Arachnoid cyst causing third cranial nerve palsy manifesting as isolated internal ophthalmoplegia and iris cholinergic supersensitivity.

An 8-month-old boy presented with anisocoria, a sluggishly reactive right pupil, and cholinergic supersensitivity as the only signs of what proved months later to be compressive third cranial nerve palsy due to an arachnoid cyst. Tonic constriction and dilation, segmental iris sphincter palsy, aberrant regeneration phenomena, ductional deficits, and ptosis were absent. The initial diagnosis was postganglionic internal ophthalmoplegia attributed to a viral ciliary ganglionopathy. Nineteen months later, he had developed an incomitant exodeviation and a supraduction deficit. Brain MRI revealed a mass consistent with an arachnoid cyst compressing the third cranial nerve in the right interpeduncular cistern. Resection of the cyst led to a persistent complete third cranial nerve palsy. This is the second reported case of prolonged internal ophthalmoplegia in a young child as a manifestation of a compressive third cranial nerve palsy. Our patient serves as a reminder that isolated internal ophthalmoplegia with cholinergic supersensitivity is compatible with a preganglionic compressive third nerve lesion, particularly in a young child.

Conversion locked-in syndrome after implantation of a spinal cord stimulator.

BACKGROUND: The locked-in syndrome is defined as quadriplegia and anarthria (loss of articulate speech) with the preservation of consciousness. It is typically caused by a lesion to the ventral pons. Conversion disorder is the deficit of voluntary motor or sensory function requiring an extensive work-up to exclude any organic cause. METHODS AND RESULTS: After surgery for an implantation of a spinal cord stimulator, a 42-year-old woman presented with quadriplegia and lower facial diplegia, but was able to open and blink her eyes. We found no organic causes to explain her condition after appropriate radiological studies looking for intracranial or intraspinal causes, and reversal drugs were administered with no immediate effect. Over the course of several hours, the patient gradually recovered and was discharged the following day. A psychology consultation was obtained during her stay and she was found to meet the criteria for a conversion disorder to explain her condition. CONCLUSIONS: Before considering a psychological cause, all organic factors should be excluded with proper tests and consultations, as conversion disorder is a diagnosis of exclusion. We report a patient who, after implantation of a spinal cord stimulator, manifested locked-in syndrome resulting from a conversion disorder.

Congenital malformations of the skull and meninges.

The surgery and management of children who have congenital malformations of the skull and meninges require multidisciplinary care and long-term follow-up by multiple specialists in birth defects. The high definition of three-dimensional CT and MRI allows precise surgery planning of reconstruction and management of associated malformations. The reconstruction of meningoencephaloceles and craniosynostosis are challenging procedures that transform the child's appearance. The embryology, clinical presentation, and surgical management of these malformations are reviewed.

Conservative management of typical pediatric postauricular dermoid cysts.

OBJECTIVE: Congenital dermoid cysts of the skull and face frequently arise in embryonic fusion planes. They may follow these planes to extend intratemporally or intracranially. Advanced imaging and operative techniques are generally recommended for these lesions. Postauricular temporal bone dermoid cysts seem to form a distinct subgroup with a lesser tendency toward deep extension. They may be amenable to more conservative management strategies. METHODS: With IRB-approval, we queried a prospectively-accrued computerized patient-care database to find all postauricular temporal dermoid lesions surgically managed by a single pediatric otolaryngologist from 2001 to 2014. We reviewed the English-language literature to identify similar series of surgically treated pediatric temporal bone dermoid cysts. RESULTS: Ten postauricular temporal dermoid cysts with pathological confirmation were identified in our surgical series. The average size of the lesions was 1.5 cm (0.3-3 cm). The average age at time of surgery was 4 years (6 months-17 years). No intracranial extension was observed at surgery. There were no recurrences noted on last follow-up (mean 65 months, range 10-150 months). A computerized literature review found no examples of intracranial extension among typical postauricular dermoid cysts. CONCLUSION: There was no intracranial or temporal extension in our series or among postauricular lesions described in the literature. Given the low incidence of deep extension we advocate neither advanced imaging nor routine neurosurgical consultation for typical postauricular lesions. Dissection in continuity with cranial periosteum facilitates intact removal of adherent lesions. Surgery is curative if the dermoid is removed intact.

The impact of age at surgery on long-term neuropsychological outcomes in sagittal craniosynostosis.

