Chronic Debilitating Headache in Adults Caused by Craniocerebral Disproportion: Treatment by Cranial Vault Expansion.

Craniocerebral disproportion is rarely considered as a cause for chronic, debilitating headache in adults. Children reported with this disorder typically suffer from headaches and lethargy for many years and have multisutural synostosis. The terms craniocerebral disproportion, craniostenosis, and slit-ventricle syndrome are used inconsistently as diagnostic designations. Three adults with craniocerebral disproportion who had been treated in infancy for two different pathologies are reported. All benefited greatly from cranial vault expansion.

Shunt Nephritis: An Increasingly Unfamiliar Diagnosis.

BACKGROUND: Shunt nephritis is a rare, reversible immune-complex mediated complication of cerebrospinal fluid (CSF) shunt infection that can progress to end-stage renal disease and even death if diagnosis is delayed. CASE DESCRIPTION: The present case report details the manifestation and clinical course of shunt nephritis in a 50-year-old patient who presented with symptoms of nephrotic syndrome 30 years after ventriculojugular shunt placement. Diagnosis was delayed due to initial negative CSF and blood cultures, but a later CSF culture was positive for Propionibacterium acnes. After treatment with intravenous antibiotics and complete removal of shunt with subsequent replacement with a new ventriculoperitoneal shunt, the nephritic symptoms resolved, but the patient continued to have reduced kidney function consistent with stage IIIa chronic kidney disease. CONCLUSION: This case emphasizes the clinical importance of having a high index of suspicion in patients with a ventricular shunt who present with symptoms consistent with nephritis, even in the setting of negative cultures and delayed presentation.

Differential Pressure Shunt for Simultaneous Diversion of Ventricular Fluid and Extracerebral Fluid.

BACKGROUND/AIMS: The management of extracerebral collections of fluid in patients with hydrocephalus can be problematic for either their simultaneous separate management or sequential management, each of which may require multiple surgeries and the management of external drains. The object of this report is to review the experience with a shunt configuration that simultaneously diverts ventricular fluid and extracerebral fluid, whether subdural or subarachnoid in location, through different outflow resistances. METHODS: The medical records, including neuroimaging of patients with hydrocephalus and clinically significant extracerebral collections of low density who were managed by implanting a differential pressure type shunt, were retrospectively reviewed. RESULTS: Four patients, 3 children and 1 adult, met inclusion criteria. Three had the entire differential pressure shunt implanted under 1 anesthetic, and 1 had a catheter inserted into the subdural space and connected into an existing ventriculoperitoneal shunt system. The extracerebral fluid collections cleared in all 4 patients, and the CSF shunt continued to function normally. CONCLUSION: A single surgical procedure to implant a differential pressure shunt can simultaneously drain and obliterate an extracerebral fluid collection while managing the hydrocephalus. Compared to routines that include external drainage, differential pressure shunting requires fewer surgeries, shorter hospitalization, with expected less expense.

Techniques for Achieving Hemostasis from Choanoid Cranial Foramina.

OBJECTIVE: This report describes 2 techniques for controlling otherwise difficult-to-control hemorrhage from choanoid bony foramina. METHODS: Medical records of 3 patients in whom vigorous and difficult-to-control bleeding from choanoid cranial foramina was successfully halted are reviewed. RESULTS: In 2 patients, vigorous arterial bleeding from the foramen spinosum was controlled with a wooden peg. In 1 patient, vigorous venous bleeding from a foramen in the convexity of the occipital bone was controlled with a wedge of bone. CONCLUSIONS: Vigorous bleeding, whether arterial or venous, from choanoid foramina can be quickly and reliably controlled by the techniques described.

Everting Fracture of Entire Frontal Bone: Management and Importance of Preliminary Reconstruction.

A 34-year-old woman with severe craniofacial trauma, which included eversion of the entire frontal bone, survived with high-quality functional and cosmetic results as a consequence of efficient and aggressive action by emergency technicians and physicians. Early preliminary steps of craniofacial reconstruction provided a structural foundation that facilitated this outcome.

Removal of Vagus Nerve Stimulator Leads and Reuse of Same Site for Reimplantation: Technique and Experience.

