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Fetal surgery for spina bifida

JNSPG 75th Anniversary Invited Review Article

Michael C. Dewan and John C. Wellons III

The two-hit hypothesis of neural injury in the wake of open neural tube defects suggests an opportunity for preservation of function and potential reversibility of early morphological changes in the fetus diagnosed with myelomeningocele. The Management of Myelomeningocele Study (MOMS) demonstrated reduced need for shunting and improved neurological function in patients treated in utero relative to postnatally, thereby offering level 1 evidence supporting fetal repair. Subsequent studies have offered additional information about urological, orthopedic, radiological, and maternal factors surrounding fetal repair. The quest for robust long-term neurocognitive and motor function data is underway and poised to shape the future of fetal repair. In addition, technical innovations such as fetoscopic surgery aim to minimize maternal morbidity while conferring the beneficial effects observed with open intrauterine intervention.

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John C. Wellons III and R. Shane Tubbs

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John C. Wellons III

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Michael C. Dewan, Jaims Lim, Chevis N. Shannon and John C. Wellons III

OBJECTIVE

Up to one-third of patients with a posterior fossa brain tumor (PFBT) will experience persistent hydrocephalus mandating permanent CSF diversion. The optimal hydrocephalus treatment modality is unknown; the authors sought to compare the durability between endoscopic third ventriculostomy (ETV) and ventriculoperitoneal shunt (VPS) therapy in the pediatric population.

METHODS

The authors conducted a systematic review of articles indexed in PubMed between 1986 and 2016 describing ETV and/or VPS treatment success/failure and time-to-failure rate in patients < 19 years of age with hydrocephalus related to a PFBT. Additionally, the authors conducted a retrospective review of their institutional series of PFBT patients requiring CSF diversion. Patient data from the systematic review and from the institutional series were aggregated and a time-to-failure analysis was performed comparing ETV and VPS using the Kaplan-Meier method.

RESULTS

A total of 408 patients were included from 12 studies and the authors' institutional series: 284 who underwent ETV and 124 who underwent VPS placement. The analysis included uncontrolled studies with variable method and timing of CSF diversion and were subject to surgeon bias. No significant differences between cohorts were observed with regard to age, sex, tumor grade or histology, metastatic status, or extent of resection. The cumulative failure rate of ETV was 21%, whereas that of VPS surgery was 29% (p = 0.105). The median time to failure was earlier for ETV than for VPS surgery (0.82 [IQR 0.2–1.8] vs 4.7 months [IQR 0.3–5.7], p = 0.03). Initially the ETV survival curve dropped sharply and then stabilized around 2 months. The VPS curve fell gradually but eventually crossed below the ETV curve at 5.7 months. Overall, a significant survival advantage was not demonstrated for one procedure over the other (p = 0.21, log-rank). However, postoperative complications were higher following VPS (31%) than ETV (17%) (p = 0.012).

CONCLUSIONS

ETV failure occurred sooner than VPS failure, but long-term treatment durability may be higher for ETV. Complications occurred more commonly with VPS than with ETV. Limited clinical conclusions are drawn using this methodology; the optimal treatment for PFBT-related hydrocephalus warrants investigation through prospective studies.

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Travis R. Ladner, Ashly C. Westrick, John C. Wellons III and Chevis N. Shannon

OBJECT

The purpose of this study was to design and validate a patient-reported health-related quality of life (HRQOL) instrument for pediatric Chiari Type I malformation (CM-I), the Chiari Health Index for Pediatrics (CHIP).

METHODS

The CHIP has 45 items with 4 components making up 2 domain scores, physical (pain frequency, pain severity, nonpain symptoms) and psychosocial; physical and psychosocial scores are combined to create an overall HRQOL score. Increasing scores (0 to 1) represent increasing HRQOL. Fifty-five patients with CM-I (mean age 12 ± 4 years, 53% male) were enrolled and completed the CHIP and Health Utilities Index Mark 3 (HUI3). Twenty-five healthy controls (mean age 11.9 ± 4 years, 40% male) also completed the CHIP. CHIP scores were compared between these groups via the Mann-Whitney U-test. For CHIP discriminative function, subscore versus presence of CM-I was compared via receiver operating characteristic curve analysis. CHIP scores in the CM-I group were stratified by symptomatology (asymptomatic, headaches, and paresthesias) and compared via Kruskal-Wallis test with Mann-Whitney U-test with Bonferroni correction (p < 0.0167). CHIP was compared with HUI3 (Health Utilities Index Mark 3) via univariate and multivariate linear regression.

