Long-term survival of an infant with complete tetraploidy: A case report.

We present the case of a girl with complete tetraploidy who has survived to her present age of 4 years and 1 month. Infants with complete tetraploidy have been described to have a limited lifespan owing to complications. We report her characteristics, medical history, and development.

Revisiting MOMS criteria for prenatal repair of spina bifida: upper gestational-age limit should be raised and assessment of prenatal motor function rather than anatomical level improves prediction of postnatal function.

OBJECTIVES: To determine if the lower-extremity neurological motor function level in fetuses with open spina bifida deteriorates within the 4-week interval between a first prenatal motor assessment at around 22 weeks of gestation and a second evaluation, prior to 'late' prenatal surgery, defined as surgery at 26-28 weeks and, in certain situations, up to 30 weeks, and to assess the association between prenatal presurgical motor-function level, anatomical level of the lesion and postnatal motor-function level. METHODS: This was a two-center cohort study of 94 singleton fetuses with open spina bifida which underwent percutaneous repair using the skin-over-biocellulose for antenatal fetoscopic repair (SAFER) technique between December 2016 and January 2022. All women underwent two prenatal systematic ultrasound evaluations, approximately 4 weeks apart, with the second one being performed less than 1 week before surgery, and one postnatal evaluation via physical examination within 2 months of birth. Motor-function classification was from spinal level T12 to S1, according to key muscle function. Each leg was analyzed separately; in case of discrepancy between the two legs, the worst motor-function level was considered for analysis. Motor-function-level evaluations were compared with each other and with the anatomical level as observed on ultrasound. Independent predictors of a postnatal reduction in motor-function level were assessed using a logistic regression model. RESULTS: Prenatal motor-function level was assessed at a median gestational age of 22.5 (interquartile range (IQR), 20.7-24.3) and 26.7 (IQR, 25.4-27.3) weeks, with a median interval of 4.0 (IQR, 2.4-6.0) weeks. The median gestational age at surgery was 27.0 (IQR, 25.9-27.6) weeks and the postnatal examination was at median age of 0.8 (IQR, 0.3-5.4) months. There was no significant difference in motor-function level between the two prenatal evaluations (P = 0.861). We therefore decided to use the second prenatal evaluation for comparison with postnatal motor function and anatomical level. Overall, prenatal and postnatal motor function evaluations were significantly different from the anatomical level (preoperative assessment, P = 0.0015; postnatal assessment, P = 0.0333). Comparing prenatal with postnatal motor-function level, we found that 87.2% of babies had similar or improved motor function compared with that prior to prenatal surgery. On logistic regression analysis, lower anatomical level of defect and greater difference between anatomical level and prenatal motor-function level were identified as independent predictors of postnatal motor function (odds ratio, 0.237 (95% CI, 0.095-0.588) (P = 0.002) and 3.44 (95% CI, 1.738-6.813) (P < 0.001), respectively). CONCLUSIONS: During a 4-week interval between first ultrasound evaluation and late fetal surgical repair of open spina bifida, motor function does not change significantly, suggesting that late repair, ≥ 26 weeks, does not impact negatively on motor-function outcome. Compared with the anatomical level of the lesion, preoperative neurological motor-function assessment via ultrasound is more predictive of postnatal motor function, and should be included in preoperative counseling. © 2023 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Regression of Chiari malformation type 2 following early postnatal meningomyelocele repair-a retrospective observation from an institutional series of patients.

PURPOSE: Spontaneous regression of Chiari malformation type 2 (CM2) is observed rarely, as CM2 is associated with meningomyelocele (MMC) that is surgically repaired either pre- or early postnatally. While the radiological regression of CM2 occurs frequently following prenatal repair of MMC, it has been reported in only a few studies after postnatal repair. METHODS: From the consecutive series of children with postnatally repaired MMC, we reviewed the clinical and radiological data regarding CM2, particularly its regression either spontaneously or following CSF diversion. RESULTS: Eighteen children underwent postnatal repair of MMC between February 2011 and April 2023. CM2 was present in 16 (89%), and hydrocephalus in 15 children (83%), requiring shunting in 14 of them. During the mean clinical observation time (from birth to April 2023) of 59 ± 51 months, three children with CM2 (19%) underwent 1-2 foramen magnum decompressions (FMD), five children (28%) 1-4 surgical untethering procedures and 13 children with shunted hydrocephalus (93%) 1-5 shunt revisions. Out of sixteen children with CM2, we observed regression of CM2 on MRI in only one case (6%) during the mean radiological follow-up (from birth to the last MRI taken) of 49 ± 51 months. CONCLUSION: In our experience, spontaneous regression of CM2 in children with postnatally repaired MMC occurs quite rarely. Pathophysiological mechanisms behind the development of CM2 in children with MMC remain unclear, but our observation supports the hypothesis of an association between the downward displacement of the hindbrain and the low intraspinal pressure secondary to CSF leakage in children born with MMC.

