ABSTRACT
Objective. To review the presentation, diagnosis and management of children with spinal dysraphism and CIT. Methods. It is a retrospective review of 146 children of spina bifida over 9 years (2000-2008) and details the clinical course and outcome of seven with associated congenital inclusion tumors. Results. 7/146 (4.7%) had spina bifida with CIT, 5 dermoid cysts and 2 mature teratoma. The diagnosis was missed by the primary physician even in the presence of a neurocutaneous marker. Spinal imaging with MRI was conclusive. All were managed with multilevel laminectomy, near total/total excision of the CIT and detethering of cord. Intramedullary involvement and established neurological deficits at presentation were associated with persistent deficits. Conclusion. Early detection and comprehensive management of CIT with spinal dysraphism ensures social fecourinary continence, preserves renal function, achieves ambulation and enables patients to lead an acceptable quality of life.
Subject(s)
Adolescent , Child , Child, Preschool , Dermoid Cyst/epidemiology , Dermoid Cyst/pathology , Dermoid Cyst/surgery , Female , Humans , Infant , Infant, Newborn , Laminectomy , Magnetic Resonance Imaging , Male , Retrospective Studies , Spinal Cord Neoplasms/congenital , Spinal Cord Neoplasms/epidemiology , Spinal Dysraphism/epidemiology , Spinal Dysraphism/pathology , Spinal Dysraphism/surgery , Teratoma/epidemiology , Teratoma/pathology , Teratoma/surgeryABSTRACT
Las malformaciones congénitas son un problema poco frecuente; considerando todas las malformaciones en conjunto, éstas se presentan en menos del 2 por ciento de los recién nacidos. Los defectos del cierre del tubo neural: anencefalia, espina bifida, acrania y meningocele, al igual que la mayoría de las malformaciones congénitas, son un grupo de afecciones de etiología multifactorial, producto de la interacción de factores genéticos y ambientales. Los factores genéticos actúan en un sistema poligenético, en el que se tienen que considerar los riesgos de recurrencia, cálculos de heredabilidad, la frecuencia de consanguineidad y las variaciones raciales, los factores ambientales, las infecciones virales, agentes físicos como la hipertemia (fiebre), deficiencia o alteraciones del metabolismo del ácido fólico, así como la exposición a diversas substancias químicas.
Subject(s)
Humans , Adult , Female , Infant, Newborn , Folic Acid/genetics , Congenital Abnormalities/genetics , Neural Tube Defects/genetics , Neural Tube Defects/mortality , Neural Tube Defects/pathology , Embryonic Development/genetics , Spinal Dysraphism/pathology , Fetus/abnormalities , Central Nervous System/embryology , Ultrasonography , Anencephaly/genetics , Anencephaly/mortality , Chemical Compounds/adverse effects , Brain/abnormalities , Gynecology , Misoprostol/pharmacology , Obstetrics , Neural Plate/abnormalitiesABSTRACT
OBJECTIVE: To see the difference in clinical profiles, radiological findings and surgical outcome of the group 1 split cord malformation and meningomyelocele (SCM with MMC) from group 2 (SCM without MMC). METHODS: 46 patients of SCM were selected from a total of 138 cases of spinal dysraphism. They were divided into two groups, based on presence or absence of MMC. Group I (SCM with MMC) n =19 patients and Group II (SCM without MMC) n=27 patients. A detail clinical evaluation and MR screening of whole spine of all cases was performed. All patients underwent surgical detethering of cord. After an average follow-up of 1.7 years, the operative results were clinically assessed and statistical significance was calculated. RESULTS: Male to female ratio was 1:09. Mean age of presentation was 3.6 years. Cutaneous markers like tuft of hair, cutaneous haemangioma, etc, had a higher incidence in group II in comparison to group I (50% vs 10.5%). The incidence of motor deficits was significant in group I in comparison to group II (63% vs 40%). The incidences of sensory loss, trophic ulcers, sphincteric dysfunction and muscle atrophy were relatively more common in group I patients, while neuro-orthopedic deformities such as congenital telepes equinovarus (CTEV), scoliosis and limb shortening were more frequent (67%) in group II children as compared to group I (53%). Type I SCM has higher incidence in group I children. Low lying conus were found in 47% patient of group I, while in group II it was noticed in 69%. The associated cranial anomalies like hydrocephalus, ACM and syrinx, were slightly higher in group I patients. At surgery, dysgenetic nerve roots, neural placode, arachnoid bands and atrophic cord were seen mainly in group I. Postoperative complications like, CSF leak, pseudomeningocele and meningitis were more commonly encountered in group I patients. The patients of group II showed better operative outcome compared to group I cases. CONCLUSION: Incidence of SCM with MMC amount to 41% of total SCM cases. Progressive neurological deficit was higher in this group (SCM with MMC) in comparison to the group harboring SCM without MMC. In view of a significant association of SCM in MMC cases, associated with other craniospinal anomalies, a thorough screening of neuraxis (by MRI) is recommended to treat all treatable anomalies simultaneously for desired outcome.