Epidural Effusion as Allergic Reaction Following Polyetheretherketone Cranioplasty: An Illustrative Case and Review of the Literature.

Well-described complications of polyetheretherketone (PEEK) cranioplasty in pediatric patients include surgical site infection, post-operative hematoma, cerebral edema, and implant fracture. We present a rare case of hypersensitivity to PEEK presenting as an epidural effusion in a 7-year-old male receiving a PEEK cranioplasty following a decompressive craniectomy. Within three weeks, the patient experienced fever and emesis. Erythrocyte sedimentation rate (ESR) was high (>130 mm/Hr) as well as C-reactive protein (CRP) (6.4 mg/dL). A brain MRI with contrast demonstrated both subgaleal and epidural fluid collections with T2 isointense columns projecting from the galeal surface, through the holes in the implant to the dural surface. The patient appeared clinically well. A sterile tap of the pericranial fluid showed no growth, b2-transferrin was negative, but the IgG level was high (>129.2 mg/dL) in the tap fluid. High-dose steroids reduced the epidural collection, but then the collection returned with steroid wean. A second cranioplasty operation replaced the PEEK flap with autologous bone. Postoperative imaging demonstrated markedly reduced fluid collections and a decreased midline shift. The patient remained clinically intact throughout the experience. PEEK allergy following cranioplasty is a rare entity and must be distinguished from infection or hematoma. Medical treatment with steroids can be attempted, but, if refractory, then appropriate treatment may necessitate removal of the offending PEEK implant.

Risk of occult spinal dysraphism based on lumbosacral cutaneous manifestations.

OBJECTIVES: Occult spinal dysraphism is a congenital failure of fusion of the posterior vertebral arches with intact skin overlying the defect. Lumbosacral cutaneous manifestations are associated with a variable risk of occult spinal dysraphism. Tethered cord syndrome is a type of occult spinal dysraphism that puts abnormal traction on the spinal cord. This study analyzed neonates and infants who were referred to our pediatric urology practice and had evidence of lumbosacral cutaneous manifestation on physical examination. METHODS: We reviewed the presence of lumbosacral cutaneous manifestations in neonates and infants evaluated in our pediatric urology clinic at our Institution over a 6-year period (1 March, 2015-28 February, 2021) with no prior diagnosis of lumbosacral cutaneous manifestation. All patients underwent a spinal ultrasound. RESULTS: The most common lumbosacral cutaneous manifestations were bifurcated/duplicated gluteal folds (33%), gluteal asymmetry (19%), and sacral dimples (14%). A total of 34 (24%) patients had an abnormal spinal ultrasound; 15 (44%) of these infants underwent a lumbar magnetic resonance imaging. A coccygeal pit was statistically marginally higher in abnormal versus normal spinal ultrasound (p = 0.07). Patients with only one lumbosacral cutaneous manifestation (N = 121) were significantly more likely to have a normal spinal ultrasound compared to those with two or more lumbosacral cutaneous manifestation (N = 17) (79% vs 53%, p = 0.03). CONCLUSION: Due to the varying risk of certain lumbosacral cutaneous manifestations with occult spinal dysraphism, all patients with a lumbosacral cutaneous manifestation should undergo spinal ultrasound. This study also highlights the importance of urodynamic studies when there are abnormal cutaneous findings. Routine physical examinations of the lumbar region for cutaneous manifestations of occult spinal dysraphism are vital to ensure prompt management of tethered cord syndrome and avoid potentially devastating consequences.

Importance of Physical Examination and Imaging in the Detection of Tethered Cord Syndrome.

Tethered cord syndrome (TCS) is a type of occult spinal dysraphism that may lead to permanent neurologic and orthopedic deficits. Infants with TCS may have lumbosacral cutaneous malformations (LsCMs). We studied 67 infants referred to a single pediatric urology practice for a urological concern unrelated to occult spinal dysraphism with no prior diagnosis of LsCM between March 1, 2015 and September 30, 2018. Each infant underwent a spinal ultrasound. If an abnormality was detected, they were referred to a pediatric neurosurgeon. The most common cutaneous manifestations were duplicated or bifurcated (46%) gluteal folds and gluteal asymmetry (16%). Fourteen (21%) of the 67 patients had an abnormal spinal ultrasound; 5 of the 14 infants underwent a lumbar magnetic resonance imaging. One infant had urodynamics studies and a tethered cord release. Pediatricians should be familiar with TCS and perform lumbar physical examinations for LsCMs suggestive of TCS to ensure prompt diagnosis and management and avoid potentially devastating complications.

Congenital myotonic dystrophy: ventriculomegaly and shunt considerations for the pediatric neurosurgeon.