Prenatal diagnosis of meningomyelocele resolves as a mature cystic teratoma in the thoracolumbar region.

A mature cystic teratoma is a mass with heterogeneous appearance, consisting of adult tissue with two or three layers: endoderm, mesoderm, and ectoderm. It is a rare, benign transformation of somatic tissue most commonly found in the sacrococcygeal region and may resemble an uncomplicated spina bifida on prenatal ultrasonography. In this case report, we describe a female newborn with an extremely rare mature cystic teratoma in the thoracolumbar region. She presented prenatally with a preliminary diagnosis of meningomyelocele, diastematomyelia, and Chiari II malformation and a possible teratoma. However, a mass containing solid glandular tissues and bony calcifications approximately 3 × 4 cm in size was observed in the thoracolumbar region upon birth. During surgical resection, no nerve roots were found in the associated meningocele. The patient retained full lower body function postoperatively following surgical excision of the thecal sac and teratoma.

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.

Growing rods in meningomyelocele lead to increased risk for complications in comparison with fusion; a retrospective study of 30 patients treated for at the University Hospital of Uppsala.

PURPOSE: To compare the complication rates of two different types of posterior instrumentation in patients with MMC, namely, definitive fusion and fusionless surgery (growing rods). METHODS: Single-center retrospective study of 30 MMC patients that underwent posterior instrumentation for deformity (scoliosis and/or kyphosis) treatment from 2008 until 2020. The patients were grouped based on whether they received definitive fusion or a growth-accommodating system, whether they had a complication that led to early surgery, osteotomy or non-osteotomy. Number of major operations, Cobb angle correction and perioperative blood loss were the outcomes. RESULTS: 18 patients received a growing system and 12 were fused at index surgery. The growing system group underwent a mean of 2.38 (± 1.03) surgeries versus 1.91 (± 2.27) in the fusion group, p = 0.01. If an early revision was necessitated due to a complication, then the number of major surgeries per patient was 3.37 (± 2.44) versus 1.77 (± 0.97) in the group that did not undergo an early revision, p = 0.01. Four patients developed a superficial and six a deep wound infection, while loosening/breakage occurred in 10 patients. The Cobb angle was improved from a mean of 69 to 22 degrees postoperatively. Osteotomy did not lead to an increase in perioperative blood loss or number of major operations. CONCLUSION: Growing systems had more major operations in comparison with fusion surgery and early revision surgery led to higher numbers of major operations per patient; these differences were statistically significant. Definitive fusion at index surgery might be the better option in some MMC patients with a high-risk profile.

Double Myelomeningocele Repair by Fetal Surgery with a Single Micro-Hysterotomy.

INTRODUCTION: Open spina bifida (OSB) is the most common congenital anomaly of the central nervous system. It is associated with severe neurodevelopmental delay, motor impairment, hydrocephalus, and bowel and bladder dysfunction. In selected cases, intrauterine spina bifida repair has been shown to improve neonatal outcomes. Rarely, the spine can have a double defect compromising two different segments and there is a lack of evidence on the feasibility and benefits of intrauterine repair in these cases. CASE PRESENTATION: We present a case with both cervicothoracic and lumbosacral myelomeningocele, Arnold-Chiari malformation type II and bilateral ventriculomegaly, that was treated successfully at 25 weeks with open micro-neurosurgery. Double myelomeningocele was successfully treated through a single 2-cm micro-hysterotomy, by performing external versions to sequentially expose and repair both defects. Weekly postoperative follow-up showed no progression of ventriculomegaly or complications attributable to the procedure. Preterm rupture of membranes prompted a conventional cesarean delivery at 32 weeks of gestation. Neurodevelopmental outcome at 20 months was within normal ranges, having achieved ambulation without orthopedic support and with no need for ventriculoperitoneal shunting. CONCLUSION: This report demonstrates for the first time the feasibility of double OSB repair through a single 2-cm micro-hysterotomy, suggesting that selected isolated cases of double myelomeningocele could be candidates for fetal intervention. Further prospective studies should be carried out to assess the potential benefit of double OSB intrauterine open repair.

