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British Journal of Neurosurgery Aug 2023Early closure of the meningocele, meningomyelocele, is essential to prevent complications such as hypothermia, infection in the postnatal period. But for some reasons,... (Review)
Review
BACKGROUND
Early closure of the meningocele, meningomyelocele, is essential to prevent complications such as hypothermia, infection in the postnatal period. But for some reasons, it is not always possible to repair early. The possibility of malign transformation should be considered in long-term due to nonhealing ulcers and cerebrospinal fluid (CSF) fistula and frequent infections in an adult patient with meningomyelocele. The possibility of conversion to malignancy in the control of an untreated meningomyelocele patient with (CSF) drainage should be especially kept in mind.
CASE DESCRIPTION
A case is described of a malignant transformation at the site of a myelomeningocele in a 22-year-old woman. The lumbosacral region was deformed by a large meningomyelocele 10 cm in diameter, with areas of scar and ulcer. For 7 years prior to this presentation, the lesion was a large fistula in the roof of the open myelomeningocele. She underwent continuous CSF draining. A biopsy was done which revealed it as squamous cell carcinoma.
CONCLUSION
Early closure of meningocele, meningomyelocele is essential to prevent complications such as hypothermia, infection in the postnatal period. Also, especially if there is continuous CSF exposure, the possibility of malignant change should be kept in mind in neglected cases presenting in an adult.
Topics: Adult; Female; Humans; Young Adult; Meningomyelocele; Meningocele; Hypothermia; Plastic Surgery Procedures; Carcinoma, Squamous Cell
PubMed: 30652927
DOI: 10.1080/02688697.2018.1554178 -
Nature Reviews. Disease Primers Apr 2015Spina bifida is a birth defect in which the vertebral column is open, often with spinal cord involvement. The most clinically significant subtype is myelomeningocele... (Review)
Review
Spina bifida is a birth defect in which the vertebral column is open, often with spinal cord involvement. The most clinically significant subtype is myelomeningocele (open spina bifida), which is a condition characterized by failure of the lumbosacral spinal neural tube to close during embryonic development. The exposed neural tissue degenerates in utero, resulting in neurological deficit that varies with the level of the lesion. Occurring in approximately 1 per 1,000 births worldwide, myelomeningocele is one of the most common congenital malformations, but its cause is largely unknown. The genetic component is estimated at 60-70%, but few causative genes have been identified to date, despite much information from mouse models. Non-genetic maternal risk factors include reduced folate intake, anticonvulsant therapy, diabetes mellitus and obesity. Primary prevention by periconceptional supplementation with folic acid has been demonstrated in clinical trials, leading to food fortification programmes in many countries. Prenatal diagnosis is achieved by ultrasonography, enabling women to seek termination of pregnancy. Individuals who survive to birth have their lesions closed surgically, with subsequent management of associated defects, including the Chiari II brain malformation, hydrocephalus, and urological and orthopaedic sequelae. Fetal surgical repair of myelomeningocele has been associated with improved early neurological outcome compared with postnatal operation. Myelomeningocele affects quality of life during childhood, adolescence and adulthood, posing a challenge for individuals, families and society as a whole. For an illustrated summary of this Primer, visit: http://go.nature.com/fK9XNa.
Topics: Female; Folic Acid; Humans; Meningomyelocele; Pregnancy; Prenatal Care; Prenatal Diagnosis; Spinal Dysraphism
PubMed: 27189655
DOI: 10.1038/nrdp.2015.7 -
Archivos Argentinos de Pediatria Jun 2021A seminal study titled Management of Myelomeningocele Study, from 2011, demonstrated that prenatal myelomeningocele defect repaired before 26 weeks of gestation improved... (Review)
Review
A seminal study titled Management of Myelomeningocele Study, from 2011, demonstrated that prenatal myelomeningocele defect repaired before 26 weeks of gestation improved neurological outcomes; based on this study, fetal surgery was introduced as a standard of care alternative. Thus, prenatal myelomeningocele diagnosis within the therapeutic window became a mandatory goal; therefore, research efforts on screening strategies were intensified, especially in the first trimester. In addition, different fetal surgery techniques were developed to improve neurological outcomes and reduce maternal risks. The objective of this review is to provide an update on the advances in prenatal screening and diagnosis during the first and second trimesters, and in open and fetoscopic fetal surgery for myelomeningocele.
