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1.
Acta Neurochir (Wien) ; 166(1): 270, 2024 Jun 17.
Article in English | MEDLINE | ID: mdl-38884665

ABSTRACT

BACKGROUND: Myelocele is a rare form of open spina bifida. Surgical repair is recommended prenatally or in the first 48 h. In some cases, the repair may be delayed, and specific surgical factors need to be considered. METHOD: We give a brief overview of the surgical anatomy, followed by a description of the surgical repair of a thoracolumbar Myelocele in an 11-month-old child. CONCLUSION: Surgical repair of the Myelocele stabilizes the neurological status, prevents local and central nervous system infections. The understanding of Myelocele anatomy enables its removal while preserving as much healthy tissue as possible and restoring normal anatomy.


Subject(s)
Lumbar Vertebrae , Thoracic Vertebrae , Humans , Thoracic Vertebrae/surgery , Thoracic Vertebrae/diagnostic imaging , Infant , Lumbar Vertebrae/surgery , Meningomyelocele/surgery , Neurosurgical Procedures/methods , Treatment Outcome , Male , Spinal Dysraphism/surgery , Magnetic Resonance Imaging
2.
Neurosurg Rev ; 47(1): 292, 2024 Jun 25.
Article in English | MEDLINE | ID: mdl-38914693

ABSTRACT

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.


Subject(s)
Neural Tube Defects , Recovery of Function , Walking , Humans , Male , Female , Walking/physiology , Child , Recovery of Function/physiology , Retrospective Studies , Neural Tube Defects/surgery , Child, Preschool , Adolescent , Meningomyelocele/surgery , Treatment Outcome , Neurosurgical Procedures/methods
3.
Rev Assoc Med Bras (1992) ; 70(6): e20231327, 2024.
Article in English | MEDLINE | ID: mdl-38896734

ABSTRACT

OBJECTIVE: The aim of this study was to understand the dynamics of families with children with myelomeningocele undergoing intrauterine fetal surgery. METHODS: A retrospective cohort pilot study was carried out with 11 mothers of children who had undergone intrauterine myelomeningocele repair. Participants in this study responded to an electronic questionnaire (via Google Forms), developed by the study authors, that consisted of 22 multiple-choice questions, of which 17 were closed-ended and 5 had a standardized format. RESULTS: The mean (± standard deviation) of the mothers' age was 37.6 (± 3.5) years. The median of gestational age at delivery and birthweight were 34.9 (range, 33 to 36.1) weeks and 2,300 (range, 1,950 to 2,763) g, respectively. The majority of mothers were white (81.8%), had university degree (81.8%), were Catholic (63.6%), and were married (100%). The majority of mothers rated their relationship with their husband, family, and friends as excellent (54.5, 72.7, and 54.5%, respectively). All 11 mothers reported that the newborn with myelomeningocele was born <37 weeks gestation and the birthweight most often<2,500 g. Approximately 64% of the mothers reported that their child required adaptations or had special needs, of which walking aids (50%) and bladder control (50%) were the most common ones. CONCLUSION: Telemedicine proved to be a useful tool in the long-term follow-up of children who underwent intrauterine surgery to correct myelomeningocele.


Subject(s)
Meningomyelocele , Mothers , Telemedicine , Humans , Meningomyelocele/surgery , Pilot Projects , Female , Adult , Retrospective Studies , Surveys and Questionnaires , Pregnancy , Infant, Newborn , Family Relations , Gestational Age
5.
BMC Pediatr ; 24(1): 350, 2024 May 22.
Article in English | MEDLINE | ID: mdl-38773409

