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Scott D. Wait, Brendan D. Killory, Gregory P. Lekovic and Curtis A. Dickman

Object

Palmar, axillary, and plantar hyperhidrosis is often socially, emotionally, and physically disabling for adolescents. The authors report surgical outcomes in all adolescents treated for palmar hyperhidrosis via bilateral thoracoscopic sympathectomy at the Barrow Neurological Institute by the senior author.

Methods

A prospectively maintained database of all adolescent patients undergoing bilateral thoracoscopic sympathectomy between 1998 and 2006 (inclusive) was reviewed. Additional follow-up was obtained as needed in clinic or by phone or written questionnaire.

Results

Fifty-four patients (40 females) undergoing bilateral procedures were identified. Their mean age was 15.4 years (range 10–17 years). Average follow-up was 42 weeks (range 0.2–143 weeks). Hyperhidrosis involved the palms alone in 10 patients; the palms and axilla in 6 patients; the palms and plantar surfaces in 17 patients; and the palms, axilla, and plantar surfaces in 21 patients. Palmar hyperhidrosis resolved completely in 98.1% of the patients. Resolution or improvement of symptoms was seen in 96.3% of patients with axillary and 71.1% of those with plantar hyperhidrosis. Hospital stay averaged 0.37 days with 68.5% of patients discharged the day of surgery. One patient experienced brief intraoperative asystole that resolved with medications and had no long-term sequelae. Otherwise, no serious intraoperative complications occurred. No patient required chest tube drainage. The percentage of patients who reported satisfaction and willingness to undergo the procedure again was 98.1%.

Conclusions

Biportal, bilateral thoracoscopic sympathectomy is an effective and low-morbidity treatment for severe palmar, axillary, and plantar hyperhidrosis.

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Douglas R. Taylor, Scott D. Wait, James W. Wheless and Frederick A. Boop

Neurocutaneous melanosis (NCM) is a rare, congenital neuroectodermal dysplasia generally resulting in early death from neurological decline due to leptomeningeal involvement. Nonmeningeal CNS epileptogenic lesions presenting in later childhood in the absence of leptomeningeal disease are rare. This report summarizes a rare presentation and curative epilepsy surgery.

The authors discuss the case of a 14-year-old girl with NCM who originally presented with intractable partial-onset seizures. The MRI, PET, and SPECT studies subsequently revealed a focal epileptogenic source in the right temporal lobe. Results of video-electroencephalography monitoring concurred with the imaging findings, and a right temporal lobectomy was performed including the medial structures. Following surgery, histopathological features of the lesion included multiple scattered mononuclear cells with brown pigmentation in the amygdala specimen. The patient remains seizure free 2 years postresection, and no longer needs medication for seizure management.

This patient presented with an atypical CNS manifestation of NCM that is curable by epilepsy surgery. Her intractable epilepsy developed secondary to amygdalar neuromelanosis, which had no associated leptomeningeal melanosis, an uncommon occurrence. As evidenced by the lack of seizure activity following resection, the patient's quality of life greatly improved after neurological surgery.

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Francisco A. Ponce, Brendan D. Killory, Scott D. Wait, Nicholas Theodore and Curtis A. Dickman

Object

Thoracoscopy may be used in place of thoracotomy to resect intrathoracic neoplasms such as paraspinal neurogenic tumors. Although these tumors are rare, they account for the majority of tumors arising in the posterior mediastinum.

Methods

A database was maintained of all patients undergoing thoracoscopic surgery for tumors. The authors analyzed the presenting symptoms, pathological diagnoses, and outcomes of 26 patients (7 males and 19 females, mean age 37.2 years) who were treated for intrathoracic tumors via thoracoscopy between January 1995 and May 2009. Fourteen patients were diagnosed incidentally (54%). Five patients (19%) presented with dyspnea or shortness of breath, 4 (15%) with pain, 1 (4%) with pneumonia, 1 (4%) with hoarseness, and 1 (4%) with Horner syndrome.

