Clinical presentation and outcomes of neonates born with neural tube defects- an experience from a level III B NICU in Western India.

PURPOSE: Neural tube defects (NTDs) are one of the most common congenital anomalies and a cause of chronic disability. The study was done to study outcomes of neural tube defects admitted at a tertiary level neonatal intensive care unit (NICU) from 2018 to 2022, a period of 4 years that also coincided with the COVID pandemic. The secondary outcome was to study the clinical presentation, associated anomalies and epidemiological features. METHODS: It was a retrospective observational study; data of infants was obtained from medical records and analysis was done. RESULTS: Thirty-four neonates were enrolled, of which there were 16 (47%) males and 18 (53%) females. History of pre-pregnancy maternal folate intake was present in 4 (11.7%) cases. 33 (97%) babies were diagnosed with meningomyelocele (MMC) and one each had anencephaly, iniencephaly and encephalocele, of which one had frontal and two had occipital encephalocele. The median age of surgery was 16 days of life with primary repair being the most common procedure followed by MMC repair with VP shunt. Twenty babies (58.8%) were discharged successfully, while 9 (26.5%) expired and 5 (14.7%) were discharged against medical advice; which can be attributed to the financial problems of the patients in a developing country. The overall deaths in our series were four (26.5%) which is slightly higher than other studies which may be due to the fact that this study was conducted during the COVID era with lesser rates of folate supplementation, reduced access to prenatal diagnosis coupled with poor follow-up and compliance of patients post-surgical repair. CONCLUSION: This study emphasizes the importance of periconceptional folic acid supplementation, prenatal diagnosis, early surgery and meticulous follow-up as being pivotal to improving outcomes in children with NTDs.

Fetal Surgery for Myelomeningocele: Neurosurgical Perspectives.

More than 30 years have elapsed since it was recognised that folic acid supplementation could substantially reduce the risk of open neural tube defects (ONTDs). During that time, many countries have adopted policies of food fortification with demonstrable reduction in the incidence of both cranial and spinal ONTDs. Improved prenatal detection and termination has also resulted in a reduction in the number of affected live births. Nonetheless, in the USA about 1500 children, and in the UK around 500 children are born each year with myelomeningocele (MMC) and so the management of MMC and its complications continues to constitute a significant clinical workload for many paediatric neurosurgical units around the world.Until recently, the options available following antenatal diagnosis of MMC were termination of pregnancy or postnatal repair. As a result of the MOMS trial, prenatal repair has become an additional option in selected cases (Adzick et al., N Engl J Med 364(11):993-1004, 2011). Fetal surgery for myelomeningocele is now offered in more than 30 centres worldwide. The aim of this chapter is to review the experimental basis of prenatal repair of MMC, to critically evaluate the neurosurgical implications of this intervention and to describe the technique of 'open' repair, comparing this with emerging minimally invasive alternatives.

What has changed in pediatric neurosurgical care in spina bifida? A 30-year UAB/Children's of Alabama observational overview.

