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2.
J Pak Med Assoc ; 74(8): 1541-1544, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-39160732

RESUMEN

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.


Asunto(s)
Meningomielocele , Procedimientos de Cirugía Plástica , Colgajos Quirúrgicos , Humanos , Meningomielocele/cirugía , Masculino , Femenino , Procedimientos de Cirugía Plástica/métodos , Lactante , Pérdida de Líquido Cefalorraquídeo/cirugía , Pérdida de Líquido Cefalorraquídeo/etiología , Complicaciones Posoperatorias
3.
Neurosurg Rev ; 47(1): 343, 2024 Jul 20.
Artículo en Inglés | MEDLINE | ID: mdl-39031287

RESUMEN

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.


Asunto(s)
Hidrocefalia , Meningomielocele , Complicaciones Posoperatorias , Derivación Ventriculoperitoneal , Humanos , Meningomielocele/cirugía , Meningomielocele/complicaciones , Hidrocefalia/cirugía , Femenino , Derivación Ventriculoperitoneal/efectos adversos , Recién Nacido , Masculino , Complicaciones Posoperatorias/epidemiología , Estudios Prospectivos , Resultado del Tratamiento , Pérdida de Líquido Cefalorraquídeo , Procedimientos Neuroquirúrgicos/métodos , Procedimientos Neuroquirúrgicos/efectos adversos , Lactante
4.
Acta Neurochir (Wien) ; 166(1): 270, 2024 Jun 17.
Artículo en Inglés | MEDLINE | ID: mdl-38884665

RESUMEN

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.


Asunto(s)
Vértebras Lumbares , Vértebras Torácicas , Humanos , Vértebras Torácicas/cirugía , Vértebras Torácicas/diagnóstico por imagen , Lactante , Vértebras Lumbares/cirugía , Meningomielocele/cirugía , Procedimientos Neuroquirúrgicos/métodos , Resultado del Tratamiento , Masculino , Disrafia Espinal/cirugía , Imagen por Resonancia Magnética
5.
J Neurosurg Pediatr ; 34(3): 207-215, 2024 Sep 01.
Artículo en Inglés | MEDLINE | ID: mdl-38905709

RESUMEN

OBJECTIVE: The aim of this study was to compare clinical and craniometric outcomes of patients treated for hydrocephalus following fetal myelomeningocele repair (fMMR) via a ventriculoperitoneal shunt (VPS) or endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC). METHODS: This was a retrospective cohort study of children who were treated for hydrocephalus following fMMR via VPS or ETV with or without CPC (ETV ± CPC) at Vanderbilt between 2012 and 2021. The primary outcomes were treatment failure and time to failure (TTF). Secondary outcomes included changes in hydrocephalus metrics (fronto-occipital horn ratio [FOHR] and head circumference measurements) and healthcare resource utilization (number of hospital admissions, clinic visits, and neuroimaging findings). RESULTS: Among 88 patients who underwent fMMR, 37 (42%) required permanent CSF diversion, of whom 19 received treatment at the authors' institution. Twelve patients underwent ETV ± CPC, and 7 underwent VPS placement at a median corrected age of 23 weeks versus 1 week (p = 0.002). The preoperative median head circumference percentiles and z-scores for patients in the ETV ± CPC cohort were similar to those of the VPS cohort (percentiles: 98.5 vs 94.0, p = 0.064; z-scores: 2.32 vs 1.60, p = 0.111). There was no difference in preoperative median FOHR measurements between the two cohorts (0.57 vs 0.59, p = 0.53). At 6 months postoperatively, the median head circumference percentile and z-score for the ETV ± CPC cohort remained similar between the two cohorts (percentiles: 98.0 vs 67.5, p = 0.315; z-scores: 2.12 vs 0.52, p = 0.307). There was no difference in the change in FOHR (-0.06 vs -0.09, p = 0.37) and change in head circumference percentile (-1.33 vs -28.6, p = 0.058) between the cohorts 6 months after the index CSF diversion procedure. One patient in the ETV ± CPC cohort experienced a seizure and a nonoperative subdural hemorrhage postoperatively; no other complications were observed. Six of the 7 patients in the VPS cohort required shunt revision with a median TTF of 9.8 months while 2 of the 12 ETV ± CPC patients required a repeat ETV at a median of 17.5 months (86% vs 17%, p = 0.013). The median number of hydrocephalus-related hospital readmissions was significantly lower in the ETV ± CPC cohort than in the VPS cohort (0 vs 1, p = 0.006). The ETV ± CPC cohort had fewer CT scans (0 vs 2, p = 0.004) and radiographs (0 vs 2, p < 0.001) than the VPS cohort. CONCLUSIONS: In a single-center cohort, hydrocephalic fMMR patients treated via ETV ± CPC remained shunt free, while a majority of patients receiving an upfront shunt required revision. This is the first study comparing ETV ± CPC with VPS in the fMMR hydrocephalus population. While larger, multicenter studies are needed, these results suggest that ETV/CPC may be a preferred means of CSF diversion following fMMR.