Results

Pathology demonstrated schwannomas in 20 patients (77%). Other diagnoses included ganglioneurofibroma, paraganglioma, epithelioid angiosarcoma, benign hemangioma, benign granular cell tumor, and infectious granuloma. One patient required conversion to open thoracotomy due to pleural scarring to the tumor. One underwent initial laminectomy due to intraspinal extension of the tumor. Gross-total resection was obtained in 25 cases (96%). The remaining patient underwent biopsy followed by radiation therapy. The mean surgical time was 2.5 hours, and the mean blood loss was 243 ml. The mean duration of chest tube insertion was 1.3 days, and the mean length of hospital stay was 3.0 days. Cases that were treated in the second half of the cohort were more often diagnosed incidentally, performed in less time, and had less blood loss than those in the first half of the cohort. There was 1 case of permanent treatment-related morbidity (mild Horner syndrome). All previously employed patients were able to return to work (mean clinical follow-up 43 months). There were no recurrences (mean imaging follow-up 54 months).

Conclusions

Endoscopic transthoracic approaches can reduce approach-related soft-tissue morbidity and facilitate recovery by preserving the normal tissues of the chest wall, by avoiding rib retraction and muscle transection, and by reducing postoperative pain. This less invasive approach thus shortens hospital stay and recovery time.

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Scott D. Wait, Francisco A. Ponce, Brendan D. Killory, Donna Wallace and Harold L. Rekate

Neuroleptic malignant syndrome (NMS) is a potentially life-threatening entity characterized by hyperthermia, autonomic deregulation, decreased mental status, increased muscle tone, and, frequently, by renal failure due to rhabdomyolysis. Classically, it follows administration of antipsychotic medication.

The authors report on 4 patients (2 children and 2 adults) in whom NMS was diagnosed after a CNS insult. No patient was receiving antipsychotic medication. The patients' hospital and clinic charts, radiographic data, and follow-up telephone conversations were reviewed retrospectively.

All 4 patients met diagnostic criteria for NMS. Three patients presented with shunt failure, and 1 patient had undergone a functional hemispherectomy 2 days earlier. One patient with shunt failure received the diagnosis retrospectively. An endoscopic third ventriculostomy alleviated his shunt failure and he remains free of NMS. The other 2 patients underwent treatment for shunt failure, but NMS remained. These 2 patients and the one who had undergone hemispherectomy underwent a trial of intrathecal baclofen, and the NMS resolved. Subsequently, an intrathecal baclofen infusion device was placed in all 3 patients, and the NMS resolved. The 2 patients in shunt failure had a lumbar intrathecal baclofen infusion device. The patient who had undergone hemispherectomy had an intracranial baclofen catheter.

Neuroleptic malignant syndrome is a rare, life-threatening disorder that can occur without the administration of neuroleptic medications. Alleviation of any CNS insult is the first order of treatment. Some patients with persistent symptoms of NMS may benefit from intrathecal delivery of baclofen.

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Scott D. Wait, Adib A. Abla, Brendan D. Killory, Peter Nakaji and Harold L. Rekate

Object

Hypothalamic hamartomas (HHs) are devastating lesions causing refractory epilepsy, rage attacks, social ineptitude, and precocious puberty. Microsurgical and/or endoscopic resection offers an excellent risk/benefit profile for cure or improvement of epilepsy.

Methods

The authors reviewed a prospective database maintained during the first 7 years of the Barrow Hypothalamic Hamartoma program. They describe and illustrate their surgical methods, and they review data from several previous publications regarding surgical outcome.

Results

To date, the authors have performed surgery in 165 patients for symptomatic HHs. Patients underwent an endoscopic, transcallosal, or skull base approach, or multiple approaches. Twenty-six patients (15.8%) required more than 1 treatment for their HH.

Conclusions

Microsurgical and endoscopic resection of symptomatic HHs are technically demanding but can be performed safely with excellent results and an acceptable risk profile. Meticulous attention to the subtleties of surgical management helps optimize outcomes.

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Francisco A. Ponce, Robert F. Spetzler, Patrick P. Han, Scott D. Wait, Brendan D. Killory, Peter Nakaji and Joseph M. Zabramski

Object

The aim of this study was to clarify the surgical indications, risks, and long-term clinical outcomes associated with the use of deep hypothermic circulatory arrest for the surgical treatment of intracranial aneurysms.