Spina bifida (SB) remains the most serious and most common congenital anomaly of the human nervous system that is compatible with life. The open myelomeningocele on the back is perhaps the most obvious initial problem, but the collective impact of dysraphism upon the entirety of the nervous system and innervated organs is an equal or greater longitudinal threat. As such, patients with myelomeningocele (MMC) are best managed in a multi-disciplinary clinic that brings together experienced medical, nursing, and therapy teams that provide high standards of care while studying outcomes and sharing insights and experiences. Since its inception 30 years ago, the spina bifida program at UAB/Children's of Alabama has remained dedicated to providing exemplary multi-disciplinary care for affected children and their families. During this time, there has been great change in the care landscape, but many of the neurosurgical principles and primary issues have remained the same. In utero myelomeningocele closure (IUMC) has revolutionized initial care and has favorable impact on several important co-morbidities of SB including hydrocephalus, the Chiari II malformation, and the functional level of the neurologic deficit. Hydrocephalus however is not solved by IUMC, and hydrocephalus management remains at the center of neurosurgical care in SB. Ventricular shunts were long the cornerstone of treatment for hydrocephalus, but we came to assess and incorporate endoscopic third ventriculostomy with choroid plexus coagulation (ETV-CPC). Educated and nurtured by an experienced senior mentor, we dedicated ourselves to fundamental concepts but persistently evaluated our care outcomes and evolved our protocols and paradigms for improvement. Active conversations amidst networks of treasured colleagues were central to this development and growth. While hydrocephalus support and treatment of tethered spinal cord remained our principal neurosurgical charges, we evolved to embrace a holistic perspective and approach that is reflected and captured in the Lifetime Care Plan. Our team engaged actively in important workshops and guideline initiatives and was central to the development and support of the National Spina Bifida Patient Registry. We started and developed an adult SB clinic to support our patients who aged out of pediatric care. Lessons there taught us the importance of a model of transition that emphasized personal responsibility and awareness of health and the crucial role of dedicated support over time. Support for sleep, bowel health, and personal intimate cares are important contributors to overall health and care. This paper details our growth, learning, and evolution of care provision over the past 30 years.

Understanding factors influencing primary treatment with intradetrusor onabotulinumtoxinA versus augmentation cystoplasty in patients with spina bifida.

PURPOSE: Surgical interventions in the urologic management of children with neurogenic bladder secondary to spina bifida aim to preserve upper tract function, prevent urinary tract infections, and optimize quality of life. However, since the introduction of intravesical onabotulinumtoxinA (Botox) in the management of these patients, the indications for choosing Botox over augmentation cystoplasty (AC) remain undefined. The objective of this study was to determine which factors lead patients to undergo Botox versus AC as a primary surgical treatment after failing medical management. METHODS: We retrospectively reviewed the records of pediatric patients with myelomeningocele undergoing either primary Botox or primary AC at our institution between 2013 and 2018. We recorded demographic and clinical information. We identified 10 important clinical decision-making factors: bladder trabeculation, vesicoureteral reflux, or hydronephrosis on imaging; end-filling pressure (EFP) ≥40 cm H2O, detrusor overactivity, detrusor-sphincter dyssynergia, or reduced capacity on urodynamic studies; physician-perceived bladder hostility; and patient/family desire for continence and independence. The presence of these factors was compared between patients undergoing either primary Botox or primary AC. RESULTS: We identified 14 and 50 myelomeningocele patients who underwent primary AC and primary Botox, respectively. We found no significant differences in age, sex, race, or history of reconstructive surgery (antegrade continence enema or catheterizable channel). For the 10 decision-making factors, desire for independence/continence (p = <0.001) and reduced capacity (p = 0.002) were significantly associated with AC, while trabeculation (p = 0.006), EFP ≥40 cm H2O (p = 0.029), rising slope (p = 0.019), and physician-perceived hostility (p = 0.012) were significantly more common with Botox. CONCLUSIONS: At our institution, quality of life measures prompted AC over objective urodynamic or imaging findings before attempting Botox. These findings support a shared decision-making approach when considering surgical intervention for neurogenic bladder secondary to myelomeningocele.

Double Lumbar Localization of Myelomeningocele: Case Report.