ABSTRACT

BACKGROUND: Neural tube defects (NTDs) account for the largest proportion of congenital anomalies of the central nervous system and result from failure of the neural tube to close spontaneously between the 3rd and 4th weeks of in utero development. Prognosis and treatment outcome depends on the nature and the pattern of the defect. The nature of treatment outcomes and its pattern associated with grave prognosis is not well known in the study area. OBJECTIVE: The aim of study was to determine the patterns and short term neurosurgical management outcomes of newborns with neural tube defects admitted at Felege Hiwot Specialized Hospital. METHODS: Institutional based retrospective cross-sectional study among neonates, who were admitted at Felege Hiwot Specialized Hospital with neural tube defects from January 1st to December, 30th, 2018 was conducted. All Charts of Neonates with confirmed diagnosis of neural tube defects were included as part of the study. Trained data collectors (medical interns) supervised by trained supervisors (general practitioners) collected the data using a pretested data extraction format. Data were coded, entered and analyzed using SPSS version 23 software. Frequency and cross tabulations were used to summarize descriptive statistics of data, and tables and graphs were used for data presentation. RESULT: About 109 patients had complete documentation and imaging confirmed neural tube defects. Myelomeningocele was the commonest pattern 70 (64.2%). Thoracolumbar spine was the commonest site of presentation 49(45%). The most common associated impairment was hydrocephalus 37(33.9%). Forty-five (41.1%) had multiple complications. The mortality rate was 7.3%, 44% were discharged with sequalae and 36.7% were discharged without impairment. The significant causes of death were infection 66.7% and Chiari crisis 33.3%. CONCLUSION: Myelomeningocele was the most frequent clinical pattern of neural tube defect and thoracolumbar spine was the commonest site. Isolated neural tube defect was the commonest finding. There were multiple complications after surgery accompanied with meningitis and hydrocephalus. The mortality rate among neonates with neural tube defects was considerably high. The commonest causes of death were infection and Chiari crisis.


Subject(s)
Neural Tube Defects , Humans , Infant, Newborn , Cross-Sectional Studies , Retrospective Studies , Ethiopia/epidemiology , Neural Tube Defects/surgery , Female , Male , Neurosurgical Procedures/statistics & numerical data , Neurosurgical Procedures/methods , Treatment Outcome , Hydrocephalus/surgery , Hospitals, Special/statistics & numerical data , Meningomyelocele/surgery , Meningomyelocele/complications
6.
Adv Tech Stand Neurosurg ; 49: 95-122, 2024.
Article in English | MEDLINE | ID: mdl-38700682

ABSTRACT

Open spina bifida, also known as myelomeningocele (MMC), is the most challenging and severe birth defect of the central nervous system compatible with life and it is due to a failure in the dorsal fusion of the nascent neural tube during embryonic development. MMC is often accompanied by a constellation of collateral conditions, including hydrocephalus, Arnold - Chiari II malformation, brainstem disfunction, hydrosyringomyelia, tethered cord syndrome and scoliosis. Beyond early surgical repair of the dorsal defect, MMC requires lifelong cares. Several additional surgical procedures are generally necessary to improve the long-term outcomes of patients affected by MMC and multidisciplinary evaluations are crucial for early identification and management of the various medical condition that can accompany this pathology. In this chapter, the most common pathological entities associated with MMC are illustrated, focusing on clinical manifestations, treatment strategies and follow up recommendations.


Subject(s)
Meningomyelocele , Neurosurgical Procedures , Humans , Meningomyelocele/surgery , Neurosurgical Procedures/methods
7.
Niger J Clin Pract ; 27(4): 537-540, 2024 Apr 01.
Article in English | MEDLINE | ID: mdl-38679779

ABSTRACT

ABSTRACT: Tethered cord syndrome is a neurological disorder closely associated with congenital spinal dysraphism. Aberrant dorsal nerve roots may be one of the possible and relatively rare tethering pathologies, especially in the complex form of occult spinal dysraphism such as caudal regression syndrome or split cord malformation. We report an illustrative case of caudal regression syndrome with spinal cord tethering due to a combination of a contiguous bundle of an aberrant dorsal nerve root, and a dorsal-type lipomyelomeningocele, with a thickened filum terminale treated with microneurosurgical untethering.


Subject(s)
Meningomyelocele , Neural Tube Defects , Humans , Male , Cauda Equina/abnormalities , Magnetic Resonance Imaging , Meningomyelocele/complications , Meningomyelocele/surgery , Neural Tube Defects/complications , Neural Tube Defects/surgery , Spinal Cord/abnormalities , Spinal Nerve Roots/abnormalities , Infant
8.
Fetal Pediatr Pathol ; 43(3): 225-233, 2024.
Article in English | MEDLINE | ID: mdl-38634787

