Spasticity and movement disorders in cerebral palsy.

PURPOSE: To review the neurosurgical treatments of children with movement disorders associated with cerebral palsy (CP) during the previous decades, up to the present day. METHODS: An extensive literature review was undertaken to identify important publications about this subject. My experience treating children with these disorders over the past three decades was included in the individual sections. RESULTS: Peripheral neurotomies have been developed for children with focal spasticity. For those with spastic paraparesis, selective lumbar rhizotomies were developed, and for those with spastic quadriparesis, intrathecal baclofen infusions were developed. Both effectively alleviate spasticity in the affected extremities. Generalized dystonia associated with CP has been treated with deep brain stimulation with mild improvement, but treatment with intrathecal baclofen and intraventricular baclofen improve those movements markedly. No effective treatment has been reported for children with athetoid CP. For those with choreiform CP, deep brain stimulation may be effective but intrathecal baclofen does not appear to be. CONCLUSION: Treatment of children with movement disorders associated with CP increased slowly in the 1970s and 1980s but accelerated rapidly in the 1990s with the introduction of lumbar dorsal rhizotomies and intrathecal baclofen. In the last 30 years, tens of thousands of children with spasticity and movement disorders associated with CP have been treated by pediatric neurosurgeons, and their care has become an integral component of current pediatric neurosurgical practice.

Hydranencephaly treatments: retrospective case series and review of the literature.

OBJECTIVE: The objective of this study was to review treatment options for infants with hydranencephaly and to consider the pros and cons of each treatment modality. METHODS: This paper is a review of hydranencephaly as well as a retrospective analysis evaluating the outcomes of 52 infants with hydranencephaly who were treated at the Kijabe Hospital, Kijabe, Kenya, in one of four ways: ventriculoperitoneal shunt (VPS) insertion, endoscopic choroid plexus coagulation (CPC), open choroid plexectomy (CPlx), and palliative care. The primary outcome measure was control of head size, with the aim of improving patient care. One-year mortality was a secondary outcome. RESULTS: Of the 52 patients analyzed, 11 underwent VPS insertion, 17 CPC, 14 CPlx, and 10 were treated palliatively. Head size was controlled at the 3-month evaluation interval in 5 of 7 infants treated with VPS, 10 of 16 of those treated with CPC, 6 of 9 of those treated with CPlx, and 1 of 4 treated palliatively. The number of infants in each category with complete follow-up data that were needed to analyze change in head size was lower than the total number of patients included in each category. Mortality at 1 year of age was 9 of 11 in the VPS group, 14 of 17 in the CPC group, 6 of 14 in the CPlx group, and 7 of 10 in the palliative group. CONCLUSIONS: Head size decreased by 1 cm or more in similar proportions (62%-71%) of infants with hydranencephaly who were treated by VPS insertion, CPC, and CPlx, and progressed in those who received palliative care. Mortality at 1 year of age was similar in infants treated by a VPS, CPC, and palliative care (70%-82%), but lower (43%) in those treated with CPlx.

Pediatric Ventriculoperitoneal Shunts Revision Rate and Costs in High-Volume sub-Saharan Department.

BACKGROUND: Ventriculoperitoneal shunt (VPS) placement is one of the most common neurosurgical procedures. VPSs are associated with high costs, which predominantly arise from relatively high complication and revision rates. It is reasonable to assume that revision rates in developing countries would be higher. In this study we report the single-institution revision rates and costs from a high-volume department in sub-Saharan Africa. METHODS: A pediatric neurosurgical database was studied in the extent of 5 years. The 30-day shunt failure rate, overall revision rate, and costs were calculated, and results were compared with previously published studies from developed countries. RESULTS: In the selected time period 1840 VPS surgeries were performed, of which 592 were shunt revisions (32.14%). The majority of revision surgeries was performed in the first year- 501 (representing 84.63%); second year, 64; third year, 21; fourth year, 6; and fifth year, 2. The overall shunt revision rate was 28.94% with a 30-day revision rate of 14.58%. During the course of the study, costs of VPS surgery, the shunt, and daily ward charges did not change significantly. The average total charge for VPS insertion was 60,000 KES (586 USD), VPS removal 30,000 KES (293 USD), and VPS revision 50,000 KES (489 USD). CONCLUSIONS: This retrospective study proves that VPSs, with their known complication risks, can be performed in a sub-Saharan missionary hospital with acceptable costs and results that are comparable with those achieved in some Western hospitals. Keys to those outcomes include high volume and a highly experienced team.

Ventriculoperitoneal shunt perforations of the gastrointestinal tract.