INTRODUCTION: Myelomeningocele (MMC) is a malformation resulting from the neural tube's failure to close during embryonic development, and the majority of the cases of neural tube defects (NTDs) were prevalent as single location lesions along the spine; however, multiple NTDs (MNTDs) are a very rare condition. Only a few cases of MNTDs were found in the literature. CASE PRESENTATION: We report the case of a 2-month-old male infant prenatally diagnosed with MMC, presented with two unconnected lumbar and lumbosacral epidermal, soft, dome-shaped swellings located on both sides of the midline (paravertebral) covered by intact skin. MRI revealed double MMC at the level of L4-L5, with spinal nerve roots. The patient underwent surgical repair of the defects by replacing the spinal cord and its nerve roots inside the thecal sac and recreating a covering layer around the neural structures to resemble thecal sac. The outcome was favorable, and postoperative head CT scan did not show any complication. CONCLUSION: Our case report is considered the first from Algeria to report the condition and the first to report the occurrence of double lesions in the same spine region. MMC can be associated with neurological deficits or other congenital anomalies, thus it is necessary to thoroughly examine such patients. However, there was no antenatal folic acid deficiency in our case. We recommend antenatal care with adequate folic acid supplementation given that its deficiency during pregnancy is considered a ubiquitous risk factor for the condition. The optimal timing for surgery of MMC cases is 8 ± 5 days. Prenatal intrauterine repair of the condition provides favorable outcomes but carries high fetal and maternal risks. Surgical repair should include the sac removal, the reconstruction of the placode, and the closure of the overlying meninges. With early diagnosis and proper repair of such cases, MMC has good prognosis and favorable outcomes.

Postnatal Surgical Correction of Myelomeningoceles: Preoperative and Intraoperative Risk Factors Associated with Postoperative Neurologic Outcomes.

OBJECTIVE: Open spina bifida or myelomeningocele (MMC) is a congenital defect that results from failure of caudal neurulation. We present a case series of patients who were treated with postnatal surgical correction for MMC, evaluating the possible preoperative and intraoperative risk factors associated with neurologic outcomes. METHODS: A retrospective chart review of patients who underwent postnatal surgical correction for MMCs over 11 years at our institution was performed. MMCs were classified based on their morphologic configuration into 3 types. Type I includes defects without a sac and there is cerebrospinal fluid (CSF) leak. Type II includes where there is a sac ≤4 cm, with or without CSF leak. Type III includes defects with a sac that are greater than 4 cm. RESULTS: Fifty patients were included. The median age of gestation at surgery was 37.4 weeks. There were 30 females (60%). All mothers received adequate folate supplementation. All patients underwent surgical correction in the first 48 hours. Lower extremity motor function at the last clinical follow-up was normal in 34 patients (68%). CSF leak, infection, and mortality were 8%, 2%, and 0%, respectively. Twenty-one patients (42%) underwent ventriculoperitoneal shunt for hydrocephalus. CONCLUSIONS: Despite there being no statistically significant associations with a timely closure, all cases were treated within the first 48 hours and this could influence the low complication rate. Individuals of Hispanic background who received appropriate folate supplementation still had high rates of MMC and we posit that this may be caused in part by a genetic/molecular predisposition.

In utero myelomeningocele repair and incidence of lower urinary tract surgery. Results of a prospective study.

INTRODUCTION: The purpose of this study was to determine the incidence of lower urinary tract surgery in patients with myelomeningocele (MMC) operated in utero as well as in the subgroup of patients with a minimum age of 5 years. METHODS: We use a previously published protocol to categorize bladder patterns and propose the treatment: high risk pattern-anticholinergics and clean intermittent catheterization (CIC); incontinent and normal patterns-clinical surveillance and underactivity pattern- CIC. Non-response to clinical treatment with maintenance of detrusor pressure higher than 40 cmH20 with worsening of hydronephrosis or recurrent urinary infection were imperative indications for surgery and urinary incontinence due to sphincter deficiency and leakage pressure below 40 cmH20 in patients aged 5 years and older were possible indications too. We identified the prevalence of urinary tract surgery and concomitant treatment of constipation and refractory fecal incontinence by convenience, the technique performed and surgery outcomes. RESULTS: A total of 122 patients are part of the prospective protocol. The first UE was performed in 119 patients (median age of 4 months). The categorization of the bladder pattern was high risk in 52.1%, incontinent in 25.2%, hypocontractile 4.2% and normal in 18.5%. Current follow-up was 29.9 months (1-99 months). Surgery was performed on 10 patients (8.4%): 3 vesicostomies, 2 surgeries to treat vesicoureteral reflux, 6 bladder augmentations, 3 ACE and a sling surgery. Considering only patients older than 5 years, we identified 65 patients and seven surgeries performed (10.8%). DISCUSSION: Most bladder reconstruction studies are case series of single institutions or cooperative services, most of which are retrospective. The prospective clinical interpretation and imaging and urodynamic exams over time allowed us prospectively to define the risk of surgery in patients with myelomeningocele operated in utero. It must be considered that the response to the initial urological treatment and even the failure that leads to the need for surgery have to be evaluated in a context of follow-up time. For this reason we have studied separately patients over 5 years-old and also the group considered to be at greatest risk for surgery, the high-risk group of the categorization already described. CONCLUSION: We found an incidence of 10.8% of surgeries in patients over 5 years-old and 12.9% for the high-risk group. This information may be useful to educate parents when stating urological treatment.

