Chan YY, Sandlin SK, Kurzrock EA. (2017) Urological Outcomes of Myelomeningocele and Lipomeningocele. Current Urological Reports. 2017 May;18(5):35. doi: 10.1007/s11934-017-0684-9. Review.

 

Abstract

PURPOSE OF REVIEW:

Spina bifida is caused by incomplete neural tube closure during the first trimester. This condition may lead to bowel and bladder dysfunction as well as truncal weakness and motor anomalies. Presentations vary between myelomeningoceles and lipomeningoceles and may result in different outcomes. This review seeks to explore our current understanding of the variations in outcomes between individuals with myelomeningocele and lipomeningocele.

RECENT FINDINGS:

Prenatal intervention has become a standard of care for prenatal diagnoses of myelomeningocele and has been shown to reduce shunt placement and improve motor skills. However, urological benefit from early intervention remains to be seen. Early surgical repair, however, may be beneficial for patients with lipomeningocele. Literature on the urological outcomes of patients with myelomeningocele and lipomeningocele is lacking. Further research is needed to better elucidate differences in long-term urological outcomes between these two pathologies.

Update on Urological Management of Spina Bifida from Prenatal Diagnosis to Adulthood.

Snow-Lisy DC, Yerkes EB, Cheng EY. (2015) Update on Urological Management of Spina Bifida from Prenatal Diagnosis to Adulthood.

J Urol. 2015 Apr 1. pii: S0022-5347(15)03647-2. doi: 10.1016/j.juro.2015.03.107

Abstract

PURPOSE:

We review the current literature regarding urological management of spina bifida from prenatal diagnosis to adulthood.

MATERIALS AND METHODS:

We searched MEDLINE, EMBASE and PubMed for English articles published through December 2014 using search terms "spina bifida," "spinal dysraphism" and "bladder." Based on review of titles and abstracts, 437 of 1,869 articles were identified as addressing topics related to open spina bifida in pediatric patients, or long-term or quality of life outcomes in adults with spina bifida. We summarize this literature to inform clinical guidelines and create a framework for disease management.

RESULTS:

The birth prevalence of spina bifida in the United States has recently plateaued at approximately 30 per 100,000. With improved management more individuals are surviving to adulthood, with an economic impact of $319,000 during the lifetime of an individual with spina bifida. Recent advances in prenatal surgery have demonstrated that prenatal closure of spina bifida is possible. To assess safety and efficacy, the National Institutes of Health sponsored MOMS (Management of Myelomeningocele Study) was undertaken, in which subjects were randomized to prenatal or postnatal closure. Until the urological results of this trial are published, the impact of prenatal intervention on future bladder function remains unclear. Controversy continues regarding the optimal use and timing of urodynamic studies, and the indications for initiation of clean intermittent catheterization and anticholinergics in infants and children. Many favor expectant management, while others argue for a more proactive approach. Based on the current literature, both approaches appear to protect the child from renal injury, although delayed intervention may increase rates of bladder augmentation. The current literature regarding this topic is difficult to interpret and compare due to heterogeneity of patient populations, variable outcome measures and lack of reporting of quality of life outcomes. Surgical intervention is indicated for those at risk for renal deterioration and/or is considered for children who fail to achieve satisfactory continence with medical management. Traditionally surgery concentrates on 3 areas, ie bladder and bladder neck, and creation of catheterizable channels. For those with a hostile bladder enterocystoplasty remains the gold standard for bladder augmentation, although use of bowel for augmentation remains suboptimal due to secondary complications, including increased risk of infections, metabolic abnormalities, neoplastic transformation and risk of life threatening perforation. Recent advances in tissue engineering technology may provide an alternative to traditional augmentation. However, recent results from phase II trials using current techniques to augment the bladder with engineered bladder tissue are disappointing. Catheterizable channels to the bladder and ascending colon further facilitate continence measures and promote independent care. While surgical reconstruction is clearly successful in improving continence, recent outcome studies have questioned the true impact of this type of surgery on quality of life. With improved survival transitional care issues, including health related independence, sexual health needs and development of a support system, are increasingly important. Transitional care remains a significant issue for which few public health measures are being quantitatively evaluated.