OBJECT The purposes of this study were to evaluate the frequency with which children presented with ventriculoperitoneal (VP) shunt perforations of the gastrointestinal (GI) tract, to determine the type of shunts that caused the perforations, and to compare the stiffness of perforating catheters with the stiffness of catheters from other manufacturers. METHODS Medical records were reviewed of 197 children who were admitted with VP shunt malfunction. Catheter stiffness was evaluated by measuring relative resistance to cross-sectional compression, resistance to column buckling, and elasticity in longitudinal bending. Catheter frictional force was measured per unit length. RESULTS Six children were identified whose VP shunts had perforated the GI tract; 2 shunts subsequently protruded through the anal orifice, 1 protruded through the oral cavity, and 3 presented with subcutaneous abscesses that tracked upward from the intestine to the chest. All perforating shunts were Chhabra shunts. Catheter stiffness and resistance to bending were greatest with a Medtronic shunt catheter, intermediate with a Codman catheter, and least with a Chhabra catheter. Frictional force was greatest with a Chhabra catheter and least with a Medtronic catheter. CONCLUSIONS The frequency of perforations by Chhabra shunts appears to be higher than the frequency associated with other shunts. The increased frequency does not correlate with their stiffness but may reflect their greater frictional forces.

Sources of delayed provision of neurosurgical care in a rural kenyan setting.

BACKGROUND: Delay to neurosurgical care can result in significant morbidity and mortality. In this study, we aim to identify and quantify the sources of delay to neurosurgical consultation and care at a rural setting in Kenya. METHODS: A mixed-methods, cross-sectional analysis of all patients admitted to the neurosurgical department at Kijabe Hospital (KH) was conducted: A retrospective analysis of admissions from October 1 to December 31, 2013 and a prospective analysis from June 2 to June 20, 2014. Sources of delay were categorized and quantified. The Kruskal-Wallis test was used to identify an overall significant difference among diagnoses. The Mann-Whitney U test was used for pairwise comparisons within groups; the Bonferroni correction was applied to the alpha level of significance (0.05) according to the number of comparisons conducted. IBM SPSS version 22.0 (SPSS, Chicago, IL) was used for statistical analyses. RESULTS: A total of 332 admissions were reviewed (237 retrospective, 95 prospective). The majority was pediatric admissions (median age: 3 months). Hydrocephalus (35%) and neural tube defects (NTDs; 27%) were most common. At least one source of delay was identified in 192 cases (58%); 39 (12%) were affected by multiple sources. Delay in primary care (PCPs), in isolation or combined with other sources, comprised 137 of total (71%); misdiagnosis or incorrect management comprised 46 (34%) of these. Finances contributed to delays in 25 of 95 prospective cases. At a median delay of 49 and 200.5 days, the diagnoses of hydrocephalus and tumors were associated with a significantly longer delay compared with NTDs (P < 0.001). CONCLUSION: A substantial proportion of patients experienced delays in procuring pediatric neurosurgical care. Improvement in PCP knowledge base, implementation of a triage and referral process, and development of community-based funding strategies can potentially reduce these delays.

Bacteria causing ventriculoperitoneal shunt infections in a Kenyan population.

OBJECT: Ventriculoperitoneal shunt (VPS) infections are a major cause of morbidity and mortality in patients with hydrocephalus. Most data about these infections come from the Western literature. Few data about infecting organisms in Africa are available, yet knowledge of these organisms is important for the prevention and treatment of infectious complications. The purpose of this study was to determine the organisms cultured from infected shunts in a rural Kenyan hospital. METHODS: The authors conducted a retrospective study of patients with VPS infections recorded in the neurosurgical database of BethanyKids at Kijabe Hospital between September 2010 and July 2012. RESULTS: Among 53 VPS infections confirmed by culture, 68% occurred in patients who were younger than 6 months. Seventy-nine percent of the infections occurred within 2 months after shunt insertion. Only 51% of infections were caused by Staphylococcus species (Staphylococcus aureus 25%, other Staphylococcus species 26%), whereas 40% were caused by gram-negative bacteria. All S. aureus infections and 79% of other Staphylococcus infections were sensitive to cefazolin, but only 1 of 21 gram-negative bacteria was sensitive to it. The majority of gram-negative bacterial infections were multidrug resistant, but 17 of the 20 gram-negative bacteria were sensitive to meropenem. Gram-negative bacterial infections were associated with worse outcomes. CONCLUSIONS: The high proportion of gram-negative infections differs from data in the Western literature, in which Staphylococcus epidermidis is by far the most common organism. Once a patient is diagnosed with a VPS infection in Kenya, immediate treatment is recommended to cover both gram-positive and gram-negative bacterial infections. Data from other Sub-Saharan countries are needed to determine if those countries have the same increased frequency of gram-negative infections.

Distal spinal cord stimulation in infants with myelomeningoceles: preliminary study.