Understanding the Mothers of Children with Spina Bifida and Hydrocephalus in Tanzania.

OBJECTIVE: To identify if there are cultural, medical, educational, economic, nutritional and geographic barriers to the prevention and treatment of spina bifida and hydrocephalus. METHODS: The mothers of infants with spina bifida and hydrocephalus admitted to Muhimbilli Orthopaedic Institute, Dar Es Salaam, Tanzania, between 2013 and 2014 were asked to complete a questionnaire. A total of 299 infants were identified: 65 with myelomeningoceles, 19 with encephaloceles, and 215 with isolated hydrocephalus. The questionnaire was completed by 294 of the mothers. RESULTS: There was a high variation in the geographic origin of the mothers. Approximately 85% traveled from outside of Dar Es Salaam. The mean age was 29 (15-45) years old with a parity of 3 (1-10). The rates of consanguinity, obesity, antiepileptic medication, HIV seropositivity, and family history were 2%, 13%, 0%, 2%, and 2%, respectively. A maize-based diet was found in 84%, and only 3% of woman took folic acid supplementation, despite 61% of mothers stating that they wished to conceive another baby. Unemployment was high (77%), a low level of education was common (76% not attended any school or obtaining a primary level only), and 20% were single mothers. Hospital only was the preferred method of treatment for 94% of the mothers, and 85% of the babies were born in a hospital. CONCLUSIONS: Our study highlights some of the cultural, educational, geographic, nutritional, and economic difficulties in the prevention and management of spina bifida and hydrocephalus in Tanzania.

Does Kyphectomy Improve the Quality of Life of Patients With Myelomeningocele?