BACKGROUND: The relationship between surgical age and long-term neuropsychological outcomes in sagittal-suture craniosynostosis remains equivocal. Whole-vault cranioplasty and strip craniectomy are performed at various times in individuals with sagittal-suture craniosynostosis. This study used comprehensive neurological testing to examine the relationship between age at time of surgery and long-term neuropsychological function. METHODS: Seventy sagittal-suture craniosynostosis patients who had previously undergone either whole-vault cranioplasty or strip craniectomy were examined divided into three groups: treatment before 6 months (n = 41), between 6 and 12 months (n = 21), and after 12 months (n = 8). To examine long-term cognitive functioning, participants between the ages of 5 and 25 years underwent neurodevelopmental tests to evaluate intelligence, achievement, and learning disabilities. RESULTS: Compared with those treated between 6 and 12 months and after 12 months, patients who underwent surgery before 6 months demonstrated higher full-scale IQ (p < 0.01) and verbal IQ (p < 0.01). Patients who received surgery before 6 months also demonstrated superior abilities in word reading (p < 0.01), reading comprehension (p < 0.01), spelling (p < 0.01), and numerical operations (p < 0.05) relative to those who had surgery between 6 and 12 months old. A statistically significant higher percentage of patients treated after 6 months had one or more reading-related learning disabilities as compared with those undergoing earlier surgery. CONCLUSIONS: This study suggests that surgery before 6 months old results in improved long-term neurological outcomes. Future studies should examine how the technique of surgery impacts these neuropsychological measures. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

The effects of whole-vault cranioplasty versus strip craniectomy on long-term neuropsychological outcomes in sagittal craniosynostosis.

BACKGROUND: The optimal type of surgical management for isolated sagittal synostosis remains a source of significant debate. There is a paucity of data regarding possible differences in long-term neuropsychological outcomes following treatment with whole-vault cranioplasty or endoscopic strip craniectomy. This study provides the first comparative analysis examining the effects of the two techniques related to long-term intellectual functioning. METHODS: A total of 70 patients were enrolled in this multicenter study, 29 of whom had previously undergone endoscopic strip craniectomy and 41 of whom had previously undergone whole-vault cranioplasty. All patients completed a battery of neurodevelopmental tests (Beery-Buktenica Developmental Test of Visual-Motor Integration, Wechsler Abbreviated Scale of Intelligence, and Wechsler Fundamentals) to evaluate various domains of neuropsychological function. RESULTS: In a group comparison of those treated before 6 months of age, whole-vault patients obtained higher scores relative to endoscopic strip craniectomy patients on visuomotor integration, full-scale intelligence quotient, verbal intelligence quotient, word reading, and reading comprehension (p < 0.05 for all). When compared against strip craniectomy performed before 3 months of age, the whole-vault group still showed significantly higher scores in verbal intelligence quotient, reading comprehension, and word reading (p < 0.05 for all). CONCLUSIONS: The type of surgical intervention for isolated sagittal synostosis impacts long-term neuropsychological outcomes. Patients undergoing early whole-vault cranioplasty attained higher intelligence quotient and achievement scores relative to those undergoing strip craniectomy. Surgical management with whole-vault cranioplasty performed before 6 months of age provides the most favorable long-term intellectual outcomes in patients with isolated sagittal synostosis. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, II.

The spectrum of musculoskeletal problems in lipomyelomeningocele.

PURPOSE: Patients with lipomyelomeningocele (LMMC) represent a unique population within the spectrum of spinal dysraphism. The natural history of LMMC remains poorly defined. The description and prevalence of the presenting orthopaedic clinical signs and symptoms for LMMC have been infrequent and often documented only in general terms. The goal of this study is to define the patterns and prevalence of presenting clinical musculoskeletal signs and symptoms in LMMC patients. METHODS: This study was a retrospective review of charts of all patients identified as having LMMC in our spina bifida clinic. Patient charts with incomplete data or diagnoses other than LMMC were excluded from the analysis. Data collected included age at initial tethered cord release (TCR); repeat TCR; limb length discrepancy; foot deformities; asymmetry of motor and sensory deficits; presence of scoliosis; orthotic needs; assistive devices; functional status. RESULTS: We identified 32 patients with LMMC (21 female and 11 male patients). The majority of patients had their primary TCR by ≤1 year of age (59 %), with 22 and 19 % having primary TCR at ages 1-15 and >15 years, respectively. Fifteen patients had at least one repeat TCR, with ten of these having more than one repeat TCR. A significant relationship was noted between low back/radicular pain and repeat TCR (p < 0.001). Ten patients (31%) had a limb length discrepancy of >2.5 cm, and 53 % of patients had asymmetric involvement. Nine patients (28 %) had scoliosis of whom only one required operative treatment. Fifteen patients had foot deformities. Thirteen patients (41 %) had two or more orthopaedic procedures in addition to other neurologic or urologic procedures. CONCLUSION: The presenting musculoskeletal clinical signs and symptoms in patients with LMMC are uniquely different in terms of both pattern and frequency compared to myelomeningocele and other forms of spinal dysraphism. We noted a high prevalence of asymmetrical involvement, a high operative burden, and a high rate of repeat symptomatic tethered cord syndrome requiring TCR. As previously noted by others, TCR in LMMC does not prevent long-term functional deterioration. These findings may be important to our colleagues providing counsel to their patients with LMMC and to their families.

Potential complications of posterior spine fusion and instrumentation in patients with cerebral palsy treated with intrathecal baclofen infusion.

