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Journal of Indian Association of... 2024Classically, cloacal exstrophy presents with omphalocele, bladder exstrophy, imperforate anus, and spinal defects. We report a rare variant of cloacal exstrophy in a...
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.
PubMed: 38912027
DOI: 10.4103/jiaps.jiaps_241_23 -
Revista Da Associacao Medica Brasileira... 2024The aim of this study was to understand the dynamics of families with children with myelomeningocele undergoing intrauterine fetal surgery.
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.
Topics: Humans; Meningomyelocele; Pilot Projects; Female; Adult; Retrospective Studies; Mothers; Telemedicine; Surveys and Questionnaires; Pregnancy; Infant, Newborn; Family Relations; Gestational Age
PubMed: 38896734
DOI: 10.1590/1806-9282.20231327 -
Acta Neurochirurgica Jun 2024Myelocele 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...
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.
Topics: Humans; Thoracic Vertebrae; Infant; Lumbar Vertebrae; Meningomyelocele; Neurosurgical Procedures; Treatment Outcome; Male; Spinal Dysraphism; Magnetic Resonance Imaging
PubMed: 38884665
DOI: 10.1007/s00701-024-06163-2 -
Cureus May 2024Introduction Congenital malformation studies serve several purposes, including establishing baseline rates, monitoring changes over time, exploring the origins of these...
Introduction Congenital malformation studies serve several purposes, including establishing baseline rates, monitoring changes over time, exploring the origins of these defects, and helping in planning health services. Increasing public awareness about pediatric surgical interventions is another goal of these studies. However, the impact of congenital malformations is often underestimated in developing countries due to insufficient healthcare data and diagnostic facilities, particularly in rural areas. Families affected by the birth of a child with congenital malformations face significant stress and hardship. Methods The main aims of this study were to evaluate the clinical pattern of congenital structural malformations in our region (Uttarakhand, India), identify possibly associated factors of congenital malformations, and find out the immediate outcome of congenital malformations in enrolled participants. Results Among a total of 150 cases, 73 (48.7%) cases were inborn, whereas 77 (51.3%) cases were outborn. Investigation of congenital malformation revealed cleft lip or palate in 37 (24.7%) cases, congenital heart disease (CHD) in 33 (22%) cases, meningomyelocele (MMC) in 18 (12.0%) cases, anorectal malformation (ARM) in 11 (7.3%) cases, hypospadias in 10 (6.7%) cases, congenital talipes equinovarus (CTEV) in nine (6.0%) cases, tracheoesophageal fistula (TEF) in nine (6.0%) cases, polydactyly in seven (4.7%) cases, pelviureteric junction obstruction (PUJO) in four (2.7%) cases, duodenal atresia in three (2.0%) cases, midgut volvulus in three (2.0%) cases, umbilical sinus in two (1.3%) cases, sacrococcygeal teratoma (SCT) in one (0.7%) case, phimosis in one (0.7%) case, microtia in one (0.7%) case, and micrognathia in one (0.7%) case. Mortality was observed in 11 (7.3%) cases, whereas 105 (70%) cases were successfully discharged. Among 11 mortality cases, the cause of death was CHD in seven (63.2%) cases, TEF+CHD in two (18.1%) cases, MMC in one (9%) case, and duodenal atresia in one (9%) case. Conclusion Contrary to the common belief that advanced maternal age of greater than 35 years is a major cause, 86.6% of the congenital structural anomalies in our hospital-based study in Uttarakhand occurred in babies of mothers belonging to the age group of 18-30 years. Also, consanguineous marriage was observed in only 3.3% of cases, indicating that it may not be a major contributing factor causing congenital structural malformations in our region. External congenital anomalies are most commonly observed (60.7%), with cleft lip and cleft palate being the most common. The most frequently observed internal congenital anomaly is CHD (22%) followed by gastrointestinal (GI) (18.6%) and urinary anomalies (10.1%). Death and referral are commonly seen in CHD.
PubMed: 38883135
DOI: 10.7759/cureus.60375 -
Overcoming Airway Hurdles: A Case Report of Anesthetic Challenges in Meningomyelocele Complications.Cureus Apr 2024This case report delves into the anesthesia management in a 23-year-old male with complications of meningomyelocele, a catastrophic congenital neural tube defect. The...
This case report delves into the anesthesia management in a 23-year-old male with complications of meningomyelocele, a catastrophic congenital neural tube defect. The patient, paraplegic since birth with severe scoliosis, presented with a femoral fracture, prompting the need for careful consideration of anesthesia strategies. The challenges included counseling the family on the risks and benefits of surgery under general anesthesia, selecting an appropriate anesthetic plan for an anticipated difficult airway, and addressing ventilation strategies for restrictive lung disease. To tackle the anticipated difficult airway, an awake pediatric fiberoptic bronchoscopy was performed in the recovery room, facilitating a conscious sedation approach. In the operating room, monitored anesthesia care with dexmedetomidine infusion was employed, complemented by careful positioning and padding in the lateral position. The awake fiberoptic checkscopy proved crucial in avoiding unnecessary general anesthesia. A patient-centered approach contributed to the successful execution of a complex anesthesia plan, ensuring optimal care for this unique patient population.
