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Sandi Lam, Ramesh Grandhi, and Stephanie Greene

Meconium staining of open myelomeningoceles has been reported to occur both prenatally and postnatally, but meconium staining of the brainstem has not been previously documented. The authors present a case of meconium staining of the brainstem in an infant with a meconium-stained myelomeningocele, Chiari malformation Type II, and hydrocephalus and discuss possible implications for prenatal and perinatal care.

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Michael D. White, Michael M. McDowell, Nitin Agarwal, and Stephanie Greene

OBJECTIVE

Myelomeningocele (MMC) is frequently complicated by symptomatic hydrocephalus, necessitating early permanent CSF diversion and revision surgeries. Shunt infections are a common cause of shunt malfunction. This study aims to characterize long-term shunt-related outcomes of patients undergoing MMC closure.

METHODS

A total of 170 patients undergoing MMC closure between the years of 1995 and 2017 were identified from a retrospective review of a prospectively populated surgical database at the Children’s Hospital of Pittsburgh. Patients who underwent MMC closure and required ventriculoperitoneal (VP) shunt insertion met criteria and were included in the primary study analysis. Analysis with a Fisher exact test was performed for categorical variables, and Mann-Whitney U-tests were utilized for numerical data.

RESULTS

Of the 158 total patients undergoing MMC closure and meeting inclusion criteria, 137 (87%) required VP shunt insertion. These 137 patients demonstrated a shunt revision rate of 21.1% per person-year and a shunt infection rate of 2.1% per person-year over a mean follow-up of 10.8 years. Patients had a mean of 3.4 ± 0.6 shunt surgeries prior to their first infection. Patients undergoing immediate shunt removal, external ventricular drain placement, or shunt replacement after clearing the infection had lower rates of subsequent infections than patients who initially were managed with shunt externalization (p < 0.001). Placement of a shunt at the time of MMC closure was not found to be a risk factor for infection. Of patients with initial shunt placement after the implementation of the Hydrocephalus Clinical Research Network protocol in 2011, the authors’ institution has had a shunt infection rate of 4.2% per person-year and a revision rate of 35.7% per person-year.

CONCLUSIONS

This study describes long-term outcomes of shunted MMC patients and factors associated with shunt infections. Most patients underwent multiple revisions prior to the first shunt infection. Shunt externalization may be ineffective at clearing the infection and should be avoided in favor of early shunt removal and external ventricular drainage, followed by shunt replacement once infection is demonstrated to have cleared.

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Christopher M. Bonfield, Sandi Lam, Yimo Lin, and Stephanie Greene

Object

Attention deficit hyperactivity disorder (ADHD) and traumatic brain injury (TBI) are significant independent public health concerns in the pediatric population. This study explores the impact of a premorbid diagnosis of ADHD on outcome following mild TBI.

Methods

The charts of all patients with a diagnosis of mild closed head injury (CHI) and ADHD who were admitted to Children's Hospital of Pittsburgh between January 2003 and December 2010 were retrospectively reviewed after institutional review board approval was granted. Patient demographics, initial Glasgow Coma Scale (GCS) score, hospital course, and King's Outcome Scale for Childhood Head Injury (KOSCHI) score were recorded. The results were compared with a sample of age-matched controls admitted with a diagnosis of CHI without ADHD.

Results

Forty-eight patients with mild CHI and ADHD, and 45 patients with mild CHI without ADHD were included in the statistical analysis. Mild TBI due to CHI was defined as an initial GCS score of 13–15. The ADHD group had a mean age of 12.2 years (range 6–17 years), and the control group had a mean age of 11.14 years (range 5–16 years). For patients with mild TBI who had ADHD, 25% were moderately disabled (KOSCHI Score 4b), and 56% had completely recovered (KOSCHI Score 5b) at follow-up. For patients with mild TBI without ADHD, 2% were moderately disabled and 84% had completely recovered at follow-up (p < 0.01). Patients with ADHD were statistically significantly more disabled after mild TBI than were control patients without ADHD, even when controlling for age, sex, initial GCS score, hospital length of stay, length of follow-up, mechanism of injury, and presence of other (extracranial) injury.