BACKGROUND: Lumbar kyphosis is a complex spinal deformity occurring in approximately 8% to 20% of patients with myelomeningocele. The resulting gibbosity may cause pressure ulcers, difficulty lying down in the supine position and sitting on the ischia without support, decreasing quality of life (QOL). Surgery is generally performed to correct kyphosis and maintain vertebral alignment, but high complication rates have been reported. Despite satisfactory radiological results, the impact of surgery and its complications on health-related QOL (HRQOL) has not yet been established. QUESTIONS/PURPOSES: Among children with myelomeningocele undergoing corrective surgery for lumbar kyphosis: (1) What is the risk of complications and reoperation after this procedure? (2) Does this procedure improve HRQOL scores in these patients? METHODS: Between 2012 and 2013, five surgeons at three centers treated 32 patients for myelomeningocele-related kyphosis with kyphectomy and posterior instrumentation. During that period, all surgeons used the same indications for the procedure, which were progressive postural decompensation and chronic ulceration at the apex of the deformity. Data were prospectively collected, and all patients who underwent surgery were considered in this retrospective study. The legal guardians of one patient declined to sign the informed consent form, resulting in 31 patients included. A total of 9.7% (3 of 31) were lost to follow-up before the 2-year period, and the remaining 90.3% (28 of 31) were seen at a mean of 3 years (± 9 months) after surgery. The average age was 10 years, 7 months (± 21 months) at the time of surgery. The patients had a mean kyphosis angle of 130° ± 36° before surgery. This technique involved posterior fixation using S-shaped rods inserted through the foramina of S1 and pedicle screws inserted in the thoracic spine. The patients' caregivers answered both the generic and specific (neuromuscular module) Pediatric Quality of Life Inventory questionnaires preoperatively and 2 years postoperatively. The minimum clinically important difference (MCID) considered for the instruments used was 5. RESULTS: Reoperation was performed in 68% of patients (19 of 28), mostly to treat deep infection. In all, 18% of patients (five of 28) underwent implant removal to control infection. Eleven percent (three of 28) had a loss of reduction and pseudarthrosis. The HRQOL increased from 71 ± 11 preoperatively to 76 ± 10 postoperatively (p < 0.001), resulting in a 5-point increase (95% CI 3 to 7) in the generic questionnaire score and from 71 ± 13 to 79 ± 11 (p < 0.001), resulting in an 8-point increase (95% CI 5 to 10) in the neuromuscular Paediatric Quality of Life Inventory questionnaire score, mainly in the physical health domain on both questionnaires. CONCLUSIONS: Kyphectomy was associated with a high risk of complications and reoperations and did not seem to deliver a substantial clinical benefit for patients who underwent the procedure. Most of our HRQOL score improvements were below the minimum clinically important difference for the Pediatric Quality of Life Inventory questionnaires. Although it seems that surgeons lack a better surgical alternative when facing the challenging health impairments these patients suffer, efforts should be made to improve the technique and reduce surgical complications. Additionally, patients and caregivers should be advised of the high reoperation rate and notified that the procedure may not result in a better QOL and should thus be avoided when possible. Future studies should verify whether decreasing the complication rate could imply improvement in the HRQOL of these patients after surgery. LEVEL OF EVIDENCE: Level IV, therapeutic study.

Multiple-site neural tube defects: embryogenesis with complete review of existing literature.

OBJECTIVE: Multiple-site neural tube defects (MNTDs) are very uncommon, with the predominant number of cases being reported in developing countries. The classic theory of neural tube closure fails to explain the occurrence of these defects. Multisite closure theory, first proposed in 1995, explains most of the occurrences with a few modifications specific to a few defects. In this paper, the authors endeavor to explain all the defects, along with their genetic and embryological bases, and to review the available literature and discuss their own experience in the management of these complex cases. METHODS: The authors retrospectively reviewed the data of all the patients treated surgically for MNTDs over that past 14 years. All possible demographic data, clinical details, and radiological imaging data were reviewed. In addition, surgical parameters, complications, and status at follow-up of more than 12 months were evaluated. All previously reported cases of MNTD were analyzed, and comparisons with the present series were made. RESULTS: A total of 3 major series (including the present one) on MNTDs have been from India. A total of 57 such cases (including those of the present series) have been reported in the available literature. While previous series reported a higher incidence of spinal defects, the present series had a higher rate of cephalic defects (55%). Among the reported cases, insertion of a ventriculoperitoneal shunt was necessary in 12 (26%), and only 4 patients were operated on in 2 stages. Neurological status at presentation dictated outcome. CONCLUSIONS: MNTDs are extremely rare, and their embryogenesis is different from that of single neural tube defects. Simultaneous repair of 2 or even 3 defects is possible in a single-stage surgery. The requirement of a shunt is uncommon, and complications following surgery are rare. Folic acid supplementation may reduce the incidence of defects.

The Use of Indomethacin with Complete Amniotic Fluid Replacement and Classic Hysterotomy for the Reduction of Perinatal Complications of Intrauterine Myelomeningocele Repair.