Quality of life of families and siblings of children and adolescents with meningomyelocele.

BACKGROUND: Children with meningomyelocele may require continuous care. Consequently, there is a risk for caregiver burden and impact on family quality of life (QoL), including siblings' QoL. Some studies analysed caregivers' burden and family QoL separately. However, none of these studies evaluated siblings' QoL and the associations between these three dimensions. This study investigated the associations between caregivers' burden, family QoL and siblings' QoL in Brazilian families of children with meningomyelocele and its correlations with sociodemographic, functional and clinical variables. Siblings' QoL was specifically assessed using as a parameter the QoL of typically developed Brazilian children. METHODS: One hundred and fifty families, 150 caregivers and 68 siblings completed the Family Quality of Life Scale, Burden Interview, KIDSCREEN-27 Child and Adolescent Version and Parents Version questionnaires. RESULTS: Most families and caregivers reported a high family QoL and a low caregiver burden. Family QoL was significantly lower as caregivers' burden increased. Caregiver's burden was significantly lower with increasing family QoL levels. Self-reported siblings' QoL was significantly worse than that of typically developed peers. There were no significant differences between self and parent-reported siblings' QoL. Self-reported siblings' QoL was significantly worse as their age increased and better with increasing family QoL levels. Parent-reported siblings' QoL was significantly worse with increasing levels of caregiver's burden and significantly better as family QoL increased. There were no significant associations with functional and clinical variables. CONCLUSIONS: Despite the cross-sectional nature of the available data precludes any statements of causality, our results reinforce the relevance of knowing the factors that influence the QoL of families and siblings of children and adolescents with meningomyelocele and the relevance of actions aimed at reducing caregivers' burden, improving family QoL and meeting siblings' individual needs. Future multicenter studies may validate the generalizability of our findings.

Dorsal intercostal artery flap; A reliable option for coverage of myelomeningocele defects: A case series.

Meningomyelocele is a common congenital condition and its reconstruction poses a challenge for surgeons. The dorsal intercostal artery (DICA) flap offers a one-stage tension-free closure with adequate results. This study, spanning from January 2019 to September 2022, analyses the outcome of nine DICA flaps for meningomyelocele reconstruction, where the average size of the DICA flap was 6.8 x 4.6 cm for an average defect of 6.33 x 4 cm. Notably, no post-operative blood transfusion was required, nor any complications occurred except for one patient's septic shock-related death. Two had post-operative cerebrospinal fluid (CSF) leak, repaired primarily with one requiring VP shunt. Based on our experience, the DICA flap, with its consistent anatomy, is a reliable option for the reconstruction of meningomyelocele defects.

Cloacal Exstrophy with Normal Penis and Urethra - A Rare Variant.

Classically, cloacal exstrophy presents with omphalocele, bladder exstrophy, imperforate anus, and spinal defects. We report a rare variant of cloacal exstrophy in a 6-day-old male with an intact lower abdominal wall, normal penis, and urethra. Only two such cases have been reported in the literature.

Untreated meningomyelocele and squamous cell carcinoma: a case report and literature review.

