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Journal of Indian Association of... 2023A 7-day-old male child presented with abdominal distention and jaundice. Radiological investigations revealed an encapsulated sac encasing three fetus-in-fetu (FIF) in...
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.
PubMed: 37842209
DOI: 10.4103/jiaps.jiaps_113_23 -
Cureus Nov 2023In the present study, neonates presenting with neural tube defects (NTDs) and undergoing observation within the confines of the neonatal intensive care unit (NICU) were...
INTRODUCTION
In the present study, neonates presenting with neural tube defects (NTDs) and undergoing observation within the confines of the neonatal intensive care unit (NICU) were subjected to a comprehensive assessment encompassing concurrent morbidities, clinical manifestations, laboratory parameters, and instituted interventions.
MATERIALS AND METHODS
A retrospective examination was conducted on the medical records of 135 neonates diagnosed with congenital NTD within the temporal span from 2008 to 2018. The study cohort was drawn from the NICU of the Health Sciences University Erzurum Regional and Research Hospital Health Practice and Research Center.
DISCUSSION
The current investigation encompasses a retrospective analysis of 135 patients diagnosed with NTD who received treatment at the NICU between the years 2008 and 2018. Among these, 74 individuals (54.2%) were male, while 61 (45.8%) were female. Maternal ages ranged from 17 to 46 years, with variations in the number of pregnancies, ranging from 1 to 10. Notably, 71 cases (52.6%) were delivered through normal spontaneous delivery, whereas 64 cases (47.4%) underwent cesarean section. The familial context revealed that five patients (3.6%) had siblings with a history of NTD, while no instances were noted where mothers had received antenatal folic acid support. Birth weights of the neonates ranged from 1425 to 4500 grams. Consanguinity was identified in the parental relationships of 17 cases (12.6%). The average diameter of the neural tube sac was determined to be 4.83 ± 1.94 cm (1-12 cm). Predominantly, the lumbosacral region emerged as the most common site of NTD, with meningomyelocele being the prevailing NTD type. Hydrocephalus coexisted in 67 cases, and notably, 44 instances exhibited the development of hydrocephalus post-sac operation. Eight patients were deemed inoperable, and the initial surgery transpired at an average age of 4.3 ± 2.6 (0-17) days. Flap closure constituted 32 of the surgical interventions, while primary closure was implemented in 95 cases. Neurogenic bladder antedated the operation in 14 patients, and 12 individuals developed neurogenic bladder postoperatively. Ventriculoperitoneal shunt placement was warranted in 47 patients. The average duration of hospitalization was 22.5 ± 14.4 days. Regrettably, three patients died due to complications and infections during their hospital stay.
RESULT
NTD represents a significant cohort of pathologies necessitating a comprehensive and interdisciplinary management strategy. These anomalies are characterized by elevated morbidity and mortality rates, not only exerting substantial financial strains on societal, familial, and state healthcare resources but also inflicting profound emotional distress upon affected families. Crucially, periconceptional strategies emphasizing balanced nutrition coupled with targeted multivitamin and mineral supplementation, particularly the inclusion of folic acid, assume paramount importance in the prophylaxis of this debilitating condition.
PubMed: 38024033
DOI: 10.7759/cureus.48886 -
Veterinary Medicine and Science Nov 2023Open spina bifida is an uncommon malformation in animals, and there is a lack of imaging, clinical, and pathological characterisation of this condition in dogs.
BACKGROUND
Open spina bifida is an uncommon malformation in animals, and there is a lack of imaging, clinical, and pathological characterisation of this condition in dogs.
OBJECTIVE
Open spina bifida is rarely observed in animals due to high levels of perinatal mortality and frequent euthanasia. To the best of our knowledge, we present the first case of spina bifida in a dog was diagnosed in-utero and then followed post-partum.
METHODS
A 3-year-old Poodle was presented with twin pregnancy. Radiographic and ultrasonographic findings were suggestive of vertebral malformation and open spina bifida with myelomeningocele in one foetus. Conservative treatment was given but the puppy died 3 days after birth. Thereafter, anatomical and histopathological analysis of several organs was performed to characterise the disease.
RESULTS
When the twins were born, one puppy had a linear dorsal midline cutaneous defect extending from the level of vertebrae L2-L6. R Radiographic examination showed several congenital vertebral malformations involving the thoracic segment, lumbar segment, sacrum and scapula. Histopathological examinations confirmed the presence of open spina bifida and identified additional abnormalities in several internal organs.
CONCLUSIONS
This case presents a complete characterisation of open spina bifida, before birth and after death, using imaging and histopathology techniques.
Topics: Pregnancy; Female; Dogs; Animals; Spina Bifida Cystica; Spinal Dysraphism; Meningomyelocele; Fetus; Dog Diseases
PubMed: 37705413
DOI: 10.1002/vms3.1266 -
Cognition and emotional distress in middle-aged and older adults with spina bifida myelomeningocele.PloS One 2024To investigate cognitive functioning and emotional distress in adults aged 55 to 68 years old with spina bifida myelomeningocele (SBM), both with and without...
PURPOSE
To investigate cognitive functioning and emotional distress in adults aged 55 to 68 years old with spina bifida myelomeningocele (SBM), both with and without hydrocephalus. A secondary aim was to explore the associations between psychosocial factors in relation to emotional distress.
