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Occipital Encephalocele

Cephalocele


Presentation

  • The presentation and management of the case along with and review of the relevant literature is presented.[ncbi.nlm.nih.gov]
  • In the present study, the authors present a series of four such cases.[ncbi.nlm.nih.gov]
  • In the three reported cases, hydrocephalus was not present, concomitant malformations of the lamina terminalis were present in two, and polymicrogyria was found in all three.[ncbi.nlm.nih.gov]
  • The author presented a successful surgical strategy for management of a 4-month-old infant with a large subtorcular occipital encephalocele presented with bilateral vocal cord paralysis and swallowing dysfunction.[ncbi.nlm.nih.gov]
  • RESULTS: In the present study, 30 patients (22 girls and 8 boys), whose ages varied between newborn and 14 months, were evaluated.[ncbi.nlm.nih.gov]
Fishing
  • Pure tetrasomy 20p was confirmed using fluorescent in situ hybridization (FISH) with a telomere probe for chromosome 20p in all seven metaphase cells. The pregnancy was terminated because of associated multiple anomalies and severe oligohydramnios.[ncbi.nlm.nih.gov]
Subcutaneous Nodule
  • Mention should be made of the small, often noncystic subcutaneous nodules that similar to cephaloceles contain meninges with or without brain tissue but lack open communication with the cranial cavity.[medlink.com]
Spastic Quadriplegia
  • The most commonly associated abnormalities and symptoms of survivors include: hydrocephalus (abnormal accumulation of fluid in the brain), spastic quadriplegia (paralysis of the arms and legs), microcephaly (smaller than average head size), ataxia (uncoordinated[lisette-samisblog.blogspot.com]
  • The most commonly associated abnormalities and symptoms of survivors include: Hydrocephalus (abnormal accumulation of fluid in the brain) Spastic quadriplegia (paralysis of the arms and legs) Microcephaly (smaller than average head size) Ataxia (poor[chw.org]

Workup

Pericardial Effusion
  • Prenatal sonograms revealed polyhydramnios, subgaleal edema, microcephaly, a narrow thorax, pericardial effusion, and a severe short-limbed dwarfism with unossified tubular bones and vertebral bodies.[ncbi.nlm.nih.gov]
Staphylococcus Aureus
  • The bacteria most commonly associated with meningitis in such patients is Steptococcus pneumoniae, followed by Staphylococcus aureus then Neisseria meningitides .[ 14 ] Differential diagnosis of this could be traumatic encephalocele, ethmoid-frontal sinus[ncbi.nlm.nih.gov]

Treatment

  • The age of the patient, size of the sac, pathologies that accompanied the condition, and treatments applied were assessed.[ncbi.nlm.nih.gov]
  • The clinical course of this case is described in detail to emphasize the occurrence of life-threatening complications of these associated developmental abnormalities during the treatment of the primary pathology in the neurosurgery practice.[ncbi.nlm.nih.gov]
  • Treatment Treatment Options: Treatment is largely supportive. Attempts at repair of retinal detachments often fail and phthisis bulbi is not uncommon. References[disorders.eyes.arizona.edu]
  • Techniques to preserve the herniated brain tissue have been described, but they focus on increasing the intracranial cavity by ventriculomegaly and retracting the encephalocele into new intracranial space in a second stage, as a result of hydrocephalic treatment[ncbi.nlm.nih.gov]
  • After we carefully evaluate your baby, we tailor a treatment plan to their needs.[seattlechildrens.org]

Prognosis

  • The case reported illustrates the advantages of advance warning in an infant with occipital meningoencephalocele for determining prognosis, planning delivery by cesarean section, and preparing for immediate neurosurgical repair.[ncbi.nlm.nih.gov]
  • […] large subtorcular occipital encephalocele in neonate carries higher risk of associated hindbrain anomalies and secondary process for neurological deterioration which predict the surgical outcomes and long-term prognosis.[ncbi.nlm.nih.gov]
  • Encephaloceles may be associated with brain malformations that can have an effect on the prognosis. The location of the encephalocele greatly impacts the prognosis.[lisette-samisblog.blogspot.com]
  • […] may be associated with brain malformations that can have an effect on the prognosis.[chw.org]
  • Treatment and prognosis The prognosis is variable depending on the presence of associated anomalies and presence of microcephaly (carries a much poorer prognosis).[radiopaedia.org]

Etiology

  • These lesions affect children from the low socioeconomic class, but its etiology remains poorly understood.[ 8 ] Encephaloceles can be congenital or acquired secondary to tumor, hydrocephalus or other cause.[ 9 ] Some suggest that its etiology could be[ncbi.nlm.nih.gov]
  • The etiology is controversial.[fetalsono.com]
  • To determine the genetic etiology of ADDWOC, we performed genome-wide linkage analysis on members of the Vietnamese-American and Brazilian pedigrees.[bristol.ac.uk]

Epidemiology

  • This chapter presents our best understanding of the classification schemes, embryopathogenesis, epidemiology, management, outcomes, and prognosis for children with encephalocele.[clinicalgate.com]
  • Epidemiology of frontoethmoidal encephalomeningocoele in Burma. J Epidemiol Community Health. 1984; 38 :89–98. [ PMC free article ] [ PubMed ] [ Google Scholar ] 12. Bhagwati S, Mahapatra A. Pediatric Neurosurgery.[ncbi.nlm.nih.gov]
Sex distribution
Age distribution

Pathophysiology

  • Additional information is included concerning the pathophysiology and pathology of the disorders being discussed as well as basic information concerning the causative genes (when appropriate).[books.google.ro]

Prevention

  • Wang Y, Liu G, Canfield MA, Mai CT, Gilboa SM, Meyer RE, Anderka M, Copeland GE, Kucik JE, Nembhard WN, Kirby RS; National Birth Defects Prevention Network.[cdc.gov]
  • Centers for Disease Control and Prevention. Facts About Encephalocele. February 25th, 2011. Available at: Accessed On: 10/24/2011 Seattle’s Children Hospital. Encephalocele. Available at: Accessed on: 10/24/2011[rarediseases.org]
  • No drains: Wound dressings under slight pressure are usually sufficient to prevent subcutaneous blood collections. Scroll back to top[ispn.guide]
  • Condition specific organizations Centers for Disease Control and Prevention National Institute of Neurological Disorders and Stroke (NINDS) National Organization for Rare Disorders Additional information and resources for families are available.[health.state.mn.us]
  • In short, there is no way to predict or prevent this distressing condition. Parietal Encephaloceles How is Encephalocele Detected and Diagnosed? In most cases, encephaloceles are detected during routine scans.[howshealth.com]

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