OBJECT: The purpose of this study of infants with myelomeningoceles was to evaluate the effect of stimulation of the distal spinal cord, proximal to the neural placode, on functioning lower-extremity muscles distal to the placode. METHODS: In 13 infants with lumbosacral myelomeningoceles, the lateral spinal cord was stimulated, with a monopolar probe, between its exit from the spinal canal and the neural placode. Responses of functional muscles in the lower extremities were monitored with a neural integrity monitor. RESULTS: Stimulation of the lateral spinal cord resulted in no contraction of lower-extremity muscles in 7 of 13 infants in whom no nerves exited the cord between the spinal canal and the neural placode. In those with exiting nerves in that location, stimulation of the cord resulted in the contraction of functional muscles. Distal cordectomies at the junction between the spinal cord and the neural placode, distal to any nerve roots exiting from the cord, resulted in no loss of movement. CONCLUSIONS: Monopolar stimulation of the lateral spinal cord does not seem to cause contraction of functional lower-extremity muscles unless nerve roots to the lower extremities exit the cord distal to the spinal canal but before its junction with the neural placode. Muscle contraction in cases in which nerve roots exit the spinal cord between the canal and the placode may be caused by the spread of current from the monopolar stimulator.

Enterocutaneous fistula in the setting of ventriculoperitoneal shunt extrusion through the skin and perforation through the small bowel.

The authors report a case of a 2-year-old boy with postinfectious hydrocephalus, managed with a ventriculoperitoneal (VP) shunt and complicated by shunt extrusion through the cranial skin. The shunt was removed due to concern for infection, and the child was found to have an enterocutaneous fistula (ECF) communicating along the shunt track between the small bowel and a clavicular sinus. Self-closure of the ECF was anticipated. Thus, the fistula was managed expectantly with dressing changes of the clavicular sinus, while the patient's malnutrition was managed in accordance with World Health Organization protocols. The presentation, prognosis and management of ECFs, including the likelihood of self-resolution and the role of expectant management, are discussed. Additionally, proposed mechanisms of ECF formation in the setting of a VP shunt are discussed, with an emphasis on the roles of infection and malnutrition.

Choroid plexus coagulation in infants with extreme hydrocephalus or hydranencephaly.

UNLABELLED: OBJECT.: Severe hydrocephalus and hydranencephaly are common congenital conditions in Kenya. In patients with these conditions, ventriculoperitoneal (VP) shunts are associated with appreciable complications and endoscopic third ventriculostomies (ETVs) have limited success. Endoscopic choroid plexus coagulation (CPC) to diminish CSF production is a potential treatment option. The purpose of this study was to evaluate the effect of CPC without ETV in infants with severe hydrocephalus or hydranencephaly. METHODS: Medical records of infants with severe congenital hydrocephalus or hydranencephaly who underwent CPC in Kijabe Hospital from November 2010 to April 2013 were reviewed retrospectively. Thirty-three patients with complete medical records and preoperative radiographic images were identified. After CPC, the infants were followed in the Kijabe Hospital outpatient department, in mobile clinics, or by telephone. Success of the CPC was defined as resolution of preoperative symptoms, stabilization of head size, and avoidance of VP shunt placement. RESULTS: Patients were followed from 30 to 608 days (median of 120 days). Three patients were lost to follow-up. Of the 30 evaluable patients, CPC was considered to be successful in 13 (43.3%), including 8 of 20 patients with severe hydrocephalus and 5 of 10 with hydranencephaly. Failure of CPC was evident from increased head circumference in 14 (82%) of 17 patients and from CSF leakage in 3. Of the 17 failures, 13 occurred within 3 months of surgery. Six patients died: 3 whose CPC procedures were failures, 2 whose CPC was successful, and 1 postoperatively. Of the 17 in whom CPC failed, 10 subsequently underwent VP shunt insertion. CONCLUSIONS: CPC stabilizes macrocephaly in approximately 40% of infants with severe congenital hydrocephalus and hydranencephaly and can be considered as an alternative to VP shunt placement.

Distal cordectomies as treatment for lumbosacral myelomeningoceles.

OBJECT: The purpose of this study was to evaluate the effect of distal cordectomies on motor function in the lower extremities of infants with lumbosacral myelomeningoceles. METHODS: Medical records were reviewed in 32 infants with lumbosacral myelomeningoceles who were 1 day to 7 months old, who had some lower-extremity function preoperatively, and who were treated by distal cordectomies, dividing the cord between its exit from the intact spinal canal and the neural placode. Neurological function was evaluated before and after operation by therapists who were unaware of the surgical technique. RESULTS: Neurological function was unchanged after operation in 29 of 32 cases. For the others, hip flexion, foot plantar flexion, and toe movement were lost in 1 case each. CONCLUSIONS: The majority of infants treated by distal cordectomies retain preoperative motor function after operation.

Cerebrospinal fluid white blood cell counts in infants with myelomeningoceles.