Asunto(s)
Cauterización , Plexo Coroideo , Hidrocefalia , Meningomielocele , Tercer Ventrículo , Derivación Ventriculoperitoneal , Ventriculostomía , Humanos , Meningomielocele/cirugía , Meningomielocele/complicaciones , Hidrocefalia/cirugía , Hidrocefalia/etiología , Ventriculostomía/métodos , Plexo Coroideo/cirugía , Femenino , Masculino , Estudios Retrospectivos , Tercer Ventrículo/cirugía , Cauterización/métodos , Recién Nacido , Neuroendoscopía/métodos , Resultado del Tratamiento , Lactante , Estudios de Cohortes
6.
Childs Nerv Syst ; 40(9): 2859-2863, 2024 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-38847879

RESUMEN

INTRODUCTION: Myelomeningocele (MMC) is a prevalent form of neural tube defect. Despite advancements in treatment, MMC still poses significant health risks, including complications leading to chronic disability and mortality. Identifying prognostic risk factors for early outcomes is crucial for tailored intervention strategies. METHODS: This prospective study involved newborns and infants diagnosed with MMC who underwent surgery between 2020 and 2023 at Urmia University of Medical Sciences. Demographic data and surgical outcomes were collected, and participants were followed up for six months. Statistical analyses were conducted using descriptive statistics, Chi-Square, and independent t-test. RESULTS: The study included 29 MMC cases, with an incidence rate of 1.4 per 10,000 live births. Lesions were predominantly located in the lumbar spine. Although mortality rates appeared to increase with ascending lesion sites, this trend was not statistically significant. Short-term outcomes revealed high morbidity and mortality rates, with neurological deficits being the most prevalent complication. Multivariable analysis identified head circumference as a significant predictor of adverse outcomes (IRR = 1.37, 95% CI = 1.02 to 1.86, p = 0.04). Furthermore, an increase in birth weight was associated with a reduction in the incidence of requiring a ventriculoperitoneal shunt (IRR = 0.99, 95% CI = 0.998 to 0.999, p = 0.02). CONCLUSION: This prospective study highlights prognostic risk factors for early outcomes in MMC patients, emphasizing the need for personalized intervention strategies. By addressing modifiable risk factors and implementing targeted interventions, healthcare providers can strive to improve outcomes and enhance the quality of life for MMC patients.


Asunto(s)
Meningomielocele , Humanos , Meningomielocele/cirugía , Meningomielocele/complicaciones , Factores de Riesgo , Estudios Prospectivos , Femenino , Masculino , Pronóstico , Recién Nacido , Lactante , Resultado del Tratamiento
7.
Rev Assoc Med Bras (1992) ; 70(6): e20231327, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38896734

RESUMEN

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.


Asunto(s)
Meningomielocele , Madres , Telemedicina , Humanos , Meningomielocele/cirugía , Proyectos Piloto , Femenino , Adulto , Estudios Retrospectivos , Encuestas y Cuestionarios , Embarazo , Recién Nacido , Relaciones Familiares , Edad Gestacional
8.
Neurosurg Rev ; 47(1): 292, 2024 Jun 25.
Artículo en Inglés | MEDLINE | ID: mdl-38914693

RESUMEN

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.