OBJECTIVE: This report describes the authors' experience and technique in removing vagus nerve stimulator leads, including coils, and reuse of the same site on the vagus nerve for implantation of new coils. METHODS: The charts of all patients who underwent complete removal by the authors of vagus nerve stimulator leads between 1 September 2001 and 1 July 2015 were retrospectively reviewed. RESULTS: Thirty patients underwent 31 surgeries for removal of vagus nerve stimulator leads. Complete removal, including proximal coils around the vagus nerve, was achieved in all cases. Reimplantation was performed immediately at the same location in 24 patients, delayed in 1 patient, and never replaced in 6. Long-term vocal cord paralysis followed 2 of 9 surgeries performed with sharp dissection and followed one of 22 surgeries in which dissection was performed with monopolar microneedle electrocautery. CONCLUSIONS: Vagus nerve stimulator coils can be removed from the vagus nerve, via monopolar microneedle electrocautery, and the same site reused for immediate reimplantation with relative safety.

Resistance to venous flow: A mathematical description and implications for compartment syndromes.

BACKGROUND: The object of this work was to describe resistance to flow within a vein in a closed compartment. METHODS: A vein is mathematically modeled as a collapsible cylinder with fixed perimeter exposed to extraluminal hydrostatic pressure within a closed compartment of the body. The principle of minimization of energy is used to determine the cross-sectional area and resistance to flow through such a cylinder in various states of collapse. RESULTS: A mathematical expression for the cross-sectional area of a partially collapsed tube is derived. Resistance to flow is calculated within the tube in various states of collapse and compared with the resistance to flow in an annular tube of identical cross-sectional area. Resistance increases very rapidly in the first 5% of collapse and remains greater than that in an annular vessel of identical cross-sectional area through all further collapse. Resistance to flow closely follows a logarithmic gain as the tube undergoes collapse from extraluminal pressure until opposite sides of the vein make contact. CONCLUSION: Within a closed compartment in which there is rising pressure, resistance to flow through a vein is predicted to increase as a logarithmic function of the vein's cross-sectional area. This rapid rise in resistance and hence decline in flow are consistent with the position that in compartment syndromes of all anatomic locations, the venous contribution to resistance of flow is of paramount importance to the pathophysiology.

Aplasia Cutis Congenita of the Scalp, Composite Type: The Criticality and Inseparability of Neurosurgical and Plastic Surgical Management.

BACKGROUND/AIMS: The object of this report is to review the management of patients having the composite type of aplasia cutis congenita in the context of the relevant literature on this condition. METHODS: Clinical records, neuroimaging and photographic documentation of identified cases of composite type aplasia cutis congenita, with a comprehensive review of the literature, are the material basis of this report. RESULTS: Two neonates with composite type aplasia cutis congenita are described as examples of this disorder, and their management, including complications, is discussed. Both had satisfactory outcomes. CONCLUSIONS: Early aggressive surgical management with scalp rotation flaps has a low rate of complications, provides satisfactory functional and esthetic outcome, minimizes hospital stays, and should provide cost-effective care by reduction of the time to secure wound closure. Most bony defects, even large ones, commonly ossify completely. Optimal case management requires a synchronized neurosurgical and plastic surgical team. Intuitive nonsurgical and surgical approaches by the inexperienced can be hazardous.

Damage-control neurosurgery: Packing to halt relentless intracranial bleeding.

BACKGROUND: The objective of this study was to review the efficacy of intracranial packing as a means of tamponade for life-threatening intraoperative hemorrhage that was refractory to more common techniques for achieving hemostasis. METHODS: Neuroimaging and hospital records were reviewed for the seven adult patients who had experienced life-threateningly severe hemorrhage during intracranial surgery and in whom packing was used to control the bleeding. All packing was left in place at the time of closure and was removed when the patient's condition was considered safe for a second operation. RESULTS: Hemorrhage was successfully halted in all seven patients, and all survived their operations. Six were discharged from the hospital, but one patient with severe parenchymal injury from trauma and multiple medical comorbidities died on postoperative Day 2 after supportive care was withdrawn. Four had an improved Glasgow Outcome Scale (GOS) score at the time of last follow-up, and two of these improved from dependent to independent living. There were no postoperative intracranial or wound infections. CONCLUSION: Intracranial packing to tamponade severe intracranial hemorrhage can be a lifesaving neurosurgical maneuver. LEVEL OF EVIDENCE: Therapeutic study, level V.

Upward Translation of Cerebellar Tonsils following Surgical Expansion of Supratentorial Cranial Vault: A Unified Biomechanical Explanation of Chiari Type I.