ABSTRACT

Background: Myelomeningocele or spina bifida is an open neural tube defect that is characterized by protrusion of the meninges and the spinal cord through a deformity in the vertebral arch and spinous process. Myelomeningocele of post-natal tissue is well described; however, pre-natal tissue of this defect has no known previous histologic characterization. We compared the histology of different forms of pre-natal myelomeningocele and post-natal myelomeningocele tissue obtained via prenatal intrauterine and postnatal surgical repairs. Methods: Pre-and post-natal tissues from spina bifida repair surgeries were obtained from lipomyelomeningocele, myeloschisis, and myelomeningocele spina bifida defects. Tissue samples were processed for H&E and immunohistochemical staining (KRT14 and p63) to assess epidermal and dermal development. Results: Prenatal skin near the defect site develops with normal epidermal, dermal, and adnexal structures. Within the grossly cystic specimens, histology shows highly dense fibrous connective tissue with complete absence of a normal epidermal development with a lack of p63 and KRT14 expression. Conclusion: Tissues harvested from prenatal and postnatal spina bifida repair surgeries appear as normal skin near the defect site. However, cystic tissues consist of highly dense fibrous connective tissue with complete absence of normal epidermal development.


Subject(s)
Immunohistochemistry , Meningomyelocele , Spinal Dysraphism , Humans , Spinal Dysraphism/pathology , Spinal Dysraphism/surgery , Female , Immunohistochemistry/methods , Meningomyelocele/surgery , Meningomyelocele/pathology , Meningomyelocele/metabolism , Pregnancy , Infant, Newborn
9.
Eur J Med Res ; 29(1): 200, 2024 Mar 25.
Article in English | MEDLINE | ID: mdl-38528560

ABSTRACT

BACKGROUND: The current convention for treatment of children with myelomeningocele (MMC) is timely surgical intervention combined with long-term follow-up by a multidisciplinary specialized team. This study aims to investigate the outcomes of MMC patients treated at Namazi Hospital. METHODS: All children presenting to Namazi Hospital with myelomeningocele between May 2001 and August 2020 were eligible for this study. For those with a documented telephone number, follow-up phone surveys with the patient's caregivers, on top of the review of the medical documents were carried out to assess mortality, morbidities, and the functional outcome of the care provided to them. RESULTS: A total of 125 patients were studied (62 females). All of the patients were followed up for a mean duration of 6.28 years (range 1-23 years). The majority were located in the lumbosacral area. All of the patients underwent postnatal surgical intervention for MMC in Namazi Hospital. Mean age at surgery was 9.51 days. There were statistically significant differences between urinary and bowel incontinence and presence of scoliosis, MMT grading of the lower limbs, school attendance, number of readmissions, and requirement of laminectomy at the initial surgical intervention. CONCLUSIONS: This study is the first to characterize the long-term outcomes of MMC patients in Iran. This study illustrates that there is a great need for improved access to and coordination of care in antenatal, perioperative, and long-term stages to improve morbidity and mortality.


Subject(s)
Meningomyelocele , Child , Humans , Female , Pregnancy , Infant, Newborn , Meningomyelocele/surgery , Follow-Up Studies , Iran/epidemiology , Referral and Consultation , Retrospective Studies
10.
Childs Nerv Syst ; 40(5): 1541-1569, 2024 May.
Article in English | MEDLINE | ID: mdl-38459148

ABSTRACT

OBJECTIVES: To describe surgical treatment of 3 cases of severe and progressive thoracolumbar kyphosis in myelomeningocele and provide a systematic review of the available literature on the topic. METHODS: Medical records and pre- and post-operative imaging of 3 patients with thoracolumbar kyphosis and myelomeningocele were reviewed. A database search was performed for all manuscripts published on kyphectomy and/or surgical treatment of kyphosis in myelomeningocele. Patients' information, preoperative kyphosis angle, type of surgery, levels of surgery degrees of correction after surgery and at follow-up, and complications were reviewed for the included studies. RESULTS: Three cases underwent posterior vertebral column resection (pVCR) of 2-4 segments at the apex of the kyphosis (kyphectomy). Long instrumentation was performed with all pedicle screws constructed from the thoracic spine to the pelvis using iliac screws. According to literature review, a total of 586 children were treated for vertebral kyphosis related to myelomeningocele. At least one vertebra was excised to gain some degree of correction of the deformity. Different types of instrumentation were used over time and none of them demonstrated to be superior over the other. CONCLUSION: Surgical treatment of progressive kyphosis in myelomeningocele has evolved over the years incorporating all major advances in spinal instrumentation techniques. Certainly, the best results in terms of preservation of correction after surgery and less revision rates were obtained with long construct and screws. However, complication rate remains high with skin problems being the most common complication. The use of low-profile instrumentation remains critical for treatment of these patients.


