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Neurology India 2020
Topics: Child; Child, Preschool; Encephalocele; Failure to Thrive; Humans; Infant; Infant, Newborn; Intellectual Disability; Meningocele; Neurosurgical Procedures; Scalp; Skull
PubMed: 32129233
DOI: 10.4103/0028-3886.279713 -
European Review For Medical and... Aug 2022Encephalocele is a rare congenital neural tube defect (NTD) characterized by herniation of intracranial contents through a defect in the skull. In our study,...
OBJECTIVE
Encephalocele is a rare congenital neural tube defect (NTD) characterized by herniation of intracranial contents through a defect in the skull. In our study, encephalocele was diagnosed in our clinic and its association with hydrocephalus was evaluated. The effect of this association on the prognosis was discussed.
PATIENTS AND METHODS
Patients who underwent surgery and follow-up with the diagnosis of encephalocele in the neurosurgery clinic of our hospital in an 8-year period from 2013 to 2021 were retrospectively examined.
RESULTS
Patient records were obtained from the case notes of patients who underwent excision and repair for encephalocele. Of the 78 patients included in the study, 88.4% underwent surgery in the neonatal period. Moreover, 47% of the patients are male, and 31% are female. Encephalocele was present in 62.8% of patients and meningocele in 37.2%. Furthermore, 82.1% of encephalocele sacs were located in the occipital region. Chiari type 3 malformation was present in 57.6% of patients. Hydrocephalus developed in 56.4% of patients. There was an additional syndrome in 10.3% of the cases. The most common additional syndromes were corpus callosum dysgenesis with 39.7% and colpocephaly with 29.5%. The additional disease was present in 43.6% of patients. Preoperative and postoperative examination findings of more than half of patients were normal, but 33.3% were apathetic. Furthermore, 67.9% of patients, who underwent complete repair, survived, and 32.1% died. Hydrocephalus was present in 73.5% of patients with encephalocele (p<0.05). Hydrocephalus developed in 77.8% of patients with Chiari type 3 malformation (p<0.05). Hydrocephalus was found in 88.0% of patients with Ex (p<0.05).
CONCLUSIONS
Encephalocele, which is a subgroup of NTD, differs clinically by its location and accompanying additional anomalies. In encephaloceles, the risk of morbidity and mortality can only be reduced with the multidisciplinary approach. Hydrocephalus and Chiari type 3 malformation are common in patients with encephalocele. These associations adversely affect the prognosis of the disease. Further research should be conducted on the evaluation of risk factors of NTD and methods of prevention from NTD. In this regard, we recommend that the training be repeated at certain intervals and that people's awareness should be raised.
Topics: Encephalocele; Female; Humans; Hydrocephalus; Infant, Newborn; Male; Neural Tube Defects; Prognosis; Retrospective Studies
PubMed: 35993634
DOI: 10.26355/eurrev_202208_29407 -
The Pan African Medical Journal 2022
Topics: Anal Canal; Digestive System Abnormalities; Humans; Magnetic Resonance Imaging; Meningocele; Rectum; Sacrum; Syringomyelia
PubMed: 35519164
DOI: 10.11604/pamj.2022.41.143.33419 -
Children (Basel, Switzerland) Nov 2020Caudal Regression Syndrome (CRS) or Caudal dysgenesis syndrome (CDS) is characterized by maldevelopment of the caudal half of the body with variable involvement of the...
Caudal Regression Syndrome (CRS) or Caudal dysgenesis syndrome (CDS) is characterized by maldevelopment of the caudal half of the body with variable involvement of the gastrointestinal, genitourinary, skeletal, and nervous systems. CRS affects 1-3 newborn infants per 100,000 live births. The prevalence in infants of diabetic mothers is reported at 1 in 350 live births which includes all the variants. A related condition is sirenomelia sequence or mermaid syndrome or symmelia and is characterized by fusion of the legs and a variable combination of the other abnormalities. The Currarino triad is a related anomaly that includes anorectal atresia, coccygeal and partial sacral agenesis, and a pre-sacral lesion such as anterior meningocele, lipoma or dermoid cyst. A multidisciplinary management approach is needed that includes rehabilitative services, and patients need a staged surgical approach.
PubMed: 33158301
DOI: 10.3390/children7110211 -
SAGE Open Medical Case Reports 2022The occipital bone is an uncommon location for meningoceles protrusion. This condition occurs generally after a severe traumatism or surgical procedure. However, in some...
The occipital bone is an uncommon location for meningoceles protrusion. This condition occurs generally after a severe traumatism or surgical procedure. However, in some rare cases, the herniation can happen spontaneously. Nontraumatic clival meningoceles present an extremely rare entity and correspond to a herniating pachymeningeal collection containing cerebrospinal fluid through a zone of fragility in the clivus. Clinical presentation ranges from simple headache or rhinorrhea to severe complications such as recurrent bacterial meningitis or nerve compression. Computed tomography provides an analysis of the bone and magnetic resonance imaging provides a superior contrast resolution, helping to distinguish among the various types of clival lesions. We report the case of a young woman with a long history of idiopathic intracranial hypertension, who presented with a worsening headache. Magnetic resonance imaging confirmed a clival meningocele without other complications and the patient was put under medical surveillance.
PubMed: 35966125
DOI: 10.1177/2050313X221117334