Topics: Female; Fetoscopy; Humans; Meningomyelocele; Pregnancy; Prenatal Care; Prenatal Diagnosis
PubMed: 34033426
DOI: 10.5546/aap.2021.eng.e215 -
Ciba Foundation Symposium 1994We report a four year follow-up of 39 of 47 infants born after pre-labour Caesarean section and 68 of 79 born vaginally. Loss of motor function due to late complications... (Review)
Review
We report a four year follow-up of 39 of 47 infants born after pre-labour Caesarean section and 68 of 79 born vaginally. Loss of motor function due to late complications was more frequent in the Caesarean section group (Fisher's Exact; P = 0.004). However, the means of the differences between the X-ray levels (measured as the last intact vertebral arch seen on standard anteroposterior roentgenograms of the spinal column) subtracted from the motor levels still favour Caesarean section (mean = 3.24; SD = 2.7) over vaginal delivery (mean = 1.2; SD = 2.7) (Student's t-test; P = 0.0003). The frequencies of other complications, death and neonatal meningitis, were not significantly different. Another 38 infants born by Caesarean section after labour were more paralysed (mean of X-ray and motor difference = 1.8, SD = 2.2) following rupture of amniotic membranes than those with intact amniotic membranes with or without labour (mean = 3.4; SD = 2.2) (Student's t-test; P = 0.0067). The differences between X-ray and motor levels for patients born by Caesarean section with intact amniotic membranes and without labour (mean = 3.6; SD = 2.4) were not significantly different from those with labour and intact amniotic membranes (mean = 2.89; SD = 1.5). The number of new cases of meningomyelocele presenting to our clinic has decreased from an average of 30 per year between 1970 and 1987 to 14 between 1988 and 1992.
Topics: Cesarean Section; Delivery, Obstetric; Extraembryonic Membranes; Female; Follow-Up Studies; Humans; Meningomyelocele; Paralysis; Pregnancy; Prenatal Care; Prenatal Diagnosis
PubMed: 8005029
DOI: 10.1002/9780470514559.ch16 -
Child's Nervous System : ChNS :... Oct 2022Myelomeningocele (MMC) is the most frequent neural tube defect and is frequently associated (around 80% of cases) with hydrocephalus (HC). Both diseases can have severe... (Review)
Review
INTRODUCTION
Myelomeningocele (MMC) is the most frequent neural tube defect and is frequently associated (around 80% of cases) with hydrocephalus (HC). Both diseases can have severe clinical consequences, insomuch as they require surgical treatment whose complications are not negligible, either when performed in utero or after birth. Therefore, clinical therapies that could have an impact on the incidence and progression of MMC and HC would be certainly valuable; however, this is not the current picture, and there are no effective pharmacological treatments for such patients to this day.
AIM AND METHODS
Therefore, knowing that an inflammatory process comes associated with these disorders, mostly due to nervous tissue distension, the present article aimed at reviewing the role of corticosteroids in reducing inflammation and thus improving the outcome of patients with HC and MMC, considering the well-established anti-inflammatory effects of CS.
RESULTS
The systematic review performed herein has found varying results regarding the role of steroids (even though a positive trend was observed) on the treatment and prevention of hydrocephalus, whereas for MMC.
CONCLUSION
There are many reports demonstrating beneficial effects of CS therapy, from a clinical and histopathological point of view.
Topics: Adrenal Cortex Hormones; Anti-Inflammatory Agents; Central Nervous System; Humans; Hydrocephalus; Meningomyelocele; Retrospective Studies; Ventriculoperitoneal Shunt
PubMed: 35907004
DOI: 10.1007/s00381-022-05615-3 -
Pediatrician 1988Failure of the neural tube to close in a fetus is one of the most devastating and yet common congenital malformations. Various organ systems including the skeletal,... (Review)
Review
Failure of the neural tube to close in a fetus is one of the most devastating and yet common congenital malformations. Various organ systems including the skeletal, muscular, urologic, digestive, respiratory, skin and central nervous systems are involved. This involvement results in complicated and interrelated problems requiring services from the medical specialties of pediatrics, orthopedics, urology and neurosurgery; the allied health professions of physical therapy, occupational therapy, speech pathology, nutrition, social work and nursing; and the psychoeducational professions of education and psychology. This article attempts to summarize the information about the current needs and management techniques of children with meningomyelocele for the general pediatrician who is frequently called upon to play an important role in coordination and communication with the families and other professionals caring for these children.
Topics: Bone Diseases; Child, Preschool; Family; Female; Gastrointestinal Diseases; Humans; Male; Meningomyelocele; Pregnancy; Prenatal Care; Urologic Diseases
PubMed: 3050917
DOI: No ID Found -
Child's Nervous System : ChNS :... Jul 2021Teratomas are benign germ cell tumors originating from at least two germ layers, mostly of ectodermal and mesodermal origin. Mature teratomas are the most common subtype...