STUDY DESIGN: The authors evaluated a case series of 5 patients with cerebral palsy and a previously placed subcutaneous reservoir for continuous intrathecal baclofen infusion to control spasticity who underwent spinal arthrodesis with posterior instrumentation for progressive neuromuscular scoliosis deformities. OBJECTIVES: To present our preliminary experience and complications with posterior spine fusion in patients with cerebral palsy and continuous intrathecal baclofen infusion and provide a literature review. SUMMARY OF BACKGROUND DATA: Numerous studies have reported complications associated with intrathecal baclofen infusion, many of these catheter-related. Few reports address complications associated with the intrathecal baclofen infusion, including progressive spinal deformities and complications following spinal arthrodesis. METHODS: Five patients with spastic quadriplegia cerebral palsy had spine fusions for progressive neuromuscular scoliosis. The mean age at surgery was 14.5 years. The mean preoperative major curve was 73 degrees and mean pelvic obliquity was 19 degrees . The patients' medical records were reviewed for complications following reinsertion of the intrathecal catheter following posterior spine instrumentation and arthrodesis, and for progression of the neuromuscular scoliosis, before and after intrathecal baclofen infusion was initiated. RESULTS: Two patients presented with low-pressure headaches in the postoperative period, resulting from cerebrospinal fluid leak following subarachnoid catheter reinsertion and posterior instrumentation. Four patients documented progression of the major scoliosis curve after intrathecal baclofen infusion was begun. A mean progression of 44 degrees occurred over a mean period of 11 months before the spinal arthrodesis. CONCLUSIONS: Low pressure headaches resulting from a cerebrospinal fluid leak following catheter reinsertion may occur in the postoperative period. Preoperative concerns with the baclofen pump reservoir placed subcutaneously and pressure sores were not seen with careful prone positioning on a 4-poster frame. Progression of scoliosis in patients with cerebral palsy requiring spinal arthrodesis was demonstrated in 4 of the patients after continuous intrathecal baclofen was started. The progression of the spinal deformity as a consequence of growth, natural history, or the intrathecal baclofen infusion is unknown at the present time.

Phase II clinical trial of moderate hypothermia after severe traumatic brain injury in children.

OBJECTIVE: To determine whether moderate hypothermia (HYPO) (32-33 degrees C) begun in the early period after severe traumatic brain injury (TBI) and maintained for 48 hours is safe compared with normothermia (NORM) (36.5-37.5 degrees C). METHODS: After severe (Glasgow Coma Scale score < or =8) nonpenetrating TBI, 48 children less than 13 years of age admitted within 6 hours of injury were randomized after stratification by age to moderate HYPO (32-33 degrees C) treatment in conjunction with standardized head injury management versus NORM in a multicenter trial. An additional 27 patients were entered into a parallel single-institution trial of excluded patients because of late transfer or consent (delayed in transfer >6 h but within 24 h of admission), unknown time of injury (e.g., child abuse), and adolescence (e.g., aged 13-18 yr). Assessments of safety included mortality, infection, coagulopathy, arrhythmias, and hemorrhage as well as ability to maintain target temperature, mean intracranial pressure (ICP), and percent time of ICP less than 20 mm Hg during the cooling and subsequent rewarming phases. Additionally, assessments of neurocognitive outcomes were obtained at 3 and 6 months of follow-up. RESULTS: Moderate HYPO after severe TBI in children was found to be safe relative to standard management and NORM in children of all ages and in children with delay of initiation of treatment up to 24 hours. Although there was decreased mortality in HYPO in both studies, there was an increased potential for arrhythmias with HYPO, although they were manageable with fluid administration or rewarming. Additionally, there was a reduction in mean ICP during the first 72 hours after injury in both studies, although rebound ICP elevations in HYPO compared with those in NORM were noted for up to 10 to 12 hours after rewarming. Although functional outcome at 3 or 6 months did not differ between treatment groups, functional outcome tended to improve from the 3- to 6-month cognitive assessment in HYPO compared with NORM, although the sample size was too small for any definitive conclusions. CONCLUSION: HYPO is likely a safe therapeutic intervention for children after severe TBI up to 24 hours after injury. Further studies are necessary and warranted to determine its effect on functional outcome and intracranial hypertension.

Reflections on shunt infection.

The placement and revision of ventriculoperitoneal (VP) shunts remains a mainstay in the surgical treatment of hydrocephalus. While the North American infection rate averages nearly 8-10%, published infection rates for VP shunt infection below 1% have been reported. We retrospectively reviewed shunt operations by a single surgeon over 62 months to analyze the infection rate. In 62 months, we performed 526 shunt placements or revisions in patients up to 18 years of age. There were 7 shunt infections (1.33%). In 5 cases, the organism was Staphylococcus epidermidis, and a single shunt each was infected with Haemophilus influenzae and Staphylococcus aureus. Each infection was treated with external ventriculostomy drainage and intravenous antibiotics. The new shunt was placed at a new incision site after at least 5 days of sterile spinal fluid cultures. The mean follow-up among these patients after shunt insertion was 25 months. VP shunting remains the most common operation for hydrocephalus. Infections are linked with seizures, higher future risks of shunt infection and malfunction, and reduced IQ and school performance. Our infection rate during 62 months was limited to 1.33%. Uniform surgical technique, limited hardware and skin edge manipulation and double gloving may be important factors in limiting shunt infections.