Subject(s)
Kyphosis , Meningomyelocele , Spinal Fusion , Child , Humans , Meningomyelocele/surgery , Treatment Outcome , Spinal Fusion/methods , Retrospective Studies , Kyphosis/surgery
11.
Childs Nerv Syst ; 40(5): 1571-1575, 2024 May.
Article in English | MEDLINE | ID: mdl-38514517

ABSTRACT

The intrauterine repair of myelomeningocele presents certain advantages and has gained widespread acceptance. It significantly reduces the incidence of Chiari-2 anomalies and hydrocephalus, and it is thought to enhance the neurologic outcome. Nevertheless, several issues remain unsettled and there are no negligible disadvantages. After working with patients with myelomeningocele for 30 years, I thought about how we currently treat them. There are ethical, organizational, neurological, obstetrical, and postnatal aspects worth discussing.


Subject(s)
Arnold-Chiari Malformation , Hydrocephalus , Meningomyelocele , Humans , Meningomyelocele/surgery , Arnold-Chiari Malformation/complications , Hydrocephalus/surgery , Incidence
12.
J Neurosurg Pediatr ; 33(6): 554-563, 2024 Jun 01.
Article in English | MEDLINE | ID: mdl-38457805

ABSTRACT

OBJECTIVE: The need for permanent CSF diversion is lower in patients who have undergone prenatal surgery for myelomeningocele (MMC) than in those who have undergone postnatal closure. Differences in brain development and head growth between treatment groups are not known, particularly for those who do not require surgical treatment for hydrocephalus. The objective of this study was to determine differences in head growth and to generate MMC-specific head circumference (HC) growth curves for patients who underwent either prenatal or postnatal surgery. METHODS: The authors retrospectively identified patients from St. Louis Children's Hospital who were treated for MMC between 2016 and 2021. HC data were obtained from birth until the most recent follow-up or hydrocephalus treatment. Nonlinear least-squares regression analysis was performed to fit the data into four models: two-term power, Gompertz, West ontogenetic, and Weibull. Subsequently, the curves were assessed for their utility in predicting hydrocephalus treatment. RESULTS: Sixty-one patients (29 females [47.5%], 25 [41%] underwent prenatal surgery, mean gestational age at birth 36.6 weeks) were included in the study. The Weibull model best fit the HC data (prenatal adjusted R2 = 0.95, postnatal adjusted R2 = 0.95), while the Gompertz model had the worst fit (prenatal adjusted R2 = 0.56, postnatal adjusted R2 = 0.39) across both cohorts. Prenatal MMC repair patients had significantly larger HC measurements than their postnatal repair counterparts. The 50th percentile of the Weibull curve was determined as a useful threshold for hydrocephalus treatment: children with HC measurements that crossed and remained above this threshold were significantly more likely to have hydrocephalus treatment regardless of time of MMC repair (prenatal relative risk [RR] 10.0 [95% CI 1.424-70.220], sensitivity 85.7% [95% CI 0.499-0.984], and specificity 82.4% [95% CI 0.600-0.948]; postnatal RR 4.750 [95% CI 1.341-16.822], sensitivity 90.5% [95% CI 0.728-0.980], and specificity 75.0% [95% CI 0.471-0.924]). The HC growth curves of the MMC patients treated prenatally were significantly larger than the WHO HC curves (p < 0.001). CONCLUSIONS: The Weibull model was identified as the HC growth curve with the best fit for MMC patients and serves as a useful predictor of hydrocephalus treatment. For MMC patients with hydrocephalus, prenatal repair patients fit the model well but postnatal repair patients did not, potentially indicating different mechanisms of hydrocephalus development. Those treated prenatally had significantly larger HC measurements compared with both the general population and those treated postnatally. Further study is needed to understand the long-term cognitive outcomes and optimal management of clinically asymptomatic patients with large HC measurements who were treated prenatally for MMC.