MATERIALS AND METHODS
Cross-sectional study of eleven females and eight males with SBM, five with and twelve without hydrocephalus. Cognitive functioning was investigated with neuropsychological tests and self-report measures. Furthermore, participants completed questionnaires regarding resilience, access to social support, coping, and emotional distress. Descriptive statistics were applied, and Spearman Rho correlation coefficients were used to explore the relationships between psychosocial factors and emotional distress.
RESULTS
Eleven exhibited normal cognitive functioning. An observed difference was seen between participants with and without hydrocephalus, where six and five persons reported clinical levels of depression and anxiety, respectively. Positive perceptions of self and future were associated with lower levels of depression and anxiety.
CONCLUSION
This study adds important information about cognitive functioning and emotional distress in an understudied population. The results indicated normal cognitive functioning in adults aged 55 to 68 years with SBM without hydrocephalus. Prevalence of emotional distress was comparable with previous studies of younger adults with SBM. There is a need for longitudinal studies investigating cognition and psychological health to fully capture important aspects of the life course of SBM with and without hydrocephalus.
Topics: Female; Male; Middle Aged; Humans; Aged; Meningomyelocele; Cross-Sectional Studies; Spinal Dysraphism; Psychological Distress; Cognition; Hydrocephalus
PubMed: 38422087
DOI: 10.1371/journal.pone.0298891 -
Beijing Da Xue Xue Bao. Yi Xue Ban =... Aug 2023To summarize the clinical manifestation, classification, and experience of surgical treatment of primary tethered cord syndrome (TCS) in adults.
OBJECTIVE
To summarize the clinical manifestation, classification, and experience of surgical treatment of primary tethered cord syndrome (TCS) in adults.
METHODS
The authors retrospectively analyzed a series of 171 adult patients with primary TCS who were surgically treated under microscope from March 2007 to October 2019. There were 61 males and 110 females whose ages were 18-65 years, with an average age of (39.02±11.81) years. Clinically, the patients presented with various neurological symptoms and signs including lower back and legs pain, reflex changes, sensory disturbances, muscle weakness, and sphincter problems. They were divided into 5 types by clinical manifestations and neuro-imaging features: (1) filum terminale traction in 69 cases, (2) split cord malformation in 21 cases, (3) myelomeningocele in 20 cases, (4) lipomyelomeningocele in 36 cases, and (5) dermal sinus traction in 25 cases. All the patients underwent microsurgery to untether the spinal cord. The patients kept prone position 7 days postoperatively. The Kirollos grading was used to evaluate the outcome of intraoperative untethering. The visual analogue scale (VAS) was used to evaluate the pain, the score of critical muscle strength was used to evaluate the lower extremity motor function, and the Japanese Orthopaedic Association (JOA) sphincter function score was used to evaluate the bladder function.
RESULTS
All of the 171 patients were treated with microsurgery to release the adhesion and cut off the filum terminalis. 61 cases of them received resection of the lesions according to the etiology. All the tethered spinal cord reached Kirollos grade Ⅰ untethering and the dural sac was reconstructed. Other than 5 patients had cerebrospinal fluid leakage and incision laceration and underwent re-suture, there was no surgical complication. The local pain was relieved, the lower limbs weakness or bowel and bladder dysfunction gradually recovered postoperatively. The period of follow-up ranged from 6 months to 12.5 years with an average of (5.62±2.31) years. The neurological function was improved in 153 cases and stable in 18 cases. There was no recurrence of tethered cord be found during the follow-up period.
CONCLUSION
The primary TCS in adulthood could be classified into 5 types by clinical manifestations and neuro-imaging features and surgical treatment should be undertaken in regard to the classifications including dissection and resection of the lesion detethering the spinal cord and reconstruction of the dura sac under microscope. The outcome of surgical treatment is satisfactory.
Topics: Male; Female; Humans; Adult; Middle Aged; Retrospective Studies; Treatment Outcome; Meningomyelocele; Neural Tube Defects; Pain
PubMed: 37534645
DOI: 10.19723/j.issn.1671-167X.2023.04.012 -
International Braz J Urol : Official... 2023This 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... (Observational Study)
Observational Study
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.
Topics: Female; Child; Humans; Adolescent; Infant; Vesico-Ureteral Reflux; Retrospective Studies; Urinary Bladder, Neurogenic; Cicatrix; Kidney; Urinary Tract Infections
PubMed: 37624657
DOI: 10.1590/S1677-5538.IBJU.2023.0311 -
Cureus Sep 2023Cri-du-chat syndrome (CdCS) is a rare genetic disorder in which the short arm of chromosome 5 is deleted. This report aims to highlight a rare association with the...
Cri-du-chat syndrome (CdCS) is a rare genetic disorder in which the short arm of chromosome 5 is deleted. This report aims to highlight a rare association with the syndrome. We present a preterm male delivered at 35 weeks gestation with an antenatal diagnosis of meningomyelocele. The patient's clinical examination revealed ruptured lumbosacral meningomyelocele, lower limb hypotonia, and hyporeflexia. The patient also displayed dysmorphic features, including microcephaly, a rounded face, low-set ears, and club feet. In addition, he is noted to have a high-pitched cry. Diagnosis of Chiari tonsil hernia type II was made by magnetic resonance imaging, and whole exome sequencing has confirmed CdCS. The spina bifida was surgically corrected, and the patient has since been cared for by a multidisciplinary team. The patient's short-term follow-up revealed a significant developmental delay. Few cases of CdCS associated with meningomyelocele have been reported. More evidence is needed to support a relevant association between CdCS and meningomyelocele.
PubMed: 37908952
DOI: 10.7759/cureus.46279 -
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