Asunto(s)
Defectos del Tubo Neural , Recuperación de la Función , Caminata , Humanos , Masculino , Femenino , Caminata/fisiología , Niño , Recuperación de la Función/fisiología , Estudios Retrospectivos , Defectos del Tubo Neural/cirugía , Preescolar , Adolescente , Meningomielocele/cirugía , Resultado del Tratamiento , Procedimientos Neuroquirúrgicos/métodos
9.
Childs Nerv Syst ; 40(9): 2851-2858, 2024 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-38714605

RESUMEN

INTRODUCTION: Most myelomeningocele (MMC) cases present with ventriculomegaly or hydrocephalus, yet a comprehensive volumetric assessment of MMC intracranial structures is lacking. This study aimed to provide baseline data on intracranial structural volumes immediately after birth in MMC infants who underwent repair surgeries after birth (postnatal repair). METHODS: In this retrospective single-center study, we analyzed 52 MMC infants undergoing postnatal repair, utilizing head computed tomography scans at birth for volumetric assessment. Intracranial volume (ICV), lateral ventricles volume (LVV), choroid plexus volume (CPV), and posterior cranial fossa volume (PCFV) were measured. Hydrocephalus was classified into no hydrocephalus, progressive hydrocephalus, and hydrocephalus at birth. Comparative analysis employed the Wilcoxon rank-sum test. Receiver operating characteristic (ROC) analysis discriminated cases with and without ventriculoperitoneal shunt (VPS). RESULTS: The median values were 407.50 mL for ICV, 33.18 mL for LVV, 0.67 mL for CPV, and 21.35 mL for PCFV. Thirty-seven cases (71.15%) underwent VPS. ROC analysis revealed an LVV cut-off value of 6.74 mL for discriminating cases with and without VPS. Progressive hydrocephalus showed no significant difference in ICV but significantly larger LVV compared to no hydrocephalus. Hydrocephalus at birth demonstrated statistically larger ICV and LVV compared to the other two types. CONCLUSION: Baseline volumetric data were provided, and volumetric analysis exhibited statistical differences among three hydrocephalus types. These findings enhance our understanding of intracranial volumetric changes in MMC, facilitating more objective assessments of MMC cases.


Asunto(s)
Hidrocefalia , Meningomielocele , Humanos , Meningomielocele/cirugía , Meningomielocele/diagnóstico por imagen , Femenino , Masculino , Estudios Retrospectivos , Hidrocefalia/cirugía , Hidrocefalia/diagnóstico por imagen , Hidrocefalia/etiología , Recién Nacido , Lactante , Derivación Ventriculoperitoneal/métodos , Tomografía Computarizada por Rayos X/métodos , Encéfalo/diagnóstico por imagen , Encéfalo/patología , Plexo Coroideo/diagnóstico por imagen , Plexo Coroideo/cirugía
10.
Adv Tech Stand Neurosurg ; 49: 95-122, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38700682

RESUMEN

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.


Asunto(s)
Meningomielocele , Procedimientos Neuroquirúrgicos , Humanos , Meningomielocele/cirugía , Procedimientos Neuroquirúrgicos/métodos
12.
BMC Pediatr ; 24(1): 350, 2024 May 22.
Artículo en Inglés | MEDLINE | ID: mdl-38773409

RESUMEN

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.


Asunto(s)
Defectos del Tubo Neural , Humanos , Recién Nacido , Estudios Transversales , Estudios Retrospectivos , Etiopía/epidemiología , Defectos del Tubo Neural/cirugía , Femenino , Masculino , Procedimientos Neuroquirúrgicos/estadística & datos numéricos , Procedimientos Neuroquirúrgicos/métodos , Resultado del Tratamiento , Hidrocefalia/cirugía , Hospitales Especializados/estadística & datos numéricos , Meningomielocele/cirugía , Meningomielocele/complicaciones
13.
Childs Nerv Syst ; 40(8): 2521-2526, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-38676719

RESUMEN

PURPOSE: Historically, the presence of gray matter heterotopia was a concern for adverse postnatal neurocognitive status in patients undergoing fetal closure of open spinal dysraphism. The purpose of this study was to evaluate neurodevelopmental outcomes and the onset of seizures during early childhood in patients with a prenatal diagnosis of myelomeningocele/myeloschisis (MMC) and periventricular nodular heterotopia (PVNH). METHODS: All patients evaluated at the Center for Fetal Diagnosis and Treatment with a diagnosis of MMC between June 2016 to March 2023 were identified. PVNH was determined from prenatal and/or postnatal MRI. The Bayley Scales of Infant and Toddler Development (edition III or IV) were used for neurodevelopmental assessments. Patients were screened for seizures/epilepsy. RESULTS: Of 497 patients evaluated with a prenatal diagnosis of MMC, 99 were found to have PVNH on prenatal MRI, of which 35 had confirmed PVNH on postnatal imaging. From the 497 patients, 398 initially did not exhibit heterotopia on prenatal MRI, but 47 of these then had confirmed postnatal PVNH. The presence of PVNH was not a significant risk factor for postnatal seizures in early childhood. The average neurodevelopmental scores were not significantly different among heterotopia groups for cognitive, language, and motor domains. CONCLUSION: The presence of PVNH in patients with a prenatal diagnosis of MMC does not indicate an increased risk for neurodevelopmental delay at 1 year of age. We did not demonstrate an association with seizures/epilepsy. These findings can aid clinicians in prenatal consultation regarding fetal repair of open spinal dysraphism. Long-term follow-up is required to discern the true association between PVNH seen on prenatal imaging and postnatal seizures/epilepsy and neurodevelopmental outcomes.


