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Jason I. Lifshutz and Walter D. Johnson

Hydrocephalus has amazed and challenged clinicians throughout the history of medicine. In reviewing the treatment of hydrocephalus, the integral relationship between basic science and therapy is reaffirmed. As we embark into a new millennium, it is appropriate to reflect on the past studies of this disorder, review various attempted and currently used, and finally speculate on possible future directions in its treatment.

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Recurrent spinal hydatidosis in North America

Case report and review of the literature

Gregory D. Schnepper and Walter D. Johnson

Spinal hydatidosis is an uncommon manifestation of the parasite Echinococcus, affecting fewer than 1% of patients with hydatid disease. The authors report on a 34-year-old Turkish woman who presented with recurrent primary spinal hydatid disease. The patient originally presented with progressive numbness and paraparesis that was reversed after T5–6 laminectomy and cyst removal. Pathological findings indicated parasitic infection and she underwent treatment for cysticercosis. Nevertheless, she returned 4 years later with back pain, numbness, and monoparesis. Neuroimaging studies revealed spinal cord compression with multiple cysts that were again resected. Pathological findings were consistent with Echinococcus.

Although this disease is uncommon, particularly in North America, the authors conclude that spinal hydatidosis should be considered in the differential diagnosis of any patient who has lived or traveled within endemic areas and who presents with spine lesions and cord compression. The authors review the literature pertaining to the epidemiological features, presentation, diagnosis, neuroimaging characteristics, recommended treatments, and overall prognosis of spinal hydatidosis.

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Thomas H. Milhorat, Walter D. Johnson and John I. Miller

✓ Syrinx shunts to the spinal subarachnoid space are likely to fail if the cerebrospinal fluid pathways rostral to the syrinx are blocked. To bypass obstructions at or below the level of the foramen magnum, a technique was developed for shunting the syrinx to the posterior fossa cisterns, termed “syringocisternostomy.” Syrinxes were shunted to the cisterna magna in two patients with spinal arachnoiditis and to the cerebellopontine angle cistern in four patients with Chiari I malformations. There was symptomatic improvement and collapse of the syrinx in each case, with no complications or recurrences over a follow-up interval of 14 to 27 months (average 20.3 months). The surgical technique and results of treatment are described.

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Walter D. Johnson and Michelle M. Petrie

Cobb syndrome is a rare clinical entity that includes the combination of a vascular skin nevus and an angioma in the spinal canal present at identical dermatomal level(s) (cutaneomeningospinal angiomatosis). To date, 38 cases have been reported, only 18 of which are in adults (> 18 years of age). The majority of these cases have been described in the era predating current neuroimaging techniques, and most authors have assumed that each case involves similar vascular pathology. This report highlights 2 patients presenting with similar thoracic cutaneous vascular nevi yet with markedly differing spinal vascular pathology.

A 29-year-old man presented with cutaneous hemangioma and a progressive paraparesis and paresthesia of the lower extremities. A 20 × 20–cm port-wine stain over his right upper midback (T6–10) correlated precisely with MR imaging that demonstrated an enhancing epidural mass between T-6 and T-10 causing compression of the cord and cord edema. A 34-year-old man also presented with progressive myelopathy and a 15 × 20–cm port-wine stain within the same dermatomal region as a Type III spinal arteriovenous malformation.

Workup for each patient included pre- and postoperative contrast-enhanced MR imaging with vascular sequencing and spinal angiography. The first patient was treated with bilateral laminectomy at the T6–10 levels, with significant postoperative improvement in motor strength. The second patient underwent coil embolization of an intranidal aneurysm, with follow-up embolization 8 years later.

Cobb syndrome is an unusual entity in the adult population and should be considered when there is a constellation of cutaneous manifestation and underlying neurological deficit. The vascular skin nevus associated with Cobb syndrome is accompanied by a wide variety of vascular pathologies.

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Samer Ghostine, Michelle S. Ghostine and Walter D. Johnson

✓ The treatment of pituitary tumors has progressed into a multidisciplinary approach that involves neurosurgeons, radiation oncologists, and endocrinologists. This has allowed improved outcomes in treatment of pituitary tumors due to a combination of surgical, medical, and radiation therapies. In this study, the authors review the role of radiation therapy in the treatment of pituitary adenomas.

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Walter D. Johnson, Lilia N. Loredo and Jerry D. Slater

✓ Historically, radiation therapy has been used extensively in the treatment of malignant and aggressive intracranial tumors, and the importance of its role has been repeatedly verified by prolonged patient survival rates and increased tumor control. As more modern capabilities are employed in surgery and radiotherapy, attention is being directed to the utility of radiation as either primary or secondary treatment of benign tumors. Specifically, primary treatment encompasses irradiation of small benign tumors without biopsy confirmation of tumor type; secondary treatment involves postoperative radiation therapy, with the possibility that less-aggressive tumor resection may be performed in areas that have a higher probability of resultant neurological deficit. Current literature suggests that this is not only a possible treatment strategy, but that it may be superior to more radical resection in some cases, for example, in vestibular schwannomas and meningiomas. This article provides an overview of factors to consider in the use of radiation therapy and reviews the relationships between radiation and surgery, notably the unique complementary role each plays in the treatment of benign intracranial tumors.

