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2.1
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]

Case presentation A 4-month-old baby boy presented to us through the pediatric emergency department following a fall from his mother's lap. [ejns.springeropen.com]

Entire Body System

  • Developmental Delay

    One of the three patients with developmental delays also had a seizure; they did not show improvement at 18 months follow-up regarding developmental milestones and cognition though their seizure was well-controlled on the single epileptic drug. [asianjns.org]

    The child is well at age 3 years, but with significant developmental delay. The pathogenesis of this vertex cephalocele relates to semilobar holoprosencephaly and dorsal cyst formation. [rug.nl]

    Various physical and mental developmental delays can indicate the presence of encephaloceles. [en.wikipedia.org]

    Developmental delay In this condition, an affected child may not reach the developmental milestones at the same time as other children of the same age. [hxbenefit.com]

    delay, Intellectual disability Cdc-pdf [PDF – 280KB], Vision problems, Delayed growth, and Seizures. [cdc.gov]

Respiratoric

  • Tachypnea

    The clinical features seen