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Annals of Plastic Surgery Jul 2022Myelomeningocele is the most common form of neural tube anomalies. Early reliable skin coverage should be achieved to reduce central nervous system infections. The...
INTRODUCTION
Myelomeningocele is the most common form of neural tube anomalies. Early reliable skin coverage should be achieved to reduce central nervous system infections. The keystone flaps are gaining popularity for myelomeningocele defect reconstruction. However, the use of a traditional keystone flap is limited in very wide or transversely oriented myleomeningocele defects with inadequate lateral tissue laxity. In this study, we present our successful experience using modified rotation advancement keystone flaps for closure of the transversely oriented myelomeningocele defects.
PATIENTS AND METHODS
Between April 2019 and April 2020, the modified rotation advancement keystone flap was used for reconstruction of transversely oriented myelomeningocele defect in 7 patients (5 males and 2 females) with average age of 14 days. The localization of the lesions was lumbosacral in 5 patients and thoracolumbar in 2 patient. The average myelomeningocele defect width was 6.4 cm, whereas the average defect length was 5.7 cm. The following information was evaluated: the flap dimensions, operative time, and postoperative complications.
RESULTS
All patients had uneventful operations, except for 1 case of superficial epidermolysis over flap tip, which settled with conservative wound management. Immediate venous congestion was detected in 2 patients that completely resolved. Otherwise, all wounds healed without any evidence of complications. The mean follow-up was 4 months.
CONCLUSIONS
Although the conventional keystone flap seems to have geometric constraints to close a transversally oriented myelomeningocele defect, the modified rotation advancement keystone flap serves as a superior alternative and effective option for closure transversally oriented defect.
Topics: Adolescent; Female; Humans; Male; Meningomyelocele; Operative Time; Plastic Surgery Procedures; Surgical Flaps; Treatment Outcome
PubMed: 35502970
DOI: 10.1097/SAP.0000000000003150 -
The Pan African Medical Journal 2022
Topics: Humans; Meningomyelocele
PubMed: 36879634
DOI: 10.11604/pamj.2022.43.171.38082 -
Pediatric Cardiology Jun 2021Myelomeningocele (MMC) and congenital heart disease (CHD) are independent risk factors for increased morbidity and mortality in the newborn period and each can require...
Myelomeningocele (MMC) and congenital heart disease (CHD) are independent risk factors for increased morbidity and mortality in the newborn period and each can require significant operations shortly after birth. Few studies have examined the impact of these combined lesions. We sought to examine the incidence of CHD in patients with MMC, and to evaluate length of stay (LOS), hospital charges, and mortality. Using the Texas Inpatient Public Use Data File, ~ 6.9 million newborn records between 1/1999 and 12/2016 were examined. Hospitalizations were classified as MMC without CHD (n = 3054), CHD without MMC (n = 72,266), and MMC with CHD (n = 171). The birth prevalence of CHD with MMC was 0.3/10,000 live hospital births, with 5% of patients with MMC having CHD, and 0.2% of those with CHD having MMC. There was increased LOS in patients with both MMC and CHD (median 15 days, IQR 5-31), compared to CHD without MMC (median 6 days, IQR 2-20) and MMC without CHD (median 8 days, IQR 1-14) and higher total hospital charges (median $95,007, IQR $26,731-$222,660) compared to CHD without MMC (median $27,726, $6463-$118,370) and MMC without CHD (median $40,066, IQR $5744-$97,490). Mortality was significantly higher in patients with MMC and CHD (22.2% compared to 3.1% in MMC without CHD and 4.1% in CHD without MMC). Significance remained when limiting for patients without genetic conditions or additional major birth defects. MMC with CHD in the newborn compared to either CHD or MMC alone is associated with longer LOS, higher charges, and increased mortality.
Topics: Databases, Factual; Female; Heart Defects, Congenital; Hospital Charges; Humans; Infant, Newborn; Length of Stay; Male; Meningomyelocele; Prevalence; Texas
PubMed: 33688980
DOI: 10.1007/s00246-021-02576-3 -
AJNR. American Journal of Neuroradiology Jul 2023Short-term results demonstrate that prenatal repair of a myelomeningocele is associated with a reduction in hydrocephalus and an increased likelihood of the reversal of...
BACKGROUND AND PURPOSE
Short-term results demonstrate that prenatal repair of a myelomeningocele is associated with a reduction in hydrocephalus and an increased likelihood of the reversal of Chiari II malformations compared with postnatal repair. The purpose of this study was to identify the long-term imaging findings at school age among subjects who underwent pre- versus postnatal repair of a myelomeningocele.
MATERIALS AND METHODS
A subset of subjects enrolled in the Management of Myelomeningocele Study who underwent either prenatal ( = 66) or postnatal ( = 63) repair of a lumbosacral myelomeningocele and had follow-up brain MR imaging at school age were included. The prevalence of posterior fossa features of Chiari II malformation and supratentorial abnormalities and the change in these findings from fetal to school-age MR imaging were compared between the 2 groups.
RESULTS
Prenatal repair of a myelomeningocele was associated with higher rates of normal location of fourth ventricle and lower rates of hindbrain herniation, cerebellar herniation, tectal beaking, brainstem distortion, and kinking at school age compared with postnatal repair (all < .01). Supratentorial abnormalities, including corpus callosal abnormalities, gyral abnormalities, heterotopia, and hemorrhage, were not significantly different between the 2 groups (all > .05). The rates of resolution of brainstem kinking, tectal beaking, cerebellar and hindbrain herniation, and normalization of fourth ventricle size from fetal to school age MR imaging were higher among the prenatal compared with postnatal surgery group (all, .02).
