Multiple dysraphic anomalies with double thoracic meningocele and lumbosacral myelomeningocele, concurrent Chiari malformation Type I, diastematomyelia, lipomyelomeningocele and hydrocephalus: a case report and literature review (original) (raw)
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Pakistan Journal Of Neurological Surgery, 2022
Objective: Neural tube defects (NTDs) are common in northern areas of Khyber Pakhtunkhwa (KPK) and need a lot of community education for the parents regarding this disease, which impaired the patients for their whole life. The study aimed to assess the contribution of a family history of myelomeningocele and the resulting incidence of Chiari II malformation. Materials and Methods: A total of 131 patients were observed to determine the frequency of the Chiari II malformation in patients with myelomeningocele who presented in Ayub Teaching Hospital, Abbottabad. All neonates were sent to the radiology department for MRI. A repair procedure for meningomyelocele was done. Results: The mean age was 16.56 days. In 53.4% of neonates, there was a familial history of spinal dysraphism, while in 46.6% there was no familial history. Chiari II malformation was present in 23.7% of patients who presented with myelomeningocele. A significant difference (p-value < 0.00001) existed between the presence/absence of a family history of myelomeningocele and Chiari II malformation out of the total. Conclusion: Early surgery, along with a multidisciplinary approach, provides the best opportunity for improved results and survival.
Asian journal of neurosurgery, 2023
Background Chiari malformation type II (CM-II) may not always present as an asymptomatic disorder but prove to be difficult in managing. This is especially true for neonates who show the worst prognosis. There is confounding data over whether shunting or craniocervical junction (CVJ) decompression should be employed. This retrospective analysis summarizes the results of 100 patients diagnosed and treated for CM-II along with hydrocephalus and myelomeningocele. Methods We reviewed all the children who were diagnosed and surgically treated for CM-II at the Moscow Regional Hospital. Surgical timing was decided on the clinical conditions of each patient. Urgent surgery in the more compromised patients (usually infants) and elective surgery for patients with less severe conditions was performed. All patients first underwent CVJ decompression. Results The retrospective review yielded 100 patients operated on for CM-II with concomitant hydrocephalus and myelomeningocele. The average herniation was 11.2 AE 5.1 mm. However, herniation level did not correlate with clinical findings. Concomitant syringomyelia was observed in 60% of patients. More severe spinal deformity was observed in patients with widespread syringomyelia (p ¼ 0.04). In children of the younger age group, cerebellar symptoms and bulbar disorders were more frequently observed (p ¼ 0.03), and cephalic syndrome was noted much less frequently (p ¼ 0.005). The severity of scoliotic deformity correlated with the prevalence of syringomyelia (p ¼ 0.03). Satisfactory results were significantly more often observed in patients of the older age group (p ¼ 0.02). Patients with unsatisfactory results at the time of treatment were significantly younger (p ¼ 0.02). Conclusion If CM-II is asymptomatic, then no specific treatment is prescribed. If the patient develops pain in the occiput and neck, then pain relievers are prescribed. If a
Myelomeningocele with Associated Anomalies Case Report and Literature Review
Folia Medica
Myelomeningocele is a common defect of the development of the neural tube. It is a complex congenital malformation of the central nervous system (CNS) that can be associated with other concurrent anomalies. We report on a case of lumbar myelomeningocele with concomitant CNS malformations we followed up over a period of 15 years. A concise literature review has also been performed. The current report illustrates that the myelomeningocele is a complex anomaly that is commonly associated with a variety of other CNS malformations such as hydrocephalus and Chiari malformation. It may follow chronic progressive course with exacerbation of clinical symptoms in the long term. Patients that have undergone surgical correction of this spinal defect should be closely monitored over a long period of time because of the possibility of clinical deterioration of the concomitant anomalies such as hydrocephalus, Chiari malformation and siryngomyelia.
Pediatric Neurosurgery, 2008
Objective: Meningomyelocele (MMC) is a common central nervous system birth defect. Various congenital and acquired abnormalities have been reported with MMC, some of which are secondary to the pathophysiology and some are morbidities of the underlying disease. The aim of this study was to discuss current possible theories explaining diverse anomalies/abnormalities seen in a series of 390 patients with MMC. Methods: A retrospective study was performed using the records of 390 patients with MMC at Children’s Hospital Medical Center in Tehran, Iran, from January 2001 to January 2007. A series of 17 cases of MMC with attributed organ anomalies, not explained by a causal effect of the underlying disorder, were compiled. There were 3 cardiac anomalies including ventricular septal defect, pulmonary artery atresia and tetralogy of Fallot, 4 musculoskeletal malformations, consisting of missing rib, polydactylia and complex distal limb anomaly, 4 urological anomalies such as bladder exstrophy...