PURPOSE: Ventriculomegaly in infants with congenital myotonic dystrophy (CDM) is common, and the neurosurgical determination of shunting is complex. The natural history of CDM-associated ventriculomegaly from prenatal to natal to postnatal stages is poorly known. The relationship between macrocephaly and ventriculomegaly, incidence of shunt necessity, and early mortality outcomes lack pooled data analysis. This study aims to review clinical features and pathophysiology of CDM, with emphasis on ventriculomegaly progression, ventriculomegaly association with macrocephaly, and incidence of shunting. METHODS: This is a literature review with pooled data analysis and case report. RESULTS: One hundred four CDM patients were reviewed in 13 articles that mentioned CDM with ventriculomegaly and/or head circumference. Data was very limited: only 7 patients had data on the presence or absence of prenatal ventriculomegaly, 97 on ventriculomegaly at birth, and 32 on whether or not the ventricles enlarged post-natally. Three patients of 7 (43 %) had pre-natally diagnosed ventriculomegaly, 43 of 97 (44 %) had ventriculomegaly at birth, and only 5 of 32 (16 %) had progressive enlargement of ventricles post-natally. Only 5 of 104 patients had a documented shunt placement: 1 for obstructive, 1 for a post-hemorrhagic communicating, 2 for a communicating hydrocephalus without hemorrhage, and 1 with unknown indication. Of 13 macrocephalic patients with data about ventricular size, 12 had ventriculomegaly. CONCLUSIONS: Ventriculomegaly occurs regularly with CDM but most often does not require CSF diversion. Decisions regarding neurosurgical intervention will necessarily be based on limited information, but shunting should only occur once dynamic data confirms hydrocephalus.

Selective dorsal rhizotomy for spasticity not associated with cerebral palsy: reconsideration of surgical inclusion criteria.

Children with spastic diplegia from cerebral palsy (CP) experience measurable improvement in their spasticity and motor function following selective dorsal rhizotomy (SDR). The role of this operation in the treatment of other spasticity causes is less well defined. A literature review was undertaken to survey outcomes from SDRs performed outside the CP population. Multiple sclerosis was the most common diagnosis found, accounting for 74 of 145 patients described. Selective dorsal rhizotomies have also been reported in patients with traumatic brain and spinal cord injuries, ischemic and hemorrhagic stroke, neurodegenerative disease, hypoxic encephalopathy, and other causes of spasticity. Outcomes from surgery are generally described as favorable, although postoperative assessments and follow-up times are not standardized across reports. Long-term outcomes are sparsely reported. Larger numbers of patients and more detailed outcomes data have the potential to form a basis for expanding the inclusion criteria for SDR.

Complications associated with molding helmet therapy for positional plagiocephaly: a review.

Molding helmet therapy is a widely accepted treatment for positional plagiocephaly that is generally considered to be low risk. Multiple large outcome studies have shown good results, but adverse events are rarely reported. The literature on helmet therapy was reviewed to clarify the clinical experience with associated complications. Although significant complications were extremely rare, there was a large degree of variability in detection of lesser problems such as minor skin irritation. Patients with a primarily brachycephalic morphology may be at higher risk for poorly fitting orthoses. Most reported complications are minor and self-limited. Maintenance of good helmet hygiene appears to be the most effective strategy for reducing or eliminating complications.

Nonendoscopic, minimally invasive calvarial vault remodeling without postoperative helmeting for sagittal synostosis.

OBJECT: Multiple surgical procedures have been described for the management of isolated nonsyndromic sagittal synostosis. Minimally invasive techniques have been recently emphasized, but these techniques necessitate the use of an endoscope and postoperative helmeting. The authors assert that a safe and effective, more "minimalistic" approach is possible, avoiding the use of endoscopic visualization and routine postoperative application of a cranial orthosis. METHODS: A single-institution cohort analysis was performed on 18 cases involving infants treated for isolated nonsyndromic sagittal synostosis between 2008 and 2010 using a nonendoscopic, minimally invasive calvarial vault remodeling (CVR) procedure without postoperative helmeting. The surgical technique is described. Variables analyzed were: age at time of surgery, sex, estimated blood loss (EBL), operative time, intraoperative complications, postoperative complications, length of stay, pre- and postoperative cephalic index (CI), clinical impressions, and results of a 5-question nonstandardized questionnaire administered to patient caregivers regarding outcome. RESULTS: Eleven male and 7 female infants (mean age 2.3 months) were included in the study. The mean duration of follow-up was 16.4 months (range 6-38 months). The mean procedural time was 111 minutes (range 44-161 minutes). The mean length of stay was 2.3 days (range 2-3 days). The mean EBL in all 18 patients was 101.4 ml (range 30-475 ml). One patient had significant bone bleeding resulting in an EBL of 475 ml. Excluding this patient, the mean EBL was 79.4 ml (range 30-150 ml). There were no deaths or intraoperative complications; one patient had a superficial wound infection. The mean CI was 69 preoperatively versus 79 postoperatively, a statistically significant difference (p < 0.0001). Two patients were offered helmeting for suboptimal surgical outcome; one family declined and the single helmeted patient showed improvement at 2 months. No patient has undergone further surgery for correction of primary deformity, secondary deformities, or bony irregularities. Complete questionnaire data were available for 14 (78%) of the 18 patients; 86% of the respondents were pleased with the cosmetic outcome, 92% were happy to have avoided helmeting, 72% were doubtful that helmeting would have provided more significant correction, and 86% were doubtful that further surgery would be necessary. Small, palpable, aesthetically insignificant skull irregularities were reported by family members in 6 cases (43%). CONCLUSIONS: The authors present a nonendoscopic, minimally invasive CVR procedure without postoperative helmeting. Their small series demonstrates this to be a safe and efficacious procedure for isolated nonsyndromic sagittal synostosis, with improvements in CI at a mean follow-up of 16.1 months, commensurate with other techniques, and with overall high family satisfaction. Use of a CVR cranial orthosis in a delayed fashion can be effective for the infrequent patient in whom this approach results in suboptimal correction.

