OBJECTIVE: 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.
CONCLUSIONS: 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.

BACKGROUND: When a pregnant mother finds out she has a fetus with a congenital defect, the parents feel profound worry, anxiety, and melancholy. Anomalies can happen in singleton or twin pregnancies, though they are more common in twin pregnancies. In twins, several congenital defects are typically discordant. We present a rare case of concordant fatal anomaly in twin pregnancy in a 22-year-old African patient primigravida mother from Western Ethiopia who presented for routine antenatal care. An obstetric ultrasound scan showed anencephaly, meningomyelocele, and severe ventriculomegaly. After receiving the counseling, the patient was admitted to the ward, and the pregnancy was terminated with the medical option. Following a successful in-patient stay, she was given folic acid supplements and instructed to get preconception counseling before getting pregnant again.
CONCLUSIONS: The case demonstrates the importance of early obstetric ultrasound examination and detailed anatomic scanning, in twin pregnancies in particular. This case also calls for routine preconceptional care.

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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.

The limb body wall complex is a rare congenital disorder that is marked by various anomalies such as thoracoschisis, gastroschisis, limb defects and exencephaly. These defects are not compatible with life and result in spontaneous abortion or stillbirth. It is important to diagnose the anomalies on time and counsel the patients extensively to make the right decision. In this case report, we present a woman in her early twenties pregnant with a fetus with multiple anomalies including ectopia cordis, gastroschisis, meningomyelocele, kyphoscoliosis and congenital talipes equinovarus diagnosed as limb body wall complex at 18 weeks of gestation. Despite extensive counselling by the consultant, the couple decided to continue the pregnancy due to cultural beliefs which ultimately affected the maternal health. The fetus was spontaneously delivered at 28 weeks when the mother came to the hospital with ruptured membranes and the fetus was pronounced dead at birth. The co-occurrence of all these anomalies poses a challenge in diagnosis and management. Extensive counselling is required for the patient to make the appropriate decision for better outcomes. Cultural beliefs can play a major role in the decision-making of the patients and their families which can have a significant effect on maternal outcomes.

OBJECTIVE: Approximately 70%-80% of children born with myelomeningocele develop hydrocephalus and need CSF diversion, commonly a ventriculoperitoneal shunt (VPS) placement. The optimal timing of surgery is not known, but many centers delay VPS placement and perform it in a separate surgery to avoid shunt complications, mainly shunt contamination and infection. This systematic review and meta-analysis aimed to compare shunt-related complications between populations with VPS surgery performed either simultaneously with myelomeningocele closure or with delay.
METHODS: The authors searched MEDLINE (PubMed), Scopus, Web of Science, Cochrane Central Register of Controlled Trials, and Cochrane Database of Systematic Reviews databases on December 15, 2022, and November 11, 2023, using a predefined search strategy. Randomized and nonrandomized trials of neonates undergoing postnatal myelomeningocele closure and VPS placement before the age of 29 days were included. Cases with prenatal myelomeningocele closure and hydrocephalus treatment other than a VPS were excluded. All studies were screened and assessed by two independent reviewers. The authors performed a meta-analysis pooling risk ratios (RRs) with a 95% CI using the random-effects model. The quality of studies was assessed using the Newcastle-Ottawa Scale.
RESULTS: After screening and a full-text review of 2099 database search results, 12 studies with a total number of 4894 patients were included. All studies were nonrandomized studies. Only 2 studies were ranked as good quality on the Newcastle-Ottawa Scale. No statistically significant differences were found between simultaneous and delayed shunt insertion in terms of shunt infection (RR 0.77, 95% CI 0.41-1.42); shunt revision (RR 0.49, 95% CI 0.19-1.30); overall mortality (RR 0.87, 95% CI 0.09-8.57); wound CSF leak (RR 0.20, 95% CI 0.03-1.23); or myelomeningocele wound dehiscence (RR 0.52, 95% CI 0.07-3.71). In the subgroup analysis of studies conducted in high-income countries, simultaneous shunting was superior to delayed shunting concerning shunt infection (RR 0.49, 95% CI 0.31-0.78) and shunt revision (RR 0.30, 95% CI 0.09-0.95).
CONCLUSIONS: This systematic review and meta-analysis found no statistically significant differences in shunt-related complications between simultaneous and delayed VPS surgery in myelomeningocele-related hydrocephalus in neonates. The current literature does not support the common practice of delayed shunting or its alternative, simultaneous shunting.