BACKGROUND: Early closure of the meningocele, meningomyelocele, is essential to prevent complications such as hypothermia, infection in the postnatal period. But for some reasons, it is not always possible to repair early. The possibility of malign transformation should be considered in long-term due to nonhealing ulcers and cerebrospinal fluid (CSF) fistula and frequent infections in an adult patient with meningomyelocele. The possibility of conversion to malignancy in the control of an untreated meningomyelocele patient with (CSF) drainage should be especially kept in mind. CASE DESCRIPTION: A case is described of a malignant transformation at the site of a myelomeningocele in a 22-year-old woman. The lumbosacral region was deformed by a large meningomyelocele 10 cm in diameter, with areas of scar and ulcer. For 7 years prior to this presentation, the lesion was a large fistula in the roof of the open myelomeningocele. She underwent continuous CSF draining. A biopsy was done which revealed it as squamous cell carcinoma. CONCLUSION: Early closure of meningocele, meningomyelocele is essential to prevent complications such as hypothermia, infection in the postnatal period. Also, especially if there is continuous CSF exposure, the possibility of malignant change should be kept in mind in neglected cases presenting in an adult.

Dynamic and static ultrasound features predictive of vesicoureteral reflux and renal damage in children and adolescents with neurogenic bladder.

PURPOSE: This study aimed to analyze the diagnostic accuracy of dynamic and static ultrasound (DSUS) in detecting vesicoureteral reflux (VUR) and renal scarring in a cohort of children with neurogenic bladder (NB). MATERIALS AND METHODS: A retrospective, longitudinal, observational study was conducted using the Reporting Diagnostic Accuracy Studies guideline. The DSUS (index test) data were compared with voiding cystourethrography (VCUG) and renal scintigraphy 99mTc-dimercaptosuccinic (reference tests). Overall performance for predicting VUR and renal scarring was assessed using renal pelvic diameter (RPD)/distal ureteral diameter and renal parenchymal thinning on DSUS, respectively. RESULTS: A total of 107 patients (66 girls, median age 9.6 years) participated. Seventeen patients (15.9%) presented VUR, eight bilateral. For overall reflux grade, the AUC was 0.624 for RPD and 0.630 for distal ureteral diameter. The diagnostic performance for detecting high-grade VUR was slightly better for DSUS parameters. The AUC was 0.666 for RPD and 0.691 for distal ureteral diameter. The cut-offs of 5 mm for RPD and 6.5 mm for distal ureteral diameter presented the best diagnostic odds ratio (DOR) to identify high-grade VUR. The increase of RPD during detrusor contractions showed an accuracy of 89.2%. The thinness of renal parenchyma presented an accuracy of 88% for renal scarring. CONCLUSION: DSUS predicts VUR and renal scarring in children with NB with fair to good accuracy, and all measurements exhibited a high negative predictive value (NPV). The increase in RPD during voiding or detrusor contractions proved to be the most accurate parameter for indicating the presence of VUR in this study.

Fetus-in-Fetu: A Tale of the Trapped Triplets.

A 7-day-old male child presented with abdominal distention and jaundice. Radiological investigations revealed an encapsulated sac encasing three fetus-in-fetu (FIF) in the retroperitoneum. Laparotomy revealed a sac occupying almost the whole of the abdomen. The sac was stretching the duodenum and barely visible common bile duct, which were carefully separated. The rest of the bowel was displaced to the left. The sac containing three FIFs was excised intact. One of the fetuses was highly differentiated and had thoracic meningomyelocele, which has never been reported in FIF.

In utero myelomeningocele repair and high-risk bladder pattern. a prospective study.

OBJECTIVES: High-risk bladder pattern can be defined by Urodynamic Evaluation (UE) as overactive bladder with detrusor leak point pressure higher than 40 cmH2O and/or higher filling pressures also above 40 cmH2O. We wanted to evaluate response to treatment in myelomeningocele patients operated in utero in this subgroup. PATIENTS AND METHODS: From our prospective cohort of in utero MMC we have identified patients in the high-risk group. Treatment consisted of anticholinergics (Oxybutynin 0.2 mg/Kg) 2 or 3 times daily in association with CIC. At every UE, patients were reclassified in high-risk or low-risk patterns. Patients not responding were proposed bladder reconstruction or diversion according to age. RESULTS: Between 2011 to 2020, we have been following 121 patients and 60 (49.6%) of them were initially categorized as high-risk. The initial UE was performed at a mean age of 7.9 months and detrusor overactivity was found in 83.3% (mean maximum pressure of 76.5cmH20). When evaluating patients with 2 or more UE, we identified 44 patients (follow-up: 36.8months).  It was observed in the group of patients who underwent 2 to 5 UE, that response to treatment was validated by the finding of 40% of low-risk bladder patterns in the second UE and between 62% to 64% in the third to the fifth UE. The incidence of surgery was 13.3%. CONCLUSIONS: Early urological treatment of high-risk bladder pattern was effective in approximately 60%. We reinforce the need to correctly treat every patient with myelomeningocele, in accordance with UE, whether undergoing in utero or postnatal treatment.