Motion segment-sparing repair of symptomatic chronic pars defects.

OBJECT: The current standard of care for symptomatic chronic spondylolysis (SP) is a one-level posterior spinal fusion for defects at L-5 or direct pars repair (motion segment sparing) for more rostral SP in younger patients and if no disc degeneration or listhesis is present. Since many patients with SP undergoing operative repair are young, a procedure with the lowest biomechanical profile is desirable, and direct pars repair is recommended. The authors here explore the limits of direct pars repair. METHODS: A retrospective review of all patients who underwent direct repair of SP between 2002 and 2009 was performed. Data were analyzed for predictors of symptom relief and radiographic fusion failure. RESULTS: Of 49 patients, only 7 required a reoperation to treat clinical symptoms, and 6 of them were female (p = 0.049). In all cases of treatment failure, the patient had bilateral L-5 SP. Patients with a slip percentage as high as 30% experienced radiographic fusion and symptom relief. Disc degeneration (measured using the Modified Pfirrmann Scale) did not predict symptom persistence or radiographic fusion failure. Patients with high-grade disc disease experienced symptom relief. The authors found no predictors of treatment failure. CONCLUSIONS: The number of patients undergoing motion segment-sparing fusions of symptomatic chronic SP can be safely increased to include patients with Grade I spondylolisthesis as well as high-grade disc disease. Female patients with bilateral L-5 SP and low lordotic angles may be better served by a posterior spinal fusion from L-5 to S-1.

Decompression of Chiari malformation with and without duraplasty: morbidity versus recurrence.

OBJECT: The optimal surgical management of Chiari malformation (CM) is evolving. Evidence continues to accrue that supports decompression without duraplasty as an effective treatment to achieve symptomatic relief and anatomical decompression. The risks and benefits of this less invasive operation need to be weighed against decompression with duraplasty. METHODS: The authors performed a retrospective review of all CM decompressions from 2003 to 2007. All operations were performed by a single surgeon at a single institution. Data were analyzed for outcome, postoperative morbidity, and recurrence. RESULTS: Of 121 unique patients, 56 underwent posterior fossa decompressions without duraplasty (PFD) and 64 patients underwent posterior fossa decompressions with duraplasty (PFDD). Of the 56 PFD patients, 7 (12.5%) needed a subsequent PFDD for symptomatic recurrence. Of the 64 patients who underwent a PFDD, 2 (3.1%) needed a repeated PFDD for symptomatic recurrence. Patients treated with PFDD had an average operative time of 201 minutes in contrast to 127 minutes for those who underwent PFD (p = 0.0001). Patients treated with PFDD had average hospital stays of 4.0 days, whereas that for patients treated with PFD was 2.7 days (p = 0.0001). While in the hospital, patients treated with PFDD used low-grade narcotics, intravenous narcotics, muscle relaxants, and antiemetic medications at statistically significant differing rates. CONCLUSIONS: While PFD was associated with a higher rate of recurrent symptoms requiring repeated decompression, this may be justified by the significantly lower morbidity rate. Clearer delineation of the trade-off between morbidity and recurrence may be used to help patients and their families make decisions regarding care.

Neurosurgical uses for intraprocedural magnetic resonance imaging.

Neurosurgical procedures demand precision, and efforts to create accurate neurosurgical navigation have been central to the profession through its history. Magnetic resonance image (MRI)-guided navigation offers the possibility of real-time, image-based stereotactic information for the neurosurgeon, which makes possible a number of diagnostic and therapeutic procedures. This article will review both current options for intraoperative MRI operative suite arrangements and the current therapeutic/diagnostic uses of intraoperative MRI.

Expanding the boundaries of medical education: evidence for cross-cultural exchanges.

PROBLEM STATEMENT AND BACKGROUND: Cross-cultural experiences are in increasing demand by both graduate and undergraduate medical students, yet the benefits of these experiences are not clearly established. METHOD: A review of the literature was conducted to identify articles on the outcomes of cross-cultural experiences. Themes were identified and categorized into domains. RESULTS: Forty-two studies were found; 27 articles used qualitative methods, nine used quantitative methods, and six used both. Most (24) were from the nursing literature, 18 were from the medical literature. All studies reported positive outcomes along four domains: students' professional development, students' personal development, medical school benefits, and host population benefits. CONCLUSIONS: Studies reviewed were primarily case controlled or case series. Future research is needed that more clearly defines outcome measures and uses more rigorous methods. Although results suggest positive outcomes in all domains, additional research is needed before cross-cultural rotations can be supported based on evidence.