Conclusions

Patients who sustain mild TBIs in the setting of a premorbid diagnosis of ADHD are more likely to be moderately disabled by the injury than are patients without ADHD.

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Gurpreet S. Gandhoke, Ezequiel Goldschmidt, Robert Kellogg, and Stephanie Greene

A fetal MRI study obtained at 21 weeks’ gestation revealed a suboccipital meningocele without hydrocephalus. One day after term birth, MRI demonstrated an acquired cerebellar encephalocele, and MRI obtained 5 months later showed progressive enlargement of the encephalocele, still without obvious hydrocephalus. The patient underwent an operation in which an external ventricular drain was placed, the grossly normal cerebellum was reduced into the posterior fossa without resection, and the dural defect was closed. The drain was weaned out over 5 days, and no ventriculoperitoneal shunt was placed. Postoperative MR images revealed normal cerebellum and no hydrocephalus. The patient is developmentally normal. Meningocele and encephalocele are embryologically distinct. An acquired encephalocele could develop from hydrocephalus (which was not present in this case), or secondary to the lower resistance to expansion into the dural defect of the meningocele relative to the resistance to expansion of the fetal skull. The cerebellar tissue was normal in this case, and was thus preserved. The developmental prognosis is excellent. To the authors’ knowledge, this is the first reported case of this occurrence. It is important to differentiate between congenital and acquired encephalocele etiologies, because resection of the cerebellar tissue in an acquired encephalocele (as is routinely done in cases of congenital encephalocele) would be expected to result in neurological deficits.

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Mahesh Karandikar, Robert F. Yellon, Geoffrey Murdoch, and Stephanie Greene

Dermoid cysts, encephaloceles, and dermal sinus tracts represent abnormalities that develop during the process of embryogenesis. The elucidation of the precise timing of formation for these malformations has remained elusive at the molecular level of study. Yet, clinical experience has demonstrated that these malformations do not all occur in the same patient, suggesting a shared pathway that goes awry at distinct points for different patients, resulting in 1 of the 3 malformations. Herein the authors describe a case in which all 3 malformations were present in a single patient. This is the first description in the English literature of a sincipital encephalocele occurring with a dermoid cyst and a dermal sinus tract.

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Kimberly A. Foster, Sandi Lam, Yimo Lin, and Stephanie Greene

Object

Tethered cord (TC) is a neurological disorder caused by tissue attachments that limit the normal movement of the spinal cord. A TC can be unmasked by a cutaneous abnormality or manifest clinically in myriad neurological, urological, and orthopedic symptoms. The relationship between TC and height is previously unknown. This study investigates the association between TC release and changes in height profiles in the pediatric population.

Methods

Fifty-two children undergoing first-time TC release at a single institution were examined retrospectively. Clinical symptoms, radiographic findings, pre- and postoperative height, and height-for-age percentiles were recorded and analyzed.

Results

Children with TC experienced a statistically significant increase in age-adjusted height percentiles after TC release (p = 0.0028), with a mean increase of 7 percentile points (from 48.1st to 54.9th percentile). When stratified by age, children 5 years or older (5–18 years) demonstrated a mean percentile increase of 10 percentile points (from 46.7th to 56.4th percentile) (p = 0.0001). Among the same age group, this effect scaled significantly with age (p = 0.02, beta coefficient −1.3). There was no significant difference in height-for-age after detethering surgery in children younger than 5 years. There was no significant association between the presence of clinical symptoms or specific radiographic findings and height outcomes after surgery. Overall, 56% of TC-related clinical symptoms improved after detethering (mean follow-up 4.6 months). Among children younger than 5 years, 82% of TC-related clinical symptoms improved after detethering (average follow-up 4.5 months); in children 5–18 years, 47% of symptoms improved after detethering (average follow-up 4.8 months).