CONCLUSIONS:

Despite consensus regarding early urological involvement in the care of patients with spina bifida, controversy remains regarding optimal management. Major reconstructive urological surgeries still have a major role in the management of these cases to protect the upper urinary tract and to achieve continence. However, future studies are needed to better clarify the true impact on quality of life that these interventions have on patients and their families. Transition of urological care to adulthood remains a major avenue for improvement in disease management.
Copyright © 2015 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.

KEYWORDS:

disease management; meningomyelocele; spinal dysraphism; urinary bladder

Improved Survival Among Children with Spina Bifida in the United States.

Shin M, Kucik JE, Siffel C, Lu C, Shaw GM, Canfield MA, Correa A. (2012) Improved Survival Among Children with Spina Bifida in the United States. Journal of Pediatrics. 2012 Jun 23. 

 

Division of Birth Defects and Developmental Disabilities, National Center on Birth Defects and, Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, GA; RTI International, Atlanta, GA.

Abstract

OBJECTIVE:

To evaluate trends in survival among children with spina bifida by race/ethnicity and possible prognostic factors in 10 regions of the United States.

STUDY DESIGN:

A retrospective cohort study was conducted of 5165 infants with spina bifida born during 1979-2003, identified by 10 birth defects registries in the United States. Survival probabilities and adjusted hazard ratios were estimated for race/ethnicity and other characteristics using the Cox proportional hazard model.

RESULTS:

During the study period, the 1-year survival probability among infants with spina bifida showed improvements for whites (from 88% to 96%), blacks (from 79% to 88%), and Hispanics (from 88% to 93%). The impact of race/ethnicity on survival varied by birth weight, which was the strongest predictor of survival through age 8. There was little racial/ethnic variation in survival among children born of very low birth weight. Among children born of low birth weight, the increased risk of mortality to Hispanics was approximately 4-6 times that of whites. The black-white disparity was greatest among children born of normal birth weight. Congenital heart defects did not affect the risk of mortality among very low birth weight children but increased the risk of mortality 4-fold among children born of normal birth weight.

CONCLUSIONS:

The survival of infants born with spina bifida has improved; however, improvements in survival varied by race/ethnicity, and blacks and Hispanics continued to have poorer survival than whites in the most recent birth cohort from 1998-2002. Further studies are warranted to elucidate possible reasons for the observed differences in survival.

PMID:22727874

 

Elective cesarean delivery and long-term motor function or ambulation status in infants with meningomyelocele.

Lewis D, Tolosa JE, Kaufmann M, Goodman M, Farrell C, Berghella V. (2004) Elective cesarean delivery and long-term motor function or ambulation status in infants with meningomyelocele. Obstetrics and Gynecology. Mar;103(3):469-73.


Division of Maternal Fetal Medicine, Department of Obstetrics and Gynecology, Jefferson Medical College, Thomas Jefferson University, Philadelphia, PA, USA. DAML2@aol.com

OBJECTIVE: To determine if elective cesarean delivery, when compared with trial of labor, is associated with better long-term motor function or ambulation status in infants with myelomeningocele.