Asunto(s)
Meningomielocele , Heterotopia Nodular Periventricular , Diagnóstico Prenatal , Convulsiones , Humanos , Femenino , Masculino , Convulsiones/etiología , Convulsiones/diagnóstico por imagen , Meningomielocele/complicaciones , Meningomielocele/cirugía , Meningomielocele/diagnóstico por imagen , Lactante , Preescolar , Embarazo , Diagnóstico Prenatal/métodos , Heterotopia Nodular Periventricular/complicaciones , Heterotopia Nodular Periventricular/diagnóstico por imagen , Heterotopia Nodular Periventricular/cirugía , Imagen por Resonancia Magnética , Trastornos del Neurodesarrollo/etiología , Trastornos del Neurodesarrollo/diagnóstico por imagen , Recién Nacido
14.
J Clin Sleep Med ; 20(10): 1579-1583, 2024 Oct 01.
Artículo en Inglés | MEDLINE | ID: mdl-38661675

RESUMEN

STUDY OBJECTIVES: Advances in prenatal repair of myelomeningocele have improved outcomes involving different organ systems. There are limited data on respiratory outcomes following prenatal surgical repair. We hypothesize there is no difference in respiratory outcomes between patients with spina bifida who have undergone prenatal vs postnatal repair. METHODS: We performed a retrospective study of 46 infants < 1 year with spina bifida seen at Children's Hospital Los Angeles from 2004-2022. Demographic data, timing of closure, neonatal course, Chiari II malformation, ventriculoperitoneal shunt, polysomnography results, and need for supplemental oxygen were collected. Unpaired t test and χ2 test were used to analyze results. RESULTS: A total of 31/46 had prenatal repair of myelomeningocele; average age at repair was 27 weeks postconception. Average age at postnatal repair was 37 weeks postconception. There was no difference in age at polysomnography. There was no difference in Chiari II malformation presence (P = .61). Sixty pecent of patients with postnatal repair and 23% in the prenatal group underwent ventriculoperitoneal shunt placement (P = .01). There was no difference in polysomnography findings between the 2 groups: central apnea index (P = .11), obstructive apnea-hypopnea index (P = .64), average oxygen saturation baseline (P = .91), average oxygen saturation nadir (P = .17), average end-tidal carbon dioxide baseline (P = .87), and average end-tidal carbon dioxide maximum (P = .54). There were no significant differences in the proportion of patients on supplemental oxygen (P = .25), central sleep apnea or obstructive sleep apnea between groups. CONCLUSIONS: Patients with spina bifida who have undergone closure of neural tube defect have persistent central apneas, obstructive apneas, and significant hypoxemia. There were no differences in the frequency or severity of sleep-disordered breathing in those with prenatal repair vs postnatal repair. CITATION: Stark KG, Wang RY, Smith KA. Sleep-related breathing disorders in infants with spina bifida repaired prenatally and postnatally. J Clin Sleep Med. 2024;20(10):1579-1583.


Asunto(s)
Polisomnografía , Disrafia Espinal , Humanos , Estudios Retrospectivos , Femenino , Masculino , Disrafia Espinal/cirugía , Disrafia Espinal/complicaciones , Polisomnografía/métodos , Polisomnografía/estadística & datos numéricos , Lactante , Recién Nacido , Síndromes de la Apnea del Sueño/cirugía , Embarazo , Derivación Ventriculoperitoneal/métodos , Derivación Ventriculoperitoneal/estadística & datos numéricos , Meningomielocele/cirugía , Meningomielocele/complicaciones , Malformación de Arnold-Chiari/cirugía , Malformación de Arnold-Chiari/complicaciones , Resultado del Tratamiento
15.
Childs Nerv Syst ; 40(8): 2593-2598, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-38684568