Cerebellar tonsils moved significantly upward in 3 patients with Chiari type I who underwent supratentorial cranial vault expansion to alleviate intracranial pressure related to multisutural craniosynostosis. The Chiari type I deformities in these patients were the biomechanical consequence of posterior fossa-cerebellar disproportion caused by supratentorial craniocerebral disproportion secondary to multisutural craniosynostosis. The authors postulate that all cases of Chiari type I deformity share the sine qua non feature of posterior fossa-cerebellar disproportion.

Experience with a Low Single Cervical Incision for Implantation of a Vagus Nerve Stimulator: Technique and Advantages.

OBJECTIVE: This report describes the technique for implanting a vagus nerve stimulator via a single low anterior cervical incision and discusses the advantages of this technique over that of the more commonly used 2-incision technique. METHODS: The authors performed a retrospective review of all patients who underwent implantation of a vagus nerve stimulator by the senior author over a 10-year period. RESULTS: One hundred thirty-one patients underwent implantation of vagus nerve stimulators via the single-incision technique. There were no instances of vagus nerve injury, postoperative hematoma, or wound infection, and cosmesis was excellent. CONCLUSION: The single-incision technique described here for implantation of vagus nerve stimulators is technically straightforward and safe, and has significant advantages over the 2-incision technique.

Packing to tamponade severe intracranial hemorrhage in pediatric patients.

The medical records of all children in whom packing was used to control severe intracranial hemorrhage were reviewed. Eight children, with ages ranging from newborn to 4 years, met the inclusion criteria and all survived. Five were victims of severe closed head trauma, 2 had received penetrating cranial injuries, and 1 developed severe bleeding while undergoing surgery for a malignant tumor in the posterior fossa. Blood loss at the time of removal of the packing was minimal in 7 patients and was surgically controllable in the other. Packing is a simple, efficient, and safe maneuver which can very often halt intracranial bleeding that is considered to be otherwise uncontrollable, and can thereby limit the consequences of prolonged or repeated periods of hypotension and possible exsanguination.

Relationship of vocal cord paralysis to the coil diameter of vagus nerve stimulator leads.

OBJECT: This investigation was done to examine, following implantation of vagus nerve stimulators, the relationship of vocal cord paralysis to the inner diameter of the coils used to attach the stimulator lead to the nerve. METHODS: All data in this investigation were collected, as mandated by the FDA, by the manufacturer of vagus nerve stimulators and were made available without restrictions for analysis by the authors. The data reflect all initial device implantations in the United States for the period from 1997 through 2012. RESULTS: Vocal cord paralysis was reported in 193 of 51,882 implantations. In patients aged 18 years and older, the incidence of paralysis was 0.26% when the stimulator leads had coil diameters of 3 mm and 0.51% when the leads had 2-mm-diameter coils (p < 0.05). Across all age groups, the incidence of vocal cord paralysis increased with age at implantation for leads having 2-mm-diameter coils. CONCLUSIONS: In patients aged 18 years and older, vocal cord paralysis occurred at almost twice the rate with the implantation of vagus nerve stimulator leads having 2-mm-diameter coils than with leads having 3-mm-diameter coils. The incidence of vocal cord paralysis increases with patient age at implantation.

Breach of Posterior Wall of Frontal Sinus: Management with Preservation of the Sinus.

OBJECTIVE: To analyze outcomes after the management of mild (<1 mm) and moderately severe (>1 mm and <5 mm) breaches of the posterior wall of the frontal sinus with a goal of maintaining or restoring the functional status of the sinus. METHODS: A retrospective analysis of prospectively accrued data was performed on patients with mild and moderately severe breaches of the posterior wall of their frontal sinus who were managed with the intent to preserve the frontal sinus. Data on presenting features, pathology, details on breaches of the posterior wall, management, outcome, and complications were collected from medical records and neuroimages. RESULTS: Forty-two cases met inclusion criteria. Diagnostic categories included trauma in 34 cases, infection in 3, and other categories in another 5 cases. Five presented with cerebrospinal fluid rhinorrhea, and 26 had radiographic evidence of obstruction of a nasofrontal duct at time of presentation. Fifteen patients were managed without surgical intervention, and 27 underwent surgery. No complications occurred in the patients managed without surgery and 4 postoperative cerebrospinal leaks that were managed successfully with a period of drainage occurred in the surgical group. No patient developed meningitis or mucocele. CONCLUSIONS: Many patients with mild to moderately severe breaches of the posterior wall of the frontal sinus can be managed safely and effectively by techniques that preserve the anatomy and function of the frontal sinus.