Two-port, exteriorized uterus, fetoscopic meningomyelocele closure has fewer adverse neonatal outcomes than open hysterotomy closure.

BACKGROUND: In utero closure of meningomyelocele using an open hysterotomy approach is associated with preterm delivery and adverse neonatal outcomes. OBJECTIVE: This study compared the neonatal outcomes in in utero meningomyelocele closure using a 2-port, exteriorized uterus, fetoscopic approach vs the conventional open hysterotomy approach. STUDY DESIGN: This retrospective cohort study included all consecutive patients who underwent in utero meningomyelocele closure using open hysterotomy (n=44) or a 2-port, exteriorized uterus, fetoscopic approach (n=46) at a single institution between 2012 and 2020. The 2-port, exteriorized uterus, fetoscopic closure was composed of the following 3 layers: a bovine collagen patch, a myofascial layer, and a skin. The frequency of respiratory distress syndrome and a composite of other adverse neonatal outcomes, including retinopathy of prematurity, periventricular leukomalacia, and perinatal death, were compared between the study groups. Regression analyses were performed to determine any association between the fetoscopic closure and adverse neonatal outcomes, adjusted for several confounders, including gestational age of <37 weeks at delivery. RESULTS: The fetoscopic closure was associated with a lower rate of respiratory distress syndrome than the open hysterotomy closure (11.5% [5 of 45] vs 29.5% [13 of 44]; P=.037). The proportion of neonates with a composite of other adverse neonatal outcomes in the fetoscopic group was half of that observed patients in the open hysterotomy group; however, this difference did not reach statistical significance (4.3% [2 of 46] vs 9.1% [4 of 44]; P=.429). Here, regression analysis has demonstrated that fetoscopic meningomyelocele closure was associated with a lower risk of respiratory distress syndrome (adjusted odds ratio, 0.23; 95% confidence interval, 0.06-0.84; P=.026) than open hysterotomy closure. CONCLUSION: In utero meningomyelocele closure using a 2-port, exteriorized uterus, fetoscopic approach was associated with a lower risk of respiratory distress syndrome than the conventional open hysterotomy meningomyelocele closure.

Retrospective analysis of early- and late-operated meningomyelocele patients.

PURPOSE: Myelomeningocele (MMC) is one of the commonest congenital malformations. Hydrocephalus develops in 65-85% of cases with MMC. Only 3-10% of MMC patients have normal urinary continence. We aim to investigate the effects of early and late operation in MMC patients in terms of development of hydrocephalus, motor deficits, and bladder functions. METHODS: Medical records of MMC patients operated between January 2008 and December 2014 were reviewed retrospectively. RESULTS: We retrospectively investigated patients' records of 43 patients. Twenty of the patients were operated within the first 48 h after the delivery (early repair group), while 23 of the patients were operated after 48 h of delivery (late repair group). In the early repair group, 15 patients were operated due to hydrocephalus. Urodynamic problems were detected in 17 (85%) patients. In the late repair group, shunts were placed in 14 (61%) patients during follow-up period and urodynamic problems were detected in 19 (82.6%) patients. Mean operation time for the late group was 4.6 months. There was no statistical difference between the early and late group in terms of neurological and urodynamic deficits. The mean follow-up period was 45.5 months. CONCLUSION: In the literature, surgery in the first 48 h of life is recommended for MMC patients. There was no difference between the early- and late-operated groups by means of hydrocephalus, urodynamic functions, and motor deficits in our study. Late surgery of intact sacs may avoid complications related to surgery in the neonatal period.

Outcome in 32 cases of tethered cord in adults-is intervention justified?