Subject(s)
Head , Hydrocephalus , Meningomyelocele , Humans , Meningomyelocele/surgery , Meningomyelocele/complications , Female , Male , Retrospective Studies , Head/surgery , Infant, Newborn , Hydrocephalus/surgery , Pregnancy , Infant , Gestational Age , Cephalometry
13.
J Neurosurg Pediatr ; 33(6): 544-553, 2024 Jun 01.
Article in English | MEDLINE | ID: mdl-38457812

ABSTRACT

OBJECTIVE: Although hydrocephalus rates have decreased with intrauterine surgery for myelomeningocele (MMC), 40%-85% of children with MMC still go on to develop hydrocephalus. Prenatal ventricle size is known to be associated with later development of hydrocephalus; however, it is not known how prediction measures or timing of hydrocephalus treatment differ between pre- and postnatal surgery for MMC. The goal of this study was to determine anatomical, clinical, and radiological characteristics that are associated with the need for and timing of hydrocephalus treatment in patients with MMC. METHODS: The authors retrospectively identified patients from Barnes Jewish Hospital or St. Louis Children's Hospital between 2016 and 2021 who were diagnosed with MMC prenatally and underwent either pre- or postnatal repair. Imaging, clinical, and demographic data were examined longitudinally between treatment groups and hydrocephalus outcomes. RESULTS: Fifty-eight patients were included (27 females, 46.6%), with a mean gestational age at birth of 36.8 weeks. Twenty-three patients (39.7%) underwent prenatal surgery. For the overall cohort, the ventricle size at prenatal ultrasound (HR 1.175, 95% CI 1.071-1.290), frontal-occipital horn ratio (FOHR) at birth > 0.50 (HR 3.603, 95% CI 1.488-8.720), and mean rate of change in head circumference (HC) in the first 90 days after birth (> 0.10 cm/day: HR 12.973, 95% CI 4.262-39.486) were identified as predictors of hydrocephalus treatment. The factors associated with hydrocephalus in the prenatal cohort were FOHR at birth > 0.50 (HR 27.828, 95% CI 2.980-259.846) and the rate of change in HC (> 0.10 cm/day: HR 39.414, 95% CI 2.035-763.262). The factors associated with hydrocephalus in the postnatal cohort were prenatal ventricle size (HR 1.126, 95% CI 1.017-1.246) and the mean rate of change in HC (> 0.10 cm/day: HR 24.202, 95% CI 5.119-114.431). FOHR (r = -0.499, p = 0.008) and birth HC (-0.409, p = 0.028) were correlated with time to hydrocephalus across both cohorts. For patients who underwent treatment for hydrocephalus, those in the prenatal surgery group were significantly more likely to develop hydrocephalus after 3 months than those treated with postnatal surgery, although the overall rate of hydrocephalus was significantly higher in the postnatal surgery group (p = 0.018). CONCLUSIONS: Clinical and imaging factors associated with hydrocephalus treatment differ between those receiving pre- versus postnatal MMC repair, and while the overall rate of hydrocephalus is lower, those undergoing prenatal repair are more likely to develop hydrocephalus after 3 months of age. This has implications for clinical follow-up timing for patients treated prenatally, who may live at a distance from the treatment site.


Subject(s)
Hydrocephalus , Meningomyelocele , Humans , Hydrocephalus/surgery , Hydrocephalus/etiology , Hydrocephalus/diagnostic imaging , Meningomyelocele/surgery , Meningomyelocele/complications , Meningomyelocele/diagnostic imaging , Female , Male , Retrospective Studies , Infant, Newborn , Pregnancy , Ultrasonography, Prenatal , Gestational Age , Treatment Outcome , Infant
14.
J Neurosurg Pediatr ; 33(5): 405-410, 2024 May 01.
Article in English | MEDLINE | ID: mdl-38428005

ABSTRACT

OBJECTIVE: Among patients with a history of prior lipomyelomeningocele repair, an association between increased lumbosacral angle (LSA) and cord retethering has been described. The authors sought to build a predictive algorithm to determine which complex tethered cord patients will develop the symptoms of spinal cord retethering after initial surgical repair with a focus on spinopelvic parameters. METHODS: An electronic medical record database was reviewed to identify patients with complex tethered cord (e.g., lipomyelomeningocele, lipomyeloschisis, myelocystocele) who underwent detethering before 12 months of age between January 1, 2008, and June 30, 2022. Descriptive statistics were used to characterize the patient population. The Caret package in R was used to develop a machine learning model that predicted symptom development by using spinopelvic parameters. RESULTS: A total of 72 patients were identified (28/72 [38.9%] were male). The most commonly observed dysraphism was lipomyelomeningocele (41/72 [56.9%]). The mean ± SD age at index MRI was 2.1 ± 2.2 months, at which time 87.5% of patients (63/72) were asymptomatic. The mean ± SD lumbar lordosis at the time of index MRI was 23.8° ± 11.1°, LSA was 36.5° ± 12.3°, sacral inclination was 30.4° ± 11.3°, and sacral slope was 23.0° ± 10.5°. Overall, 39.6% (25/63) of previously asymptomatic patients developed new symptoms during the mean ± SD follow-up period of 44.9 ± 47.2 months. In the recursive partitioning model, patients whose LSA increased at a rate ≥ 5.84°/year remained asymptomatic, whereas those with slower rates of LSA change experienced neurological decline (sensitivity 77.5%, specificity 84.9%, positive predictive value 88.9%, and negative predictive value 70.9%). CONCLUSIONS: This is the first study to build a machine learning algorithm to predict symptom development of spinal cord retethering after initial surgical repair. The authors found that, after initial surgery, patients who demonstrate a slower rate of LSA change per year may be at risk of developing neurological symptoms.