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Gail Rosseau, Walter D. Johnson, Kee B. Park, Miguel Arráez Sánchez, Franco Servadei and Kerry A. Vaughan

Since the creation of the World Health Organization (WHO) in 1948, the annual World Health Assembly (WHA) has been the major forum for discussion, debate, and approval of the global health agenda. As such, it informs the framework for the policies and budgets of many of its Member States. For most of its history, a significant portion of the attention of health ministers and Member States has been given to issues of clean water, vaccination, and communicable diseases. For neurosurgeons, the adoption of WHA Resolution 68.15 changed the global health landscape because the importance of surgical care for universal health coverage was highlighted in the document. This resolution was adopted in 2015, shortly after the publication of The Lancet Commission on Global Surgery Report titled “Global Surgery 2030: evidence and solutions for achieving health, welfare and economic development.” Mandating global strengthening of emergency and essential surgical care and anesthesia, this resolution has led to the formation of surgical and anesthesia collaborations that center on WHO and can be facilitated via the WHA. Participation by neurosurgeons has grown dramatically, in part due to the official relations between WHO and the World Federation of Neurosurgical Societies, with the result that global neurosurgery is gaining momentum.

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Walter J. Hader, James Drake, Douglas Cochrane, Owen Sparrow, Edward S. Johnson and John Kestle

✓ Late failure following successful third ventriculostomy for obstructive hydrocephalus is rare, and death caused by failure of a previously successful third ventriculostomy has been reported only once. The authors present three patients who died as a result of increased intracranial pressure (ICP) after late failure of a third ventriculostomy.

Through a collaborative effort, three patients were identified who had died following third ventriculostomy at one of the authors' institutions. A 13-year-old girl with neurofibromatosis Type 1 underwent third ventriculostomy for obstructive hydrocephalus caused by a tectal lesion. Three years later her condition deteriorated rapidly over the course of 6 hours and she was found dead at home. A 4-year-old boy treated with third ventriculostomy for aqueductal stenosis presented 2 years postoperatively with symptoms of increased ICP. This patient suffered a cardiorespiratory arrest while under observation and died despite external ventricular drainage. A 10-year-old boy with previous ventriculoperitoneal (VP) shunt placement underwent conversion to a third ventriculostomy and shunt removal. Eight months after the procedure his condition deteriorated, with evidence of raised ICP, and he underwent emergency insertion of another VP shunt, but remained in a vegetative state and died of complications. Neuropathological examinations in two cases demonstrated that the third ventriculostomy was not patent, and there was also evidence of increased ICP.

Late failure of third ventriculostomy resulting in death is a rare complication. Delay in recognition of recurrent ICP symptoms and a false feeling of security on the part of family and caregivers because of the absence of a shunt and the belief that the hydrocephalus has been cured may contribute to fatal complications after third ventriculostomy. Patients with third ventriculostomies should be followed in a manner similar to patients with cerebrospinal fluid shunts.

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Michael C. Dewan, Abbas Rattani, Graham Fieggen, Miguel A. Arraez, Franco Servadei, Frederick A. Boop, Walter D. Johnson, Benjamin C. Warf and Kee B. Park

OBJECTIVE

Worldwide disparities in the provision of surgical care result in otherwise preventable disability and death. There is a growing need to quantify the global burden of neurosurgical disease specifically, and the workforce necessary to meet this demand.

METHODS

Results from a multinational collaborative effort to describe the global neurosurgical burden were aggregated and summarized. First, country registries, third-party modeled data, and meta-analyzed published data were combined to generate incidence and volume figures for 10 common neurosurgical conditions. Next, a global mapping survey was performed to identify the number and location of neurosurgeons in each country. Finally, a practitioner survey was conducted to quantify the proportion of disease requiring surgery, as well as the median number of neurosurgical cases per annum. The neurosurgical case deficit was calculated as the difference between the volume of essential neurosurgical cases and the existing neurosurgical workforce capacity.

RESULTS

Every year, an estimated 22.6 million patients suffer from neurological disorders or injuries that warrant the expertise of a neurosurgeon, of whom 13.8 million require surgery. Traumatic brain injury, stroke-related conditions, tumors, hydrocephalus, and epilepsy constitute the majority of essential neurosurgical care worldwide. Approximately 23,300 additional neurosurgeons are needed to address more than 5 million essential neurosurgical cases—all in low- and middle-income countries—that go unmet each year. There exists a gross disparity in the allocation of the surgical workforce, leaving large geographic treatment gaps, particularly in Africa and Southeast Asia.

CONCLUSIONS

Each year, more than 5 million individuals suffering from treatable neurosurgical conditions will never undergo therapeutic surgical intervention. Populations in Africa and Southeast Asia, where the proportion of neurosurgeons to neurosurgical disease is critically low, are especially at risk. Increasing access to essential neurosurgical care in low- and middle-income countries via neurosurgical workforce expansion as part of surgical system strengthening is necessary to prevent severe disability and death for millions with neurological disease.