Methods

The authors retrospectively reviewed 105 deep hypothermic circulatory arrest procedures performed in 103 patients (64 females and 39 males, with a mean age of 44.8 years) to treat 104 separate aneurysms. Patients' clinical histories, radiographs, and operative reports were evaluated. There were 97 posterior circulation aneurysms: at the basilar apex in 60 patients, midbasilar artery in 21, vertebrobasilar junction in 11, superior cerebellar artery in 4, and posterior cerebral artery in 1. Seven patients harbored anterior circulation aneurysms. Two additional patients harbored nonaneurysmal lesions.

Results

Perioperatively, 14 patients (14%) died. Five patients (5%) were lost to late follow-up. At a mean long-term follow-up of 9.7 years, 65 patients (63%) had the same or a better status after surgical intervention, 10 (10%) were worse, and 9 (9%) had died. There were 19 cases (18%) of permanent or severe complications. The combined rate of permanent treatment-related morbidity and mortality was 32%. The mean late follow-up Glasgow Outcome Scale score was 4, and the annual hemorrhage rate after microsurgical clipping during cardiac standstill was 0.5%/year. Ninety-two percent of patients required no further treatment of their aneurysm at the long-term follow-up.

Conclusions

Cardiac standstill remains an important treatment option for a small subset of complex and giant posterior circulation aneurysms. Compared with the natural history of the disease, the risk associated with this procedure is acceptable.

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Scott D. Wait, Adib A. Abla, Brendan D. Killory, Robert M. Starke, Robert F. Spetzler and Peter Nakaji

Object

Many patients undergoing carotid endarterectomy (CEA) regularly take clopidogrel, a permanent platelet inhibitor. The authors sought to determine whether taking clopidogrel in the period before CEA leads to more bleeding or other complications.

Methods

The authors performed a retrospective, institutional review board–approved review of 182 consecutive patients who underwent CEA. Clinical, radiographic, and surgical data were gleaned from hospital and clinic records. Analysis was based on the presence or absence of clopidogrel in patients undergoing CEA and was performed twice by considering clopidogrel use within 8 days and within 5 days of surgery to define the groups.

Results

Taking clopidogrel within 8 days before surgery resulted in no statistical increase in any measure of morbidity or death. Taking clopidogrel within 5 days was associated with a small but significant increase in operative blood loss and conservatively managed postoperative neck swelling. No measure of permanent morbidity or death was increased in either clopidogrel group.

Conclusions

Findings in this study support the safety of preoperative clopidogrel in patients undergoing CEA.

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Weier Li, Scott D. Wait, Robert J. Ogg, Matt A. Scoggins, Ping Zou, James Wheless and Frederick A. Boop

Object

Advances in brain imaging have allowed for more sophisticated mapping of crucial neural structures. Functional MRI (fMRI) measures local changes in blood oxygenation associated with changes in neural activity and is useful in mapping cortical activation. Applications of this imaging modality have generally been restricted to cooperative patients; however, fMRI has proven successful in localizing the motor cortex for neurosurgical planning in uncooperative children under sedation. The authors demonstrate that the use of fMRI to localize the visual cortex in sedated children can be safely and effectively performed, allowing for more accurate presurgical planning to spare visual structures.

Methods

Between 2007 and 2009, 11 children (age range 1–11 years) underwent fMRI for neurosurgical planning while under sedation. Blood oxygen level–dependent fMRI was performed to detect visual cortex activation during stimulation through closed eyelids. Visual stimulation was presented in block design with periods of flashing light alternated with darkness.

Results

Functional MRI was successful in identifying visual cortex in each of the 11 children tested. There were no complications with propofol sedation or the fMRI. All children suffered from epilepsy, 5 had brain tumors, and 1 had tuberous sclerosis. After fMRI was performed, 6 patients underwent surgery. Frameless stereotactic guidance was synchronized with fMRI data to design an approach to spare visual structures during resection. There were no cases where a false negative led to unexpected visual field deficits or other side effects of surgery. In 2 cases, the fMRI results demonstrated that the tracts were already disrupted: in one case from a prior tumor operation and in another from dysplasia.

Conclusions

Functional MRI for evaluation of visual pathways can be safely and reproducibly performed in young or uncooperative children under light sedation. Identification of primary visual cortex aids in presurgical planning to avoid vision loss in appropriately selected patients.