Teratomas are benign germ cell tumors originating from at least two germ layers, mostly of ectodermal and mesodermal origin. Mature teratomas are the most common subtype and develop from well-differentiated germ cells. Although the location is extragonadal in infants and young children, gonadal involvement occurs in adults. Midline defects can be diagnosed on prenatal imaging. In this case report, a newborn with mature cystic teratoma and a prenatal lumbar midline closure defect was presented. The perinatal preliminary diagnosis was meningomyelocele. However, a cystic sac containing exophytic solid tumoral tissues approximately 5 × 5 × 3 cm in size was seen macroscopically in the lumbar region after the birth, and this tumor was totally resected. After tumor excision, spina bifida aperta and vertebral exophytic bony tissue compatible with diastematomyelia were observed at the bottom of the surgical field and were totally resected. In the short-term follow-up, no additional problem occurred. The histopathological diagnosis was "mature cystic teratoma." In conclusion, extragonadal teratoma accompanying diastematomyelia could easily be mistaken for meningomyelocele or other common malformations. Perinatal diagnosis should be provided using radiodiagnostic methods, and total surgical excision and accurate pathological diagnosis are essential to avoid the risk of recurrence.
Topics: Child, Preschool; Dermoid Cyst; Humans; Meningomyelocele; Neoplasm Recurrence, Local; Spine; Teratoma
PubMed: 33404722
DOI: 10.1007/s00381-020-05017-3 -
European Journal of Pediatric Surgery :... Dec 1997This paper describes the epidemiology of tethered cord syndrome and its etiologies and co-morbidities following initial repair of both meningomyeloceles and... (Review)
Review
This paper describes the epidemiology of tethered cord syndrome and its etiologies and co-morbidities following initial repair of both meningomyeloceles and lipomeningomyelocele. A review of the pertinent literature and data from 654 cases of meningomyelocele and 118 cases of lipomeningomyelocele has been drawn from a computerized database, Patient Data Management System/fx. Only cases born since 1964 were analyzed for the etiologies, co-morbidities, spinal cord abnormalities detected by contrast studies or MRI and for significant symptoms and signs. Tethered cord symptoms were related to an attachment to a rigid tether for all 31 cases following lipomeningomyelocele repair but 62 (75%) of the 83 post meningomyelocele repair patients developed the symptoms of tethered cord. Causes other than, or in addition to, tethering included an obstructed cerebrospinal fluid shunt, syringohydromyelia, benign tumor and spinal cord hypoplasia. Quantitative differentiation between asymptomatic thin spinal cords and symptomatic spinal cord hypoplasia as well as between central canal enlargement and symptomatic syringohydromyelia could not be demonstrated. Collaborative, multi-center studies of larger numbers of patients are recommended.
Topics: Comorbidity; Diagnosis, Differential; Humans; Meningomyelocele; Prevalence; Spina Bifida Occulta
PubMed: 9497108
DOI: 10.1055/s-2008-1071200 -
Der Urologe. Ausg. A Feb 2015The treatment of children and adolescents with meningomyelocele has experienced a clear change in the last 30 years. The establishment of pharmacotherapy, clean... (Review)
Review
The treatment of children and adolescents with meningomyelocele has experienced a clear change in the last 30 years. The establishment of pharmacotherapy, clean intermittent catheterization (CIC) and infection prophylaxis have improved the prognosis for patients and have led to new therapeutic strategies. The interdisciplinary cooperation between neonatologists, neurosurgeons, pediatric neurologists, pediatric urologists, pediatric nephrologists, pediatric orthopedists and pediatric surgeons leads to optimization of individualized therapy. These guidelines present definitions and classifications, investigations and timing which are described in detail. The conservative and operative therapy options for neurogenic bladder function disorders are described and discussed with reference to the current literature. The brief overview provides in each case assistance for the treating physician in the care of this patient group and facilitates the interdisciplinary cooperation.
Topics: Adolescent; Child; Child, Preschool; Diagnostic Techniques, Urological; Female; Germany; Humans; Infant; Infant, Newborn; Male; Meningomyelocele; Practice Guidelines as Topic; Urinary Bladder, Neurogenic; Urology
PubMed: 25690576
DOI: 10.1007/s00120-013-3403-2 -
Pediatrics in Review Nov 2010
Review
Topics: Continuity of Patient Care; Female; Humans; Infant, Newborn; Meningomyelocele; Patient Care Team; Pregnancy; Social Support; Ultrasonography, Prenatal; alpha-Fetoproteins
PubMed: 21041422
DOI: 10.1542/pir.31-11-443