RESUMEN

A mature cystic teratoma is a mass with heterogeneous appearance, consisting of adult tissue with two or three layers: endoderm, mesoderm, and ectoderm. It is a rare, benign transformation of somatic tissue most commonly found in the sacrococcygeal region and may resemble an uncomplicated spina bifida on prenatal ultrasonography. In this case report, we describe a female newborn with an extremely rare mature cystic teratoma in the thoracolumbar region. She presented prenatally with a preliminary diagnosis of meningomyelocele, diastematomyelia, and Chiari II malformation and a possible teratoma. However, a mass containing solid glandular tissues and bony calcifications approximately 3 × 4 cm in size was observed in the thoracolumbar region upon birth. During surgical resection, no nerve roots were found in the associated meningocele. The patient retained full lower body function postoperatively following surgical excision of the thecal sac and teratoma.


Asunto(s)
Meningomielocele , Teratoma , Humanos , Teratoma/cirugía , Teratoma/diagnóstico por imagen , Meningomielocele/cirugía , Meningomielocele/diagnóstico por imagen , Femenino , Recién Nacido , Diagnóstico Prenatal/métodos , Embarazo , Vértebras Torácicas/diagnóstico por imagen , Vértebras Torácicas/cirugía , Vértebras Lumbares/cirugía , Vértebras Lumbares/diagnóstico por imagen , Adulto , Ultrasonografía Prenatal
16.
J Neurosurg Pediatr ; 34(1): 84-93, 2024 Jul 01.
Artículo en Inglés | MEDLINE | ID: mdl-38608296

RESUMEN

OBJECTIVE: Hydrocephalus is a neurological disorder with an incidence of 80-125 per 100,000 births in the United States. The most common treatment, ventricular shunting, has a failure rate of up to 85% within 10 years of placement. The authors aimed to analyze the association between ventricular catheter (VC) tissue obstructions and shunt malfunction for each hydrocephalus etiology. METHODS: Patient information was collected from 5 hospitals and entered into a REDCap (Research Electronic Data Capture) database by hydrocephalus etiology. The hardware samples were fixed, and each VC tip drainage hole was classified by tissue obstruction after macroscopic analysis. Shunt malfunction data, including shunt revision rate, time to failure, and age at surgery, were correlated with the degree of tissue obstruction in VCs for each etiology. RESULTS: Posthemorrhagic hydrocephalus was the most common etiology (48.9% of total cases). Proximal catheter obstruction was the most frequent cause of hardware removal (90.4%). Myelomeningocele (44% ± 29%), other congenital etiologies (48% ± 40%), hydrocephalus with brain tumors (45% ± 35%), and posthemorrhagic hydrocephalus (41% ± 35%) showed tissue aggregates in more than 40% of the VC holes. A total of 76.8% of samples removed because of symptoms of obstruction showed cellular or tissue aggregates. No conclusive etiological associations were detected when correlating the percentage of holes with tissue for each VC and age at surgery, shunt revision rates, or time between shunt implantation and removal. CONCLUSIONS: The proximal VC obstruction was accompanied by tissue aggregates in 76.8% of cases. However, the presence of tissue in the VC did not seem to be associated with hydrocephalus etiology.


Asunto(s)
Falla de Equipo , Hidrocefalia , Humanos , Hidrocefalia/cirugía , Hidrocefalia/etiología , Masculino , Femenino , Lactante , Derivaciones del Líquido Cefalorraquídeo/efectos adversos , Preescolar , Obstrucción del Catéter/etiología , Derivación Ventriculoperitoneal/efectos adversos , Niño , Recién Nacido , Reoperación/estadística & datos numéricos , Estudios Retrospectivos , Neoplasias Encefálicas/cirugía , Neoplasias Encefálicas/complicaciones , Adolescente , Meningomielocele/complicaciones , Meningomielocele/cirugía
17.
Fetal Diagn Ther ; 51(4): 357-364, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38643759