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Adib A. Abla, Scott D. Wait, Jonathan A. Forbes, Sandipan Pati, Roger E. Johnsonbaugh, John F. Kerrigan and Yu-Tze Ng

Object

In this paper, the authors' goal was to describe the occurrence of alternating hypernatremia and hyponatremia in pediatric patients who underwent resection of hypothalamic hamartomas (HHs) for epilepsy. Hypernatremia in patients after pituitary or hypothalamic surgery can be caused by diabetes insipidus (DI), whereas hyponatremia can occur due to a syndrome of inappropriate antidiuretic hormone, cerebral salt wasting, or excessive administration of desmopressin (DDAVP). The triphasic response after surgery in the pituitary region can also explain variations in sodium parameters in such cases.

Methods

One hundred fifty-three patients with HH who underwent surgery were enrolled in a prospective study to monitor outcomes. Of these, 4 patients (2.6%) were noted to experience dramatic alterations in serum sodium values. The medical records of these patients were identified and evaluated.

Results

Patients' ages at surgery ranged from 1.2 to 6.0 years. All patients were girls. Two patients had Delalande Type IV lesions (of 16 total Type IV lesions surgically treated) and 2 had Type III lesions (of 39 total Type III lesions). All patients had a history of gelastic seizures refractory to medication. Seizure frequency ranged from 3 to 300 per day. After surgery, all patients experienced hypernatremia and hyponatremia. The largest fluctuation in serum sodium concentration during hospitalization in a single patient was 53 mEq/L (range 123–176 mEq/L). The mean absolute difference in maximum and minimum sodium values was 38.2 mEq/L.

All patients exhibited an initial period of immediate DI (independent of treatment) after surgery followed by a period of hyponatremia (independent of treatment), with a minimum value occurring between postoperative Days 5 and 8. All patients then returned to a hypernatremic state of DI, and 3 patients still require DDAVP for DI management. A second occurrence of hyponatremia lasting several days without DDAVP administration occurred in 2 patients during their hospitalization between periods of hypernatremia. One patient stabilized in the normal range of sodium values prior to discharge from rehabilitation without the need for further intervention. At last follow-up, 3 patients are seizure-free.

Conclusions

Severe instability of sodium homeostasis with hypernatremia and hyponatremia is seen in up to 2.6% of children undergoing open resection of HH. This risk appears to be related to HH type, with a higher risk for Types III (2 [5.1%] of 39) and IV (2 [12.5%] of 16) lesions. Here, the authors describe alternating episodes of hypernatremia and hyponatremia in the postoperative period following HH surgery. Management of this entity requires careful serial assessment of volume status and urine concentration and will often require alternating salt replacement therapy with DDAVP administration.

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Ryszard M. Pluta, Scott D. Wait, John A. Butman, Kathleen A. Leppig, Alexander O. Vortmeyer, Edward H. Oldfield and Russell R. Lonser

Hemangioblastomas are histologically benign neoplasms that occur sporadically or as part of von Hippel–Lindau disease. Hemangioblastomas may occur anywhere along the neuraxis, but sacral hemangioblastomas are extremely rare. To identify features that will help guide the operative and clinical management of these lesions, the authors describe the management of a large von Hippel–Lindau disease–associated sacral hemangioblastoma and review the literature.

The authors present the case of a 38-year-old woman with von Hippel–Lindau disease and a 10-year history of progressive back pain, as well as left lower-extremity pain and numbness. Neurological examination revealed decreased sensation in the left S-1 and S-2 dermatomes. Magnetic resonance imaging demonstrated a large enhancing lesion in the sacral region, with associated erosion of the sacrum. The patient underwent arteriography and embolization of the tumor and then resection. The histopathological diagnosis was consistent with hemangioblastoma and showed intrafascicular tumor infiltration of the S-2 nerve root. At 1-year follow-up examination, pain had resolved and numbness improved.

Sacral nerve root hemangioblastomas may be safely removed in most patients, resulting in stabilization or improvement in symptomatology. Generally, hemangioblastomas of the sacral nerve roots should be removed when they cause symptoms. Because they originate from the nerve root, the nerve root from which the hemangioblastoma originates must be sacrificed to achieve complete resection.