OBJECTIVE: This study presented outcomes of classical hysterotomy with modified antiprostaglandin therapy for intrauterine repair of foetal myelomeningocele (fMMC) performed in a single perinatal centre. STUDY DESIGN: Forty-nine pregnant women diagnosed with fMMC underwent classic hysterotomy with anti-prostaglandin management, complete amniotic fluid replacement and high dose indomethacin application. RESULTS: The average gestational age (GA) at delivery was 34.4 ± 3.4 weeks, with no births before 30 weeks GA. There were 2 foetal deaths. Complete reversal of hindbrain herniation (HH), assessed in magnetic resonance imaging at 30-31 weeks GA was found in 72% of foetuses (mostly with HH grade I prior to fMMC repair). Our protocol resulted in rare use of magnesium sulphate (6%), low incidence of chorioamniotic membrane separation - chorioamniotic membrane separation (6%), preterm premature rupture of membranes - preterm premature rupture of membranes (pPROM; 15%) and preterm labour - preterm labour (PTL; 17%). The postoperative wound continuity of the uterus was usually stable (in 72% of patients), with low frequency of scar thinning (23%). CONCLUSION: Our protocol results in rare use of tocolytics, and the low occurrences of CMS, pPROM and PTL in relation to other study cohorts: Management of Myelomeningocele Study, Children's Hospital of Philadelphia, and Vanderbilt University Medical Centre.

A Case Report of a 4-Year-Old Boy with Intradural Spinal Cord Abscess Successfully Treated with Adjuvant Hyperbaric Oxygen Therapy.

Intradural spinal cord abscesses are rare infections in early childhood and usually result from pre-existing congenital anomalies of the spinal column. The formation of abscess may be the result of hematogenous spread. It is treated by surgical and parenteral antibiotic treatment, but some special cases may require additional treatments. This article presents a 4-year-old male patient who was previously operated on for spina bifida (meningocele and tethered cord syndrome) at another hospital. Upon complaints of not being able to walk after one month, he was operated on with the diagnosis of spinal intradural abscess and referred to our clinic to continue his treatment. The patient underwent an emergency operation when his new spinal magnetic resonance imaging (MRI) at our hospital showed progression of intradural abscess. Due to no regression of neurological deficits in the follow-up and the risk of another operation, antimicrobial treatment as well as hyperbaric oxygen therapy (HBOT) was planned. At the end of 20 HBOT treatment sessions, the patient started to walk with support and the antibiotic treatment was completed in six weeks. In cases where surgical and antimicrobial treatments have failed, HBOT should be considered as an additional treatment method in children with spinal abscess.

Surgical Management of Myelomeningocele-Related Spinal Deformities.

OBJECTIVE: To evaluate the optimal timing and type of surgical treatment of myelomeningocele (MMC)-related spinal deformities and long-term follow-up of surgical treatment. METHODS: We reviewed and presented clinical pictures, treatment strategies and results of 20 patients with MMC-related spinal deformities treated at our center between 2010 and 2017. RESULTS: The average patient age was 6.3 years. The average preoperative neurologic status according to a modified Japan Orthopedic Association (mJOA) scale was 7.3 points (Benzel's modification). Average functional status was 41 points according to a functional independent measure scale (FIM). The average angle of kyphosis was 83.7°, that of scoliosis was 36.7°, and that of lordosis was 67° (Cobb angles). The average duration of surgery was 234 minutes, and the average total blood loss was 175 mL. The average angle of kyphosis correction was 61°, that of scoliosis correction was 25°, and that of lordosis correction was 25° (Cobb angles). The average duration of hospitalization was 16.6 days, and the average follow-up was 34.5 months. The total number of complications was 13. Reoperation was required in 9 cases. Neurologic status according to the mJOA scale improved by 0.6 point on average. Functional status according to the FIM increased by 6.6 points on average. CONCLUSIONS: Early surgical correction of MMC-related spinal deformities improves body balance and quality of life. The dual growing rod technique is safe and effective in cases of moderate neuromuscular spinal deformities at an early age. Kyphectomy is a challenging procedure with high complication rates, especially skin problems, but there are no alternative procedures for cases of heavy rigid kyphosis.

Left-colon antegrade enema (LACE): Long-term experience with the Macedo-Malone approach.