METHODS: This is a retrospective cohort study of patients with myelomeningocele followed at the Spinal Dysfunction Program at Alfred I. duPont Hospital for Children in Wilmington, Delaware. Medical records were reviewed for gestational age at delivery, birthweight, anatomical level of lesion, and initial (0-6 months) and long-term (10 years or longer) motor function. Ambulation status (independent ambulation, ambulant with assistance, or wheelchair-bound) at 2 and 10 years was compared with those delivered by elective cesarean versus those delivered after trial of labor. RESULTS: Of the 106 patients with myelomeningocele that were identified, 87 (82%) had all the data required for this review. There were 44 patients in the elective cesarean group and 43 in the trial of labor group. There was no significant difference in gestational age at delivery or birthweight between the groups. There was statistical difference between the 2 groups when anatomical, initial, and current motor levels were compared. Compared with the elective cesarean group, patients in the trial of labor group were more likely to be ambulatory at 2 years (independently ambulant 7% versus 28%, ambulant with assistance 63% versus 65%, or wheelchair-bound 30% versus 7%, P =.003) and at 10 years (independently ambulant 5% versus 21%, ambulant with assistance 30% versus 54%, or wheelchair-bound 65% versus 25%, P <.001). However, when logistic regression analysis was used to control for motor level of myelomeningocele, no significant association was observed in ambulatory status at ages 2 and 10 years between infants delivered by elective cesarean or after trial of labor.

CONCLUSION: Elective cesarean delivery, when compared with delivery after trial of labor, was not associated with better motor function or ambulation status in myelomeningocele patients. LEVEL OF EVIDENCE: II-2

PMID: 14990408

Postnatal management and outcome for neural tube defects including spina bifida and encephalocoeles.

Thompson DN. (2009) Postnatal management and outcome for neural tube defects including spina bifida and encephalocoeles. Prenatal Diagnosis. Apr;29(4):412-9.

Department of Neurosurgery, Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, London WC1N 3JH, UK. thompd@gosh.nhs.uk

The incidence of neural tube defects (NTDs) has declined in recent decades, however myelomeningocele and encephalocele still represent one of the commoner prenatally diagnosed congenital malformations. Improved perinatal and post natal care mean that the mortality associated with these conditions has also fallen. Advances in the multidisciplinary management of children with myelomeningocele have led to significant improvements in functional outcome for many with this condition. However, there remains a substantial population of patients born with NTDs whose life expectancy is substantially reduced and who suffer significant cognitive and physical disability remaining wholly or partially dependant on the care of others into adult life. This article aims to outline the contemporary early management of these conditions and examine the prospects for functional outcome where possible, attempting to show how early anatomical features of these conditions can help predict where, along the wide spectrum of outcome, a given individual may lie.

PMID: 19194999

Fetal spina bifida repair--current trends and prospects of intrauterine neurosurgery

Fichter MA, Dornseifer U, Henke J, Schneider KT, Kovacs L, Biemer E, Bruner J, Adzick NS, Harrison MR, Papadopulos NA. Fetal spina bifida repair--current trends and prospects of intrauterine neurosurgery. Fetal Diagnosis Therapy. 2008;23(4):271-86.

Department of Plastic and Reconstructive Surgery, Technical University of Munich, Munich, Germany.

Myelomeningocele is a common dysraphic defect leading to severe impairment throughout the patient's lifetime. Although surgical closure of this anomaly is usually performed in the early postnatal period, an estimated 330 cases of intrauterine repair have been performed in a few specialized centers worldwide. It was hoped prenatal intervention would improve the prognosis of affected patients, and preliminary findings suggest a reduced incidence of shunt-dependent hydrocephalus, as well as an improvement in hindbrain herniation. However, the expectations for improved neurological outcome have not been fulfilled and not all patients benefit from fetal surgery in the same way. Therefore, a multicenter randomized controlled trial was initiated in the USA to compare intrauterine with conventional postnatal care, in order to establish the procedure-related benefits and risks. The primary study endpoints include the need for shunt at 1 year of age, and fetal and infant mortality. No data from the trial will be published before the final analysis has been completed in 2008, and until then, the number of centers offering intrauterine MMC repair in the USA is limited to 3 in order to prevent the uncontrolled proliferation of new centers offering this procedure. In future, refined, risk-reduced surgical techniques and new treatment options for preterm labor and preterm rupture of the membranes are likely to reduce associated maternal and fetal risks and improve outcome, but further research will be needed.

PMID: 18417993