OBJECT: The authors undertook this study to determine white blood cell (WBC) counts in CSF obtained from lateral ventricles and myelomeningoceles (MMCs) in infants in a developing country at the time of their initial presentation for medical evaluation. METHODS: CSF was aspirated from the lateral ventricles and from MMC sacs of 100 consecutive infants at Kijabe Hospital, Kijabe, Kenya. Peripheral blood WBC counts and CSF WBC counts were determined in the laboratory. CSF with WBC counts of 5 cells/mm(3) or greater was cultured. RESULTS: The mean WBC count in ventricular CSF was 16 cells/mm(3), with a median and mode of 0 cells/mm(3). The mean WBC count of CSF in MMC sacs was 141 cells/mm(3) (median 15 cells/mm(3)). No child had both a positive culture from ventricular CSF and a negative culture from MMC CSF. There was no correlation between age at presentation and WBC counts in the MMCs. Infants younger than 8 days old were as likely to have high WBC counts in CSF from their MMC sacs as were older children; 7 of 12 infants with 500 WBCs or more in CSF from their MMCs were younger than 8 days old. Only 5 of 58 CSF specimens from MMC sacs with 5 or more WBCs/mm(3) had positive bacterial cultures, which may be a reflection of CSF specimen processing rather than of true culture negativity. CONCLUSIONS: CSF from ventricular fluid of infants presenting with MMCs infrequently has high WBC counts, so infrequently that it does not need to be evaluated routinely. CSF in MMC sacs often has high WBC counts that suggest the presence of bacterial infection. In developing countries where culture reliability is questionable, intravenous administration of antibiotics before MMC closure for infants with high MMC WBC counts may diminish postoperative meningitis/ventriculitis.

Tethered cord syndrome secondary to the unusual constellation of a split cord malformation, lumbar myelomeningocele, and coexisting neurenteric cyst.

We describe a seminal case report of a child with a tethered cord syndrome secondary to the unusual constellation of a split cord malformation, lumbar myelomeningocele, and coexisting neurenteric cyst. A 17-year-old adolescent girl with a several-month history of myelopathy and urinary incontinence was examined whose spinal MRI scan demonstrated a type II split cord malformation with a large bone spur and an intradural neurenteric cyst in addition to lumbar myelomeningocele. Untethering of the spinal cord was achieved via a lumbar laminectomy. Pathological examination confirmed the intradural cyst to be a neurenteric cyst. Postoperatively there was stabilization of the neurological symptoms. Prophylactic surgery with total resection of the neurenteric cyst when feasible and spinal cord un-tethering appears to be associated with excellent outcomes.

Intraventricular meningioma: case report and literature review.

In this article we describe the clinical course of a child with an intraventricular meningioma and review the relevant literature. A 7-year-old girl with a several-month history of headaches had a CT scan that demonstrated a large, enhancing, intraventricular tumor. The tumor was completely excised via a superior parietal approach. Pathological examination revealed a pleomorphic meningioma. Postoperative scans demonstrated no residual tumor. Pediatric intraventricular tumors are rare. The surgical objective is complete resection, which appears to be associated with excellent outcomes.

Use of the NICO Myriad device for tumor and cyst removals in a developing country.

PURPOSE: To describe the use of the NICO Myriad, a new side-cutting aspiration device for the resection of tumors, in a developing country. METHODS: The 11-, 13-, and 15-ga handpieces were used to resect tumors exposed via craniotomies, and the 19-ga handpiece was used down the side channel of a Storz Oi endoscope to resect tumors exposed endoscopically. RESULTS: The Myriad was used to resect 23 tumors, including spinal cord tumors, posterior fossa tumors and pineal tumors, and the cysts associated with two craniopharyngiomas. No complications were associated with the Myriad. Handpieces that were re-sterilized in Steranios after the initial use could each be used two to four times thereafter. CONCLUSIONS: The Myriad is the first effective tumor removal device that can be introduced down the side channel of most endoscopes, greatly expanding the spectrum of tumors that can be treated endoscopically. Its minimal diameter allows better visibility in small, deep sites such as the pineal region than is usually available when ultrasonic aspirators are used. The cost of the device, and particularly the handpieces, will limit their utility in developing countries until re-usable handpieces are developed.

Technique for insertion of intraventricular baclofen catheters.

Intraventricular baclofen (IVB) infusion has been used recently to treat secondary dystonia. Techniques of catheter implantation have not been thoroughly described. The object of this study was to describe a technique developed in the past 6 years to implant intraventricular catheters for baclofen infusion. Thirty-one patients underwent endoscopic placement of intraventricular catheters for IVB, primarily patients whose anatomy made implantation of intrathecal catheters difficult or inappropriate. Using the technique described, catheters were successfully inserted into the desired ventricular location in each patient. Catheters remained in the desired location in 29 of 31 cases. Catheters can be inserted into the cerebral ventricles for infusion of baclofen. The technique described herein has been associated with few complications.