Journal of Pediatrics & Neonatal Care, 2018
Background: Congenital anomaly is one of the most common causes of perinatal death throughout the world. Consanguinity, nutritional deficiency, infection during pregnancy, malaria etc. are most prevalent in Bangladesh. The study was aimed to explore the prevalence and types of congenital malformations along with their immediate outcome. Materials and Methods: This observational study was conducted among all the admitted high risk pregnancies who were admitted with prenatally diagnosed congenital anomalies from January, 2015 to December, 2015. During this tenure 705 high risk patients were admitted among which 125 patients had prenatally diagnosed congenital anomalous fetus and included for the study to explore the prevalence and types of congenital malformations and their immediate outcome. For the study purpose, congenital anomalies were broadly classified into major and minor groups. Major anomalies were considered when the defects seemed to cause stillbirth or neonatal death or needed medical termination for lethal anomalies (anencephaly, multiple congenital anomalies) or severe anomalies that without medical intervention would cause handicap or death. Mild anomalies/defects were considered which might require medical intervention but compatible with life. Results: The mean of gestational age at the time of delivery was 31.77± 6.23(SD) wks. 125 (17.7%) women had congenital anomaly of their baby. Among these anomalies central nervous system anomalies was the most frequent (34.4%), followed by renal anomalies (22.4%) and others in order of frequency were gastro-intestinal, skeletal, non-immune hydrops, cardiac anomalies. The major anomalies were 81.6% and multiple congenital anomalies were in 28.8%. Among the congenital anomalous fetus, perinatal death was 43.2% and appeared to be the most common cause of death in new borne babies. Conclusion: In this study, Central nervous system (CNS) anomalies were found the commonest congenital anomalies and pointing to the risk of burden of these anomalies on the future handicapping condition and have their impact on social and economical consequences. So, emphasis is needed on prenatal diagnosis of congenital anomalies by routine scanning for anomalies by standard ultrasonograph, so that opportunity of secondary prevention can be considered in fatal cases and appropriate treatment can be ensured immediately after birth. Emphasis should also need in the use of fetal echocardiograph for prenatal detection of cardiac anomalies.
Fetal Diastematomyelia: Rare Case
IOSR Journal of Dental and Medical Sciences, 2012
A ten-month-old infant with Diastematomyelia, as a very rare variety of spinal dysraphism is presented in this article Routine USG in a 36 weeks pregnant woman revealed a spinal abnormality of the fetus in thoracolumbar [D12-L1] regions. At birth, examination of the newborn showed a small midline swelling and bluish discoloration of skin and scoliosis in the lumbar region. There was associated umbilical hernia.no neurological deficit was noted. Postnatally 8 month plane radiograph showed multiple deformed dorsal vertebrae and mild scoliosis.MR imaging revealed diastematomyelia was seen at thoracolumbar [D12-L1] regions. It was type I with a bony spur measuring 1.0 X0.5 cm the two hemicords were asymmetrical. There was no cord tethering. The conus ended at the L4 level.. There was associated thoracolumbar spina bifida measuring 1.6 cm with a small meningocele measuring 2.5 X 0.6 cm. multilevel syringohydromelia and multiple deformed dorsal vertebrae and mild scoliosis of dorsal spine was seen. Thus what makes this case different is inspite of having so many microanatomical defects, there is no neurologic deficit. So its advisible to do corrective surgery to avoid further complicatiions-thethered cord syndrome before the baby starts to take steps.
2007
Reported is a unique case of Chiari imalforma tion with syringomyelia and medullar hemorrhage, which became symptomatic 4 days after a craniocervical trauma when the patient was doing neck exercises. The patient manifested respiratory arrest, tetraparesis, and persistent lower cranial nerve dysfunction. Magnetic resonance imaging studies revealed a Chiari i ma!formation with a large cervicothoracic syringomyelia defect and medullar hemorrhage. The patient underwent surgery but some symptoms persisted after the operation. Type i Chiari ma!formation should be included in the differential diagnosis of patients who present with extremity weakness (upper or lower), lower cranial nerve palsy, or respiratory arrest after trauma or neck exercises.
The Association of Hydrocephalus and Arnold-Chiari Malformation with Spina Bifida in the Fetus
Neuropathology and Applied Neurobiology, 1980
Twenty-one fetuses with spina bifida and twenty apparently normal fetuses of between 14-23 weeks gestation were examined for the presence of hydrocephalus and Arnold-Chiari malformation. Sacral, lumbo-sacral, lumbar, thoraco-lumbo-sacral and thoraco-lumbar lesions were found. Some were closed, some were open. The amount of neural tissue at the site of the lesion was very variable. In three cases, diastematomyelia was found. The heads of spina bifida fetuses were not enlarged. Hydrocephalus was found in eleven of them and the Arnold-Chiari malformation in twelve. In nine fetuses the abnormalities co-existed. Hydrocephalic brains showed depression of the tentorium, abnormalities in the corpus callosum and absence of the lateral fissure. The more cephalad and extensive the spinal lesion, the more likely it was to be accompanied by brain abnormalities. Isolated sacral defects were not associated either with hydrocephalus or with the Arnold-Chiari malformation. Pathological changes in the brain occurred in the presence of both open and closed spinal lesions and were apparently unrelated to fetal age. No evidence was found as to the sequence in which these brain abnormalities appeared.