Subject(s)
Algorithms , Machine Learning , Meningomyelocele , Neural Tube Defects , Humans , Neural Tube Defects/surgery , Neural Tube Defects/diagnostic imaging , Female , Male , Meningomyelocele/surgery , Meningomyelocele/diagnostic imaging , Infant , Retrospective Studies , Neurosurgical Procedures/methods , Magnetic Resonance Imaging , Predictive Value of Tests
15.
Clin Neurol Neurosurg ; 239: 108214, 2024 04.
Article in English | MEDLINE | ID: mdl-38503112

ABSTRACT

BACKGROUND AND PURPOSE: Spina bifida is the second major cause of congenital disorders and the most common central nervous system congenital malformation compatible with life primarily. Herein, we describe the short-term outcome of post-natal Myelomeningocele (MMC) surgical management and predictors of its postoperative complications and mortality. METHODS: This retrospective chart review studies the children who underwent post-natal surgical management for MMC in Namazi hospital, a tertiary referral center, in southern Iran from May 2001 to September 2020. RESULTS: 248 patients were included in this study. The mean age at the operation was 8.47 ± 8.69 days. The most common site of involvement of MMC was Lumbosacral (86%, n = 204). At the evaluation conducted prior to the operation, cerebrospinal fluid leak was observed in 7% (n=16) of the patients. Postoperatively, 5.7% of the patients expired in the 30-day follow-up after the operation (n = 14), while 24% needed readmission (n = 47). The most common complications leading to readmission were wound dehiscence (n = 10, 42%) and wound purulence (n = 6, 25%). Only the site of the lesion (p-value = 0.035) was associated with postoperative complication. After controlling for potential confounders, the site of the lesion (adjusted odds ratio = 0.146, 95% confidence interval = 0.035-0.610, p-value = 0.008) and age at surgery (adjusted odds ratio = 1.048, 95% confidence interval = 1.002-1.096, p-value = 0.041) were significantly associated with mortality CONCLUSIONS: The age of the patients at the surgery and the site of the lesion are the two factors that were associated with mortality. However, further investigations into preoperative interventions and risk factors to mitigate the risk of complications and mortality are highly encouraged.


Subject(s)
Hydrocephalus , Meningomyelocele , Child , Humans , Infant, Newborn , Meningomyelocele/surgery , Meningomyelocele/complications , Retrospective Studies , Hydrocephalus/surgery , Postoperative Complications/epidemiology , Treatment Outcome
16.
Braz J Anesthesiol ; 74(3): 844495, 2024.
Article in English | MEDLINE | ID: mdl-38521500

ABSTRACT

BACKGROUND: Myelomeningocele (MMC) is a neural tube defect disease. Antenatal repair of fetal MMC is an alternative to postnatal repair. Many agents can be used as tocolytics during the in utero fetal repair such as ß2-agonists and oxytocin receptor antagonists, with possible maternal and fetal repercussions. This study aims to compare maternal arterial blood gas analysis between terbutaline or atosiban, as tocolytic agents, during intrauterine MMC repair. METHODS: Retrospective cohort study. Patients were divided into two groups depending on the main tocolytic agent used during intrauterine MMC repair: atosiban (16) or terbutaline (9). Maternal arterial blood gas samples were analyzed on three moments: post induction (baseline, before the start of tocolysis), before extubation, and two hours after the end of the surgery. RESULTS: Twenty-five patients were included and assessed. Before extubation, the terbutaline group showed lower arterial pH (7.347 ± 0.05 vs. 7.396 ± 0.02 for atosiban, p = 0.006) and higher arterial lactate (28.33 ± 12.76 mg.dL-1 vs. 13.06 ± 6.35 mg.dL-1, for atosiban, p = 0.001) levels. CONCLUSIONS: Patients who received terbutaline had more acidosis and higher levels of lactate, compared to those who received atosiban, during intrauterine fetal MMC repair.