AIMS: We evaluated the long-term results with a left antegrade continence enema (LACE) approach: "Macedo-Malone (MM) procedure" to define parameters such as clinical durability of the technique and patients' compliance with the method. METHODS: We reviewed the medical records of all patients that underwent the MM procedure in our institution since 2001 and conducted a telephonic interview to investigate the use of the enema and satisfaction with the procedure. RESULTS: Thirty-five MM procedures were performed, but eight patients lost to follow-up were excluded. Fifteen patients (55%) were female. Myelomeningocele was the clinical diagnoses in 25 (93%). Mean age at surgery was 9 years (3-27 years) and mean follow-up was 75 months (median: 56 months). The indication for LACE was clinically intractable constipation with fecal leakage. Most patients still used the stoma regularly to do the enema (74%). Mean washout time was 27 min (2-90 min). The revision rate due to stenosis was 22.2% (6/27) and all underwent suprafascial revision. We performed one classical MACE by infrafascial approach as a salvage procedure. Overall reoperation rate was 25.8% and fecal continence was 89%. There were no reports of leakage through the stoma. Among patients who still used the stoma, 74% were satisfied with surgery and would strongly recommend the procedure to another patient. CONCLUSIONS: The MM procedure is a straightforward procedure, which can be performed in 15-20 min, avoids additional entero-entero anastomosis, precludes the use of the appendix, and has shown comparable results to either "classical" MACE or other LACE variants. Neurourol. Urodynam. 36:111-115, 2017. © 2015 Wiley Periodicals, Inc.

A novel colonoscopic approach for the management of a Malone antegrade continence enema channel, which cannot be catheterized in the immediate postoperative period: a case report.

Early Malone antegrade continence enema (MACE) complications are rare, but can be devastating, particularly if they involve loss of the channel. Management of these complications is not well described. We report on a patient who had her MACE channel successfully salvaged in the immediate postoperative period using a colonoscopic retrograde wire and catheter placement after failing antegrade percutaneous endoscopic management. To our knowledge, this is the first report of a novel, colonoscopic, minimally invasive technique of managing select MACE channels, which cannot be otherwise recatheterized. We also review the management of postoperative MACE complications.

Ten years' minimum follow-up with the ileal continent catheterizable reservoir: a test of time.

OBJECTIVE: To evaluate long-term efficacy of an original technique of catheterizable ileal reservoir designed for bladder augmentation and/or substitution, precluding the need to use a Yang-Monti channel or appendix. MATERIALS AND METHODS: Our series comprised 19 patients, operated in 1998-2000, with a mean age of 10.1 years (1.6-30). Two were excluded from analysis because lost to follow-up after 1.5 and 7 years of surveillance. The primary disease was posterior urethral valves (9), myelomeningocele (4), anorectal malformation (1), rhabdomyosarcoma (1), medullary astrocytoma (1) and urethral stricture (1). Surgery consisted of creating a continent catheterizable ileum-based reservoir from a 35-cm ileum segment. RESULTS: Mean follow-up was 11.2 years (10-12.4). All 16 patients eligible for final evaluation of the procedure were continent, excluding two lost of follow-up and one that underwent undiversion. Complications noted were: stomal stenosis (3), leakage associated with false passage (1) and false passage (1). One patient underwent a Bricker undiversion procedure, an appendicovesicostomy was performed in 2, and 1 patient received a Monti channel to replace the outlet conduit. The overall complication rate was 29.4% (5/17). CONCLUSION: We have confirmed the long-term efficacy of the technique. The results are comparable to other 'gold standard' treatments, with the advantage of being simpler, faster and sparing the appendix for other uses (Malone antegrade continent enema), as well as precluding the need to create a Monti channel.

Novel treatment immediately after myelomeningocele repair applying low-level laser therapy in newborns: a pilot study.