Recurrent cerebrospinal fluid shunt infection and the efficacy of reusing infected ventricular entry sites.

OBJECT: The objective of this investigation was to review the clinical characteristics of recurrent CSF shunt infections in a large pediatric neurosurgical practice and to assess the safety and efficacy of reusing original ventricular entry sites for external ventricular drainage during treatment of infections and for subsequent reinsertion of shunts. METHODS: Prospectively accrued clinical data on all patients treated at Children's Hospital Colorado for CSF shunt infections within a 10.5-year span were retrospectively investigated. RESULTS: One hundred twenty-one consecutive cases of CSF shunt infection met inclusion criteria. Recurrent shunt infection attributable to the management of these infections occurred in 14 cases (11.6%). Three recurrent infections were with their original organisms, 7 were organisms different from the original organisms, and 4 were indeterminate. CONCLUSIONS: Half or more of recurrent shunt infections were with organisms different from the original organism, and hence were new-type infections introduced during the management of the original infections. Incomplete eradication of original pathogens accounted for 3 (21.4%) of the 14 recurrent infections. Reusing recently infected or contaminated ventricular entry sites, both for CSF drainage during treatment and for implantation of new shunts, was as safe, with regard to risk of recurrent infection, as switching to new entry sites. Prior evidence of shunt infection is not, alone, a sufficient reason to change to a previously well-functioning site, and reuse of contaminated ventricular entry sites avoids all risks associated with making new ventricular entries.

Syndromic craniosynostosis, fibroblast growth factor receptor 2 (FGFR2) mutations, and sacrococcygeal eversion presenting as human tails.

INTRODUCTION: There have been 23 previously published cases of patients with syndromic craniosynostosis and human tails. In many of these, the tail was composed of prominent coccygeal and sacral vertebrae, curved in a retroverted instead of in the normal anterograde fashion. This has been termed sacrococcygeal eversion. In those cases in which genetic testing results are reported, there were fibroblast growth factor receptor 2 (FGFR2) mutations. METHODS: We present three patients with Pfeiffer syndrome and sacrococcygeal eversion. Two had genetic testing and both had FGFR2 mutations, one of them a novel mutation in patients with syndromic craniosynostosis and sacrococcygeal eversion. We also briefly review the literature on craniosynostosis and human tails. RESULTS: All but one reported patient has had either Pfeiffer, Crouzon, or Beare-Stevenson syndrome. Most patients, including ours, have had severe manifestations of their syndrome. Although the pathogenesis of sacrococcygeal eversion is unknown, a similarly posteriorly curved tail bud develops in normal human embryos during the second month of gestation. CONCLUSIONS: Perhaps increased FGFR2 activation during this embryonic period leads to abnormal differentiation or regression of the tail bud and, in turn, sacrococcygeal eversion, in certain patients with severe syndromic craniosynostosis.

Multidisciplinary approach to cerebrospinal fluid shunt infection with an appeal for attention to details in assessment and standardization in reporting.

OBJECT: The goal of this study was to evaluate the problems encountered in monitoring CSF shunt infection, including the collection, analysis, and reporting of data. The authors propose a system that would produce more accurate, and hence more meaningful, information on shunt infection than do the methodologies and customs now in common use. METHODS: The authors reviewed and analyzed 19 years of quarterly records of a committee that has addressed CSF shunt infection in an ongoing manner. RESULTS: There are strong incentives, political and otherwise, to identify low rates of CSF shunt infection. Details of the composition and operation of a multidisciplinary approach to CSF shunt infection are summarized. Many factors affect the occurrence of shunt infection and its accurate assessment and reporting. Easily accessible sources for the identification of cases of shunt infections and for the assessment of an at-risk population often contain discrepancies in significant numbers. CONCLUSIONS: Multidisciplinary oversight of the entire matter of CSF shunt infection enhances the chances for collecting accurate data, identifying causes of infection, and developing effective preventative strategies. Valid data require a mechanism for finding all individuals within the at-risk pool; the accurate identification of patients who had shunt infections; standard, pragmatic, and robust criteria for diagnosis of shunt infection; and multidisciplinary oversight of the entire process.