RESUMEN

INTRODUCTION: No evidence-based protocols exist for fetal cardiac monitoring during fetoscopic myelomeningocele (fMMC) repair and intraprocedural spectral Doppler data are limited. We determined the feasibility of continuous fetal echocardiography during fMMC repair and correlated Doppler changes with qualitative fetal cardiac function during each phase of fMMC repair. METHODS: Patients undergoing fMMC repair had continuous fetal echocardiography interpreted in real-time by pediatric cardiology. Fetal data included fetal heart rate (FHR), qualitative cardiac function, mitral and tricuspid valve inflow waveforms, and umbilical artery (UA), umbilical vein (UV), ductus arteriosus (DA), and ductus venosus (DV) Dopplers. RESULTS: UA abnormalities were noted in 14/25 patients, UV abnormalities were observed in 2 patients, and DV and DA abnormalities were each noted in 4 patients. Qualitative cardiac function was normal for all patients with the exception of one with isolated left ventricular dysfunction during myofascial flap creation, concurrent with an abnormal UA flow pattern. All abnormalities resolved by the first postoperative day. CONCLUSIONS: Continuous fetal echocardiography was feasible during all fMMC repairs. Spectral Doppler changes in the UA were common during fMMC procedures but qualitative cardiac dysfunction was rare. Abnormalities in the UV, DV, and DA Dopplers, FHR, and cardiac function were less common findings.


Asunto(s)
Fetoscopía , Meningomielocele , Humanos , Meningomielocele/cirugía , Meningomielocele/diagnóstico por imagen , Fetoscopía/métodos , Femenino , Embarazo , Ultrasonografía Prenatal/métodos , Monitoreo Fetal/métodos , Corazón Fetal/diagnóstico por imagen , Corazón Fetal/cirugía , Corazón Fetal/fisiopatología , Ecocardiografía Doppler/métodos , Adulto , Estudios de Factibilidad , Arterias Umbilicales/diagnóstico por imagen
18.
World Neurosurg ; 187: e673-e682, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-38685347

RESUMEN

OBJECTIVE: Reports on the management and survival of children with myelomeningocele defects in Bangladesh are limited. This study describes the characteristics and outcomes of these children, focusing on the timing of surgical repair and factors affecting survival. METHODS: We enrolled patients with myelomeningoceles in a case-control study on arsenic exposure and spina bifida in Bangladesh. Cases were subsequently followed at regular intervals to assess survival. Demographic, clinical, and surgical characteristics were reviewed. Univariate tests identified factors affecting survival. RESULTS: Between 2016 and 2022, we enrolled 272 patients with myelomeningocele. Postnatal surgical repair was performed in 63% of cases. However, surgery within 5 days after birth was infrequent (<10%) due to delayed presentation, and there was a high rate (29%) of preoperative deaths. Surgical repair significantly improved patient survival (P < 0.0001). Older age at time of surgery was also associated with improved survival rates, which most likely represents that those who survived to older ages prior to surgery accommodated better with their lesions. Patients who presented with ruptured lesions had lower survival rates. CONCLUSIONS: Timely neurosurgical repair of myelomeningoceles in Bangladesh is hindered by late patient presentation, resulting in a high preoperative patient death rate. Neurosurgical intervention remains a significant predictor of survival. Increased access to neurosurgical care and education of families and non-neurosurgical providers on the need for timely surgical intervention are important for improving the survival of infants with myelomeningoceles.


Asunto(s)
Meningomielocele , Procedimientos Neuroquirúrgicos , Humanos , Bangladesh/epidemiología , Meningomielocele/cirugía , Masculino , Femenino , Procedimientos Neuroquirúrgicos/métodos , Lactante , Estudios de Casos y Controles , Recién Nacido , Preescolar , Tiempo de Tratamiento , Niño , Resultado del Tratamiento , Tasa de Supervivencia
19.
Niger J Clin Pract ; 27(4): 537-540, 2024 Apr 01.
Artículo en Inglés | MEDLINE | ID: mdl-38679779

RESUMEN

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.


Asunto(s)
Meningomielocele , Defectos del Tubo Neural , Humanos , Masculino , Cauda Equina/anomalías , Imagen por Resonancia Magnética , Meningomielocele/complicaciones , Meningomielocele/cirugía , Defectos del Tubo Neural/complicaciones , Defectos del Tubo Neural/cirugía , Médula Espinal/anomalías , Raíces Nerviosas Espinales/anomalías , Lactante
20.
Fetal Pediatr Pathol ; 43(3): 225-233, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38634787

RESUMEN

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.


Asunto(s)
Inmunohistoquímica , Meningomielocele , Disrafia Espinal , Humanos , Disrafia Espinal/patología , Disrafia Espinal/cirugía , Femenino , Inmunohistoquímica/métodos , Meningomielocele/cirugía , Meningomielocele/patología , Meningomielocele/metabolismo , Embarazo , Recién Nacido
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