PubMed: 38813317
DOI: 10.7759/cureus.59192 -
BMC Pediatrics May 2024Neural 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...
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 1 to December, 30, 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.
Topics: Humans; Infant, Newborn; Cross-Sectional Studies; Retrospective Studies; Ethiopia; Neural Tube Defects; Female; Male; Neurosurgical Procedures; Treatment Outcome; Hydrocephalus; Hospitals, Special; Meningomyelocele
PubMed: 38773409
DOI: 10.1186/s12887-024-04837-5 -
Experimental Cell Research Jun 2024Prenatal surgery for the treatment of spina bifida (myelomeningocele, MMC) significantly enhances the neurological prognosis of the patient. To ensure better protection...
Prenatal surgery for the treatment of spina bifida (myelomeningocele, MMC) significantly enhances the neurological prognosis of the patient. To ensure better protection of the spinal cord by large defects, the application of skin grafts produced with cells gained from the amniotic fluid is presently studied. In order to determine the most appropriate cells for this purpose, we tried to shed light on the extremely complex amniotic fluid cellular composition in healthy and MMC pregnancies. We exploited the potential of micro-Raman spectroscopy to analyse and characterize human amniotic fluid cells in total and putative (cKit/CD117-positive) stem cells of fetuses with MMC in comparison with amniotic fluid cells from healthy individuals, human fetal dermal fibroblasts and adult adipose derived stem cells. We found that (i) the differences between healthy and MMC amniocytes can be attributed to specific spectral regions involving collagen, lipids, sugars, tryptophan, aspartate, glutamate, and carotenoids, (ii) MMC amniotic fluid contains two particular cell populations which are absent or reduced in normal pregnancies, (iii) the cKit-negative healthy amniocyte subpopulation shares molecular features with human fetal fibroblasts. On the one hand we demonstrate a different amniotic fluid cellular composition in healthy and MMC pregnancies, on the other our work confirms micro-Raman spectroscopy to be a valuable tool for discriminating cell populations in unknown mixtures of cells.
Topics: Humans; Spectrum Analysis, Raman; Amniotic Fluid; Meningomyelocele; Female; Pregnancy; Fetus; Fibroblasts; Cells, Cultured; Adult
PubMed: 38697275
DOI: 10.1016/j.yexcr.2024.114048 -
Neurology India Mar 2024Dural closure is an important part of neurosurgery, failure which may lead to wound infection, pseudomeningoceles, meningitis, severe morbidity to a patient, and even...
INTRODUCTION
Dural closure is an important part of neurosurgery, failure which may lead to wound infection, pseudomeningoceles, meningitis, severe morbidity to a patient, and even mortality. In cases where primary dura closure is not possible, a bovine pericardial patch is a good alternative with the benefits of suturability and the ability to provide watertight closure, hence preventing Cerebrospinal Fluid (CSF) leak. The present study demonstrates the use of the bovine pericardial patch in both cranial and spinal disorders for dural closure as well as for transposition technique in microvascular decompression.
OBJECTIVES
The aim of our study is to understand the advantages and feasibility of a bovine pericardial patch in various neurosurgical procedures.
MATERIAL METHODS
Fifty-one patients were analyzed prospectively and followed up in which glutaraldehyde-free bovine pericardial patch was used in various cranial and spinal disorders.
RESULTS
The most common indications where a bovine pericardial patch was used, in decreasing order of frequency, were meningioma excision surgery (47%), followed by Chiari malformation operated for foramen magnum decompression (17.6%), meningomyelocele (7.8%), spinal dural defects (7.8%), trigeminal neuralgia (5.8%), traumatic decompression with lax duraplasty (4%), glioma (4%), encephalocele (4%), and skull base defects (2%). Two patients had complications, one with CSF leak and the other had superficial wound infection, which were managed appropriately.
CONCLUSIONS
The use of a bovine pericardial patch as a dural substitute in various cranial and spinal disorders is feasible with good outcomes, and it can be considered an ideal dural substitute.
Topics: Humans; Pericardium; Cattle; Male; Female; Adult; Middle Aged; Neurosurgical Procedures; Animals; Dura Mater; Aged; Adolescent; Young Adult; Prospective Studies; Child
PubMed: 38691472
DOI: 10.4103/ni.ni_327_22 -
The Pan African Medical Journal 2024
Topics: Humans; Infant, Newborn; Magnetic Resonance Imaging; Meningomyelocele; Neural Tube Defects
PubMed: 38681107
DOI: 10.11604/pamj.2024.47.61.42654 -
Nigerian Journal of Clinical Practice Apr 2024Tethered cord syndrome is a neurological disorder closely associated with congenital spinal dysraphism. Aberrant dorsal nerve roots may be one of the possible and...
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.
Topics: Humans; Male; Cauda Equina; Magnetic Resonance Imaging; Meningomyelocele; Neural Tube Defects; Spinal Cord; Spinal Nerve Roots; Infant
PubMed: 38679779
DOI: 10.4103/njcp.njcp_838_23