BACKGROUND/AIMS: The use of low-level laser therapy (LLLT) in neurosurgery is still hardly disseminated and there are situations in which the effects of this therapeutic tool would be extremely relevant in this medical field. The aim of the present study is to analyze the effect of LLLT on tissue repair after the corrective surgical incision in neonates with myelomeningocele, in an attempt to diminish the incidence of postoperative dehiscences following surgical repair performed immediately after birth. MATERIALS AND METHODS: Prospective pilot study with 13 patients submitted to surgery at birth who received adjuvant treatment with LLLT (group A). A diode laser CW, λ = 685 nm, p = 21 mW, was applied punctually along the surgical incision, with 0.19 J delivered per point, accounting for a total of 4-10 J delivered energy per patient, according to the surgical wound area and then compared with the results obtained in 23 patients who underwent surgery without laser therapy (group B). RESULTS: This pilot study disclosed a significant decline in dehiscences of the surgical wounds in neonates who were submitted to LLLT (7.69 vs. 17.39%). CONCLUSION: This new adjuvant therapeutic modality with LLLT aided the healing of surgical wounds, preventing morbidities, as well as shortening the period of hospital stay, which implies a reduction of costs for patients and for the institution.

Pattern and factors affecting management outcome of spina bifida cystica in Ile-Ife, Nigeria.

BACKGROUND AND OBJECTIVE: There is paucity of data on the pattern and factors affecting the management outcome of patients with spina bifida cystica in the Ife-Ijesa zone, Nigeria. This study was designed to address this research question. METHOD: One hundred and six consecutive cases of spina bifida cystica who presented in our hospital from January 1990 to December 2004 were reviewed. We obtained information on sociodemographic factors, medical history and management as well as clinical outcome. SPSS was used to analyze the data. RESULT: Males constituted 54.7% and females 45.2% of cases. Mortality was high in those presented after the 4th week of life (p = 0.04). The malformation occurred in the lumbar and lumbosacral regions in 77.4%. Myelomeningocele was the most common type (86.8%). Hydrocephalus was recorded in 53.8% of patients. Surgical closure was done for 91.5% of the patients. About 77% of all the patients were discharged while 22.7% died. This was significantly related to age at presentation (p = 0.04) and infection before surgery (p = 0.045). Postoperative complications were more frequent in patients with ruptured lesions (p = 0.025), a larger size of defect (p = 0.028) and a lower birth weight (p = 0.006). CONCLUSION: Myelomeningocele is the most common type of spina bifida cystica in our environment. Late presentation and preoperative infection are associated with high mortality in our patients.

Cecal volvulus: a report of two cases occurring after the antegrade colonic enema procedure.

BACKGROUND: Many children with chronic constipation and fecal incontinence have benefited from the antegrade colonic enema (ACE) procedure. Routine antegrade colonic lavage often allows such children to avoid daytime soiling. This report describes 2 children in whom the ACE procedure was complicated by a cecal volvulus. METHODS: A retrospective review of 164 children with an ACE procedure was conducted. Two instances of cecal volvulus were identified. RESULTS: The first child presented with abdominal pain and difficulty intubating the ACE site. Over the subsequent day, his pain worsened, and radiographs depicted a colonic obstruction. At laparotomy, a cecal volvulus resulting in bowel necrosis was observed, and resection of the affected bowel and appendix (in the right lower quadrant) and end ileostomy was required. He subsequently had the stoma closed and a new ACE constructed with a colon flap. The second child presented with shock and evidence of an acute abdomen. At laparotomy, a cecal volvulus was noted, and ileocolic resection including the ACE stoma (located at the umbilicus) and an ileostomy and Hartmann pouch was performed. He had a protracted hospital course requiring ventilator and inotropic support. He currently is well and still has an ileostomy stoma. CONCLUSIONS: A high index of suspicion for a potentially life-threatening cecal volvulus should be maintained in children undergoing an ACE procedure who present with abdominal pain, evidence of bowel obstruction, or difficulty in advancing the ACE irrigation catheter.