INTRODUCTION: Adults rarely present with tethered cord syndrome, and this review examines whether it is justifiable to perform surgical intervention in this group. METHODS: Between 2003 and 2017, we performed surgical intervention in 32 adults with tethered cord syndrome. The age range varied from 22 to 65 years. Twenty-six had pain, 20 had motor deficits, and 18 had sensory deficits, whereas 17 presented with sphincter disturbances. Three had undergone surgery for meningomyelocele as infants. Six patients had major spinal deformity in the shape of kyphosis or scoliosis. All patients underwent digital radiographs and MR scans of the whole spine. The oldest with a leaking open meningocele was 41 years old. RESULTS: The mean follow-up was 3.2 years. Twenty-one out of 26 patients presenting with pain had their VAS scores improve significantly; 14 had motor deficits which improved. However, sensory deficits got better in only 7 out of 18 patients, and sphincter improvement was documented (via urodynamics studies and bladder ultrasound) in only 4 out of 17 patients. In 15 cases, surgery was performed under neurophysiological monitoring, and overall improvement was documented in 11 of these patients compared with 7 of the remaining 17 patients. Surgery for spinal deformity was performed in 6 patients, and deformity correction as well as pain reduction was achieved in all. CONCLUSION: Surgery leads to significant reduction of pain by untethering and in those with spinal deformity by correction of the same. Improvement in sensory changes and sphincter problems occurred in few patients. Neuromonitoring certainly has improved our results.

Fully developed accessory lower limb with tethered cord: Are these two anomalies related?

BACKGROUND: Spinal dysraphic anomalies may be hidden beneath grotesque cutaneous stigmata, like a fully developed accessory lower limb. CASE DESCRIPTION: Authors describe a 4-year-old boy who had socially unacceptable malformation in form of an accessory lower limb, in addition to perfectly normal both lower limbs with underlying low-lying tethered cord. Radiological studies showed underlying tethered cord and dysraphic spinal column. CONCLUSION: Successful surgical correction was undertaken along with detethering of cord. The present case asks for evaluation of all cutaneous stigmata over spinal regions in newborns for spinal dysraphic states.

Leiomyosarcoma in a cervical myelomeningocele: a rare complication in a neglected case.

BACKGROUND AND IMPORTANCE: Asymptomatic myelomeningoceles are usually more of cosmetic deformities and left without evaluation and untreated, amongst low socio-economic strata. Such midline swellings, if left neglected may become the seat for malignancies, usually of squamous cell origin. CLINICAL PRESENTATION: The authors report a case of an 18-year-old male patient who developed leiomyosarcoma within chronically neglected cervical myelomeningocele. CONCLUSION: A non-healing ulcer of any aetiology is a bed for carcinogenesis. Neglected myelomeningoceles, for any reason, may convert a benign entity to a malignant pathology. To our best knowledge, this is the first case of a leiomyosarcoma arising de novo from myelomeningocele.

Shunt timing in meningomyelocele and clinical results: analysis of 80 cases.

PURPOSE: Meningomyelocele is a serious pathology that requires immediate surgical treatment. Its management is difficult due to accompanying other pathologies and hydrocephalus. Shunt timing is still controversial. Therefore, this study retrospectively assessed 80 patients in order to improve the shunt timing and management of patients with meningomyelocele. METHODS: A total of 80 patients were followed up for 18-48 (average, 23) months. Patients were analyzed for the following variables: delivery method and time, head circumference monitoring, shunt timing, complication rates of patients who underwent shunting, during the early or follow-up period, accompanying pathologies, size, and localization of lesion. RESULTS: Patients including 46 males and 34 females have been operated. In 40% of patients, the accompanying pathology was determined. Approximately 85% of patients had hydrocephalus, and a ventriculoperitoneal shunt was placed on 36 symptomatic and 22 patients with hydrocephalus that developed during the follow-up. Differences in shunt-related and general complications were not significant between patients who underwent shunt placement during the same session and patients who underwent shunt placement during the follow-up. However, the incidence of cerebrospinal fluid fistula formation from the wound in patients who underwent shunt placement during the same session was significantly lower than those who underwent shunt placement during follow-up. CONCLUSIONS: Immediate surgery (within the first 48 h) provides positive results, which is consistent with the existing literature. According to the logistic regression analysis, the placement of the meningomyelocele sac in the lumbosacral region is decisive in shunt insertion. Placing the shunt in the same session for patients with hydrocephalus and later for patients who developed hydrocephalus during the follow-up is recommended as a favorable treatment.