To discern the efficacy of simultaneous versus delayed VPS surgery in managing hydrocephalus linked with MMC repair: The debate over the concurrent or deferred placement of ventriculoperitoneal shunts (VPS) during myelomeningocele (MMC) repair in hydrocephalic neonates necessitates a nuanced evaluation of associated risks and benefits. While VPS placement can mitigate cerebrospinal fluid (CSF) leaks and minimize wound dehiscence post-MMC repair, it concurrently introduces potential hazards such as infections and shunt-related malfunctions. This prospective cohort study focused on144 newborns with spinal myelomeningocele and hydrocephalus. Divided into two groups based on the timing of dysraphism repair and VPS placement, 101 children underwent concurrent procedures, while 43 received deferred VPS insertion post-MMC closure. Female patients constituted 60% of the cohort, with lumbar lesions being predominant. The median age for MMC closure was three days. Analysis revealed that the deferred insertion group exhibited higher rates of shunt malfunctions, CSF leaks, and wound dehiscence compared to the concurrent insertion group. Although indications hinted at a potential increase in shunt infections in the immediate insertion group, statistical significance was lacking. The study established a statistically significant association between the timing of shunt insertion during MMC repair and specific outcomes, such as CSF leaks and wound dehiscence. The findings suggest that concurrent shunt insertion during MMC repair may reduce the incidence of these complications compared to deferred insertion. However, no substantial differences emerged in terms of shunt infection and malfunction, emphasizing the persistent challenges associated with these major complications.

BACKGROUND: Individuals with myelomeningocele (MMC) present with neurological and orthopaedic deficiencies, requiring orthoses during walking. Orthoses for counteracting dorsiflexion may restrict activities such as rising from a chair.
OBJECTIVE: How are sit-to-stand (STS) movements performed with ankle joint-restricted ankle-foot orthoses (AFO) and knee-ankle-foot orthoses with a free-articulated knee joint (KAFO-F)?
METHODS: Twenty-eight adults with MMC, mean age 25.5 years (standard deviation: 3.5 years), were divided into an AnkleFree group (no orthosis or a foot orthosis) and an AnkleRestrict group (AFOs or KAFO-Fs). Study participants performed the five times STS test (5STS) while their movements were simultaneously captured with a three-dimensional motion system. Centre of mass (CoM) trajectories and joint kinematics were analysed using statistical parametric mapping.
RESULTS: The AnkleRestrict group performed the STS slower than the AnkleFree group, median 8.8 s (min, max: 6.9, 14.61 s) vs 15.0 s (min, max: 7.5, 32.2 s) (p = 0.002), displayed reduced ankle dorsiflexion (mean difference: 6°, p = 0.044) (74-81 % of the STS cycle), reduced knee extension (mean difference: 14°, p = 0.002) (17-41 % of the STS cycle), larger anterior pelvic tilt angle (average difference: 11°, p = 0.024) (12-24 % of the STS cycle), and larger trunk flexion angle (on average 4°, p = 0.029) (6-15 % of the STS cycle).
CONCLUSIONS: The differences between the AnkleFree and AnkleRestrict groups in performing the STS seem consistent with the participants functional ambulation: community ambulation in the AnkleFree group, and household and nonfunctional ambulation with less hip muscle strength in the majority of the AnkleRestrict group. No differences in the 5STS CoM trajectories or the kinematics were found with respect to the AFO and KAFO-Fs groups. Because orthoses are constructed to enable walking, the environment needs to be adjusted for activities in daily living such as the STS movement.

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.

After myelomeningocele (MMC) repair, a secondary tethered spinal cord occurs in almost all patients. The tethered spinal cord may result in progressive neurological deterioration and walking disability. This retrospective cohort study aimed to highlight the walking recovery one year after tethered cord release and its relation to the preoperative conus level. We reviewed the medical records at our university hospital from January 2014 to December 2022. The patients who underwent spinal cord untethering following lumbosacral MMC repair were included. We assessed the walking recovery one year after cord release using the modified Benzel scale. Thirty-seven patients met our selection criteria. There were 19 girls (51.4%) and 18 boys (48.6%). Their mean age at presentation was 8.6 years. The preoperative conus vertebral levels ranged between L4 and S3. One year after spinal cord release, 37.8% of the patients regained their walking ability. All the patients whose preoperative conus level was at S2 or S3 regained their walking ability. In contrast, all the patients with preoperative conus levels at L4 or L5 didn\'t regain their ability to walk. One-third (33.3%) of patients whose conus was at the S1 level regained their walking ability one year after cord release. One year after tethered cord release, 37.8% of the patients regained their walking ability. We found that the walking recovery was statistically associated with the preoperative conus level. A multicenter prospective study is required to support the results of this study.