CONCLUSIONS
Prenatal repair of a myelomeningocele is associated with persistent improvement in posterior fossa imaging findings of Chiari II malformation at school age compared with postnatal repair.
Topics: Pregnancy; Female; Humans; Meningomyelocele; Follow-Up Studies; Hydrocephalus; Brain; Arnold-Chiari Malformation
PubMed: 37385677
DOI: 10.3174/ajnr.A7926 -
Ultrasound in Obstetrics & Gynecology :... Feb 2023
Topics: Pregnancy; Female; Humans; Meningomyelocele; Fetus; Fetoscopy; Prenatal Care
PubMed: 36178849
DOI: 10.1002/uog.26081 -
Neurosurgical Review Jun 2024After myelomeningocele (MMC) repair, a secondary tethered spinal cord occurs in almost all patients. The tethered spinal cord may result in progressive neurological...
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.
Topics: Humans; Male; Female; Walking; Child; Recovery of Function; Retrospective Studies; Neural Tube Defects; Child, Preschool; Adolescent; Meningomyelocele; Treatment Outcome; Neurosurgical Procedures
PubMed: 38914693
DOI: 10.1007/s10143-024-02497-8 -
Pediatric Neurosurgery 2023Myelomeningocele (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...
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.
Topics: Infant; Pregnancy; Female; Humans; Male; Meningomyelocele; Neural Tube Defects; Spine; Spinal Cord; Fetus
PubMed: 37075706
DOI: 10.1159/000530726 -
The Journal of Surgical Research Feb 2022Prenatal surgical closure of Myelomeningocele (MMC) is considered part of the current age armamentarium. Clinical data has demonstrated the need for innovative patches...
BACKGROUND
Prenatal surgical closure of Myelomeningocele (MMC) is considered part of the current age armamentarium. Clinical data has demonstrated the need for innovative patches to maximize the benefits and decrease the risks of this approach. Our team has developed a minimally invasive reverse thermal gel (RTG) patch with cellular scaffolding properties. Here, we demonstrate the initial gross and microscopic histological effects of this RTG patch in the fetal ovine model of MMC.
MATERIALS AND METHODS
A fetal ovine MMC defect was created at 68-75 days gestation, RTG patch application or untreated at 100-103 days, and harvest at 135-140 days. The RTG was applied to the defect and secured in place with an overlay sealant. Defect areas underwent gross and microscopic analysis for inflammation and skin development. Brains were analyzed for hindbrain herniation and hydrocephalus.
RESULTS
The untreated fetus (n = 1) demonstrated an open defect lacking tissue coverage, evidence of spinal cord injury, increased caspase-3, Iba1 and GFAP in spinal cord tissues, and hindbrain herniation and ventricular dilation. RTG treated fetuses (n = 3) demonstrated defect healing with well-organized dermal and epidermal layers throughout the entire healed tissue area overlaying the defect with minimal inflammation, reduced caspase-3, Iba1 and GFAP in spinal cord tissues, and no hindbrain herniation or ventricular dilation.
CONCLUSION
An RTG patch applied to MMC defects in fetal sheep promoted skin coverage over the defect, was associated with minimal inflammation of the spinal cord tissues and prevented brain abnormalities. The present findings provide exciting results for future comprehensive radiological, functional, and mechanistic evaluation of the RTG.
Topics: Animals; Brain; Female; Fetus; Meningomyelocele; Pregnancy; Sheep; Spinal Cord
PubMed: 34655937
DOI: 10.1016/j.jss.2021.08.015 -
Stem Cell Reviews and Reports Feb 2022Neural tube defects (NTDs) are among the most common congenital defects during neurulation. Spina bifida is a type of NTD that can occur in different forms. Since... (Review)
Review
Neural tube defects (NTDs) are among the most common congenital defects during neurulation. Spina bifida is a type of NTD that can occur in different forms. Since myelomeningocele (MMC) is the most severe form of spina bifida, finding a satisfactory treatment for MMC is a gold standard for the treatment of spina bifida. The Management of Myelomeningocele Study (MOMS) demonstrated that intrauterine treatment of spina bifida could ameliorate the complications associated with spina bifida and would also reduce the placement of ventriculoperitoneal (VP) shunt by 50%. Recently developed tissue engineering (TE) approaches using scaffolds, stem cells, and growth factors allow treatment of the fetus with minimally invasive methods and promising outcomes. The application of novel patches with appropriate stem cells and growth factors leads to better coverage of the defect with fewer complications. These approaches with less invasive surgical procedures, even in animal models with similar characteristics as the human MMC defect, paves the way for the modern application of less invasive surgical methods. Significantly, the early detection of these problems and applying these approaches can increase the potential efficacy of MMC treatment with fewer complications. However, further studies should be conducted to find the most suitable scaffolds and stem cells, and their application should be evaluated in animal models. This review intends to discuss advanced TE methods for treating MMC and recent successes in increasing the efficacy of the treatment.
Topics: Animals; Female; Meningomyelocele; Neural Tube; Pregnancy; Spinal Dysraphism; Stem Cell Transplantation; Tissue Engineering
PubMed: 33742349
DOI: 10.1007/s12015-021-10150-w -
Child's Nervous System : ChNS :... Jan 2021Meningomyelocele is a serious pathology that requires immediate surgical treatment. Its management is difficult due to accompanying other pathologies and hydrocephalus....
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.
Topics: Female; Fistula; Humans; Hydrocephalus; Male; Meningomyelocele; Retrospective Studies; Ventriculoperitoneal Shunt
PubMed: 32632579
DOI: 10.1007/s00381-020-04786-1