Conclusions

The authors observed a statistically significant gain in height-for-age percentiles in children undergoing surgical release of TC. The authors' data suggest that such gains may be more significant in older children (≥ 5 years) and the increase appears to scale positively with youth in the older cohort. In this study, postoperative height gains did not appear to correlate with the presence of TC-related clinical symptoms or radiographic findings. Further investigation is necessary to elucidate any potential correlation between release of TC and height changes in children postoperatively.

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Ali Kooshkabadi, Brian Jankowitz, Phillip A. Choi, Gregory M. Weiner, and Stephanie Greene

The authors present the case of a boy who was successfully managed through the spontaneous thrombosis of a cavernous internal carotid artery (ICA) aneurysm, the subsequent occlusion of the ICA, its recanalization, and ultimate endovascular sacrifice, using only two angiograms because of the diagnostic capability of CT angiography. Spontaneous recanalization of the ICA following occlusion in the setting of a giant aneurysm has not been previously reported.

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Jennifer L. Perez, Michael M. McDowell, Benjamin Zussman, Ashutosh P. Jadhav, Yosuke Miyashita, Patrick McKiernan, and Stephanie Greene

Aneurysmal rupture can result in devastating neurological consequences and can be complicated by comorbid disease processes. Patients with autosomal recessive polycystic kidney disease (ARPKD) have a low rate of reported aneurysms, but this may be due to the relative high rate of end-stage illnesses early in childhood. Authors here report the case of a 10-year-old boy with ARPKD who presented with a Hunt and Hess grade V subarachnoid hemorrhage requiring emergency ventriculostomy, embolization, and decompressive craniectomy. Despite initial improvements in his neurological status, the patient succumbed to hepatic failure. Given the catastrophic outcomes of subarachnoid hemorrhage in young patients, early radiographic screening in those with ARPKD may be warranted.

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Robert Kellogg, Philip Lee, Christopher P. Deibert, Zachary Tempel, Nathan T. Zwagerman, Christopher M. Bonfield, Stephen Johnson, and Stephanie Greene

OBJECTIVE

The authors reviewed 20 years’ experience with the surgical management of open myelomeningocele in a well-defined retrospective cohort from a single large academic medical center. Their goal was to define the characteristics of a modern cohort of children with myelomeningocele to allow for evidence-based decision-making for the treatment of these patients.

METHODS

After IRB approval was obtained, the authors queried an operative database maintained by the Department of Neurological Surgery at Children’s Hospital of Pittsburgh for patients who underwent closure of a myelomeningocele between 1995 and 2015. They identified 153 infants, and a retrospective chart review was performed.

RESULTS

Eighty-eight percent of the patients required placement of a ventriculoperitoneal shunt, and 15% of these patients acquired shunt-related infections. Eighteen percent of patients underwent Chiari malformation type II (CM-II) decompression. Sixteen percent of patients underwent a tethered cord release. Three percent of patients died within the 1st year of life. Predictors of an early demise included poor Apgar scores, large head circumference, and need for early CM-II decompression. Functional motor outcome was slightly better than predicted by anatomical level of defect.

CONCLUSIONS

Myelomeningoceles represent a severe birth defect with life-threatening complications. The authors provide long-term follow-up data and insight into factors that contribute to early death.

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Jason S. Hauptman, Robert Bollo, Rama Damerla, Brian Gibbs, Cecilia Lo, Aviva Katz, and Stephanie Greene

Myelomeningocele and gastroschisis, on their own, are both relatively common entities encountered in pediatric surgical care. Coexistence of these pathologies, however, is exceedingly rare. The authors report on 2 patients who presented with myelomeningocele and gastroschisis at birth. They obtained blood for whole-exome analysis for one of the patients and identified 3 mutations that could be related to the underlying anomalies: homozygous mutations in FAM171B and ABCA1 and a hemizygous (X-linked) mutation in COL4A5. Of these, FAM171B and ABCA1 both have function that may be related to the underlying disease.