RESULTS

CHIP physical and psychosocial subscores were, respectively, 24% and 18% lower in CM-I patients than in controls (p < 0.001); the overall HRQOL score was 23% lower as well (p < 0.001). The area under the curve (AUC) for CHIP physical subscore versus presence of CM-I was 0.809. CHIP physical subscore varied significantly with symptomatology (p = 0.001) and HUI3 pain-related quality of life (R2 = 0.311, p < 0.001). The AUC for CHIP psychosocial subscore versus presence of CM-I was 0.754. CHIP psychosocial subscore varied significantly with HUI3 cognitive- (R2 = 0.324, p < 0.001) and emotion-related (R2 = 0.155, p = 0.003) quality of life. The AUC for CHIP HRQOL versus presence of CM-I was 0.820. Overall CHIP HRQOL score varied significantly with symptomatology (p = 0.001) and HUI3 multiattribute composite HRQOL score (R2 = 0.440, p < 0.001).

CONCLUSIONS

The CHIP is a patient-reported, CM-I-specific HRQOL instrument, with construct validity in assessing pain-, cognitive-, and emotion-related quality of life, as well as symptomatic features unique to CM-I. It holds promise as a discriminative HRQOL index in CM-I outcomes assessment.

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R. Shane Tubbs, John C. Wellons III, Jeffrey P. Blount, Paul A. Grabb and W. Jerry Oakes

✓The authors report on three pediatric patients with ventriculoperitoneal (VP) shunts who presented with chronic right shoulder pain. Imaging revealed that the distal peritoneal catheter was positioned between the right hemidiaphragm and liver. Following surgical repositioning of the distal tubing, all patients experienced resolution of their shoulder pain, which has not recurred. Although seemingly rare, referred shoulder pain from a VP shunt should be added to the list of complications seen with this method of cerebrospinal fluid diversion. The clinician who cares for patients with VP shunts may wish to evaluate cases of shoulder pain without obvious neural or musculoskeletal cause by performing imaging of the distal shunt tubing.

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R. Shane Tubbs, John C. Wellons III, Jeffrey P. Blount and W. Jerry Oakes

✓The authors present three children born with myelomeningocele and hydrocephalus. Each presented with symptoms/signs of ventriculoperitoneal shunt malfunction. All patients at the time of presentation exhibited significant urinary bladder infections and were appropriately treated for their infection. No patient was found to have an underlying shunt infection. All patients without medically threatening symptoms were carefully observed and noted to have resolution of their shunt dysfunction symptoms/signs following treatment of their urinary bladder infections and thus did not undergo a shunt operation.

Based on the courses of these patients, we believe that significant urinary bladder infection in patients with myelodysplasia in whom a shunt has been placed may often be enough to bring a subclinical shunt malfunction to clinical attention or even to be the cause of temporary distal peritoneal shunt malabsorption. Although the exact mechanism for this dysfunction is unclear, treatment of the bladder infection may address the symptomatic shunt dysfunction in some patients so as to avoid operative intervention. We emphasize, however, that careful observation of these patients should be performed during hospitalization because they often rely on adequate cerebrospinal fluid diversion. Only patients with mild symptomatology should be observed first as the initial line of treatment.

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R. Shane Tubbs, John C. Wellons III, Bermans J. Iskandar and W. Jerry Oakes

Object. Historically, cutaneous stigmata representative of occult spinal dysraphism (OSD) have included lumbar hemangiomas. Frequently, this skin change is found in conjunction with other cutaneous alterations such as dermal sinus tracts and subcutaneous lipomas. Debate has recently surrounded the question of whether these skin changes in isolation might indicate underlying spinal disease. The authors reviewed their experience in their most recent 120 cases in which OSD was diagnosed.

Methods. The authors retrospectively reviewed records obtained in 120 patients with OSD. They found that many of the patients reviewed harbored only a flat capillary hemangioma as an indicator of OSD. In 21 patients (17.5%) with only midline lumbosacral flat capillary hemangiomas, underlying OSD was present. No single variety of OSD had a higher incidence of association with this single cutaneous stigma.

Conclusions. Based on their experience, the authors recommend magnetic resonance (MR) imaging evaluation in cases involving this skin lesion in isolation to discern the potential for surgically significant spinal cord anomalies. Prospective studies are now needed to examine MR images obtained in all children with this lesion in the midline lumbosacral spine and assess for OSD.