bolton standards. A Z-score test was used; significance was set at p < 0.05.

RESULTS

The early surgery group had 11 patients, with an average follow-up of 5 years; the late surgery group had 33 patients. Most tumors were benign; 1 patient with a panclival arteriovenous malformation was a significant outlier for all measurements. Comparing the measurements obtained in the early surgery group to Bolton standard norms, the authors found no significant difference in postoperative SNA (p = 0.10), SNB (p = 0.14), or ANB (0.67) angles. The S-N distance was reduced both pre- and postoperatively (SD 1.5, p = 0.01 and p = 0.009). Sex had no significant effect. Compared to patients who had surgery after the age of 7 years, the early surgery group demonstrated no significant difference in pre- to postoperative changes with regard to S-N distance (p = 0.87), SNA angle (p = 0.89), or ANB angle (p = 0.14). Lesion type (craniopharyngioma, angiofibroma, and other types) had no significant effect in either age group.

CONCLUSIONS

Though our cohort of patients with skull base lesions demonstrated some abnormal measurements in the maxillary-mandibular relationship before their operation, their postoperative cephalometrics fell within the normal range and showed no significant difference from those of patients who underwent operations at an older age. Therefore, there appears to be no evidence of impact of endoscopic endonasal skull base surgery on craniofacial development within the growth period studied.