Subject(s)
Meningomyelocele , Terbutaline , Tocolytic Agents , Vasotocin , Humans , Retrospective Studies , Terbutaline/therapeutic use , Terbutaline/administration & dosage , Female , Meningomyelocele/surgery , Adult , Tocolytic Agents/administration & dosage , Pregnancy , Vasotocin/analogs & derivatives , Vasotocin/therapeutic use , Cohort Studies , Blood Gas Analysis
17.
J Orthop Surg Res ; 19(1): 114, 2024 Feb 03.
Article in English | MEDLINE | ID: mdl-38308272

ABSTRACT

OBJECTIVE: Neural tube defects are the most common congenital disorders after cardiac anomalies. Lumbar kyphosis deformity is observed in 8-15% of these patients. This deformity severely limits the daily lives of these patients. In our study, we aimed to correct the kyphosis angle of the patients with lumbar kyphosis associated with myelomeningocele (MMC) and allow them to continue their growth without limiting their lung capacity by applying kyphectomy and sliding growing rod technique. PATIENTS AND METHODS: In this study, we retrospectively evaluated 24 patients with congenital lumbar kyphosis deformity associated with MMC, aged between 4 and 9 years, and who applied to Umraniye Training and Research Hospital between the dates of 2018 and 2021. We evaluated preoperative and postoperative kyphosis angles, correction rates, bleeding during operations, operation time, level of instrumentation, number of the resected vertebrae, initial levels of the posterior defects, duration of hospital stays, annual lengthening, and weight of the patients. RESULTS: Mean age was 5.04 (between 4 and 9). Mean preoperative and early postoperative kyphosis angles were 129.8° (87-175°) and 0.79° (- 20-24°), respectively. The kyphotic deformity correction rate was 99.1%. A difference was found regarding kyphosis measurements between preoperative and early period values (p < 0.05). The annual height lengthening of patients was calculated as 0.74 cm/year and 0.77 cm/year between T1-T12 and T1-S1, respectively. Mean preoperative level of hemoglobin (Hgb) was 11.95, postoperative Hgb value was 10.02, and the decrease was significant (p < 0.05). In terms of complications, 50% (12) had broken/loosen screws, 50% (12) had undergone debridement surgery, 37.5% (9) had vacuum-assisted closure therapy, and 33.3% (8) had to get all of their implants removed. CONCLUSION: We believe that our sliding growing rod technique is a new and updated surgical method that can be applied in these patient groups, facilitating the life, rehabilitation process, and daily care of MMC patients with lumbar kyphosis. This technique seems to be a safe and reliable method which preserves lung capacity and allows lengthening.


Subject(s)
Kyphosis , Meningomyelocele , Scoliosis , Spinal Fusion , Humans , Child, Preschool , Child , Meningomyelocele/complications , Meningomyelocele/diagnostic imaging , Meningomyelocele/surgery , Retrospective Studies , Kyphosis/diagnostic imaging , Kyphosis/etiology , Kyphosis/surgery , Spine , Scoliosis/complications , Scoliosis/diagnostic imaging , Treatment Outcome , Lumbar Vertebrae/diagnostic imaging , Lumbar Vertebrae/surgery , Spinal Fusion/methods
19.
Fetal Diagn Ther ; 51(2): 175-183, 2024.
Article in English | MEDLINE | ID: mdl-38190813

ABSTRACT

INTRODUCTION: The Management of Myelomeningocele Study (MOMS) eligibility criteria preclude in utero surgery for fetal spina bifida (fSB) when the maternal body mass index (BMI) is ≥35 kg/m2. Some centers still respect this criterion, while others, like ours, do not. This study aimed to assess whether maternal and fetal safety is compromised with higher maternal BMIs. METHODS: Data of 192 patients with open fSB repair at our center were retrospectively analyzed. According to their BMI, patients were divided into three groups: group 1 (BMI <30 kg/m2), group 2 (BMI 30-35 kg/m2), and group 3 (BMI >35 kg/m2). Subgroup analysis was performed to assess differences in maternal and fetal outcomes. Additionally, complications were divided into grades 1 to 5 according to their severity and outcome consequences and compared among groups. RESULTS: Out of 192 patients, 146 (76.0%) had a BMI <30 kg/m2, 28 (14.6%) had a BMI 30-35 kg/m2, and 18 (9.4%) had a BMI >35 kg/m2. Significant differences occurring more often in either group 2 or 3 compared to group 1 were maternal wound seroma (50% or 56% vs. 32%, p = 0.04), amniotic fluid leakage (14% or 6% vs. 2%, p = 0.01) as well as vaginal bleeding (11% or 35% vs. 9%, p = 0.01). On the contrary, duration of tocolysis with atosiban was shorter in patients with BMI >30 kg/m2 (4 or 5 vs. 6 days, p = 0.01). When comparing severity of maternal or fetal complications, grade 1 intervention-related complications occurred significantly more often in group 3 compared to group 1 or 2 (78% vs. 45% or 57%, p = 0.02). Gestational age at delivery was around 36 weeks in all groups without significant differences. CONCLUSION: This investigation did not identify clinically relevant maternal and/or fetal outcome problems related to BMIs >35 kg/m2. Additional studies are however needed to confirm our results.


Subject(s)
Meningomyelocele , Spina Bifida Cystica , Spinal Dysraphism , Pregnancy , Female , Humans , Infant , Retrospective Studies , Fetus/surgery , Meningomyelocele/surgery , Meningomyelocele/complications , Obesity/complications , Spinal Dysraphism/complications , Spinal Dysraphism/surgery , Spina Bifida Cystica/surgery
20.
Am J Obstet Gynecol MFM ; 6(3): 101278, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38232818

ABSTRACT

BACKGROUND: Fetoscopic spina bifida repair is increasingly being practiced, but limited skill acquisition poses a barrier to widespread adoption. Extensive training in relevant models, including both ex vivo and in vivo models may help. To address this, a synthetic training model that is affordable, realistic, and that allows skill analysis would be useful. OBJECTIVE: This study aimed to create a high-fidelity model for training in the essential neurosurgical steps of fetoscopic spina bifida repair using synthetic materials. In addition, we aimed to obtain a cheap and easily reproducible model. STUDY DESIGN: We developed a 3-layered, silicon-based model that resemble the anatomic layers of a typical myelomeningocele lesion. It allows for filling of the cyst with fluid and conducting a water tightness test after repair. A compliant silicon ball mimics the uterine cavity and is fixed to a solid 3-dimensional printed base. The fetal back with the lesion (single-use) is placed inside the uterine ball, which is reusable and repairable to allow for practicing port insertion and fixation multiple times. Following cannula insertion, the uterus is insufflated and a clinical fetoscopic or robotic or prototype instruments can be used. Three skilled endoscopic surgeons each did 6 simulated fetoscopic repairs using the surgical steps of an open repair. The primary outcome was surgical success, which was determined by water tightness of the repair, operation time <180 minutes and an Objective Structured Assessment of Technical Skills score of ≥18 of 25. Skill retention was measured using a competence cumulative sum analysis of a composite binary outcome of surgical success. Secondary outcomes were cost and fabrication time of the model. RESULTS: We made a model that can be used to simulate the neurosurgical steps of spina bifida repair, including anatomic details, port insertion, placode release and descent, undermining of skin and muscular layer, and endoscopic suturing. The model was made using reusable 3-dimensional printed molds and easily accessible materials. The 1-time startup cost was €211, and each single-use, simulated myelomeningocele lesion cost €9.5 in materials and 50 minutes of working time. Two skilled endoscopic surgeons performed 6 simulated, 3-port fetoscopic repairs, whereas a third used a Da Vinci surgical robot. Operation times decreased by more than 30% from the first to the last trial. Six experiments per surgeon did not show an obvious Objective Structured Assessment of Technical Skills score improvement. Competence cumulative sum analysis confirmed competency for each surgeon. CONCLUSION: This high-fidelity, low-cost spina bifida model allows simulated dissection and closure of a myelomeningocele lesion. VIDEO ABSTRACT.


Subject(s)
Meningomyelocele , Spinal Dysraphism , Pregnancy , Female , Humans , Meningomyelocele/diagnosis , Meningomyelocele/surgery , Silicon , Spinal Dysraphism/diagnosis , Spinal Dysraphism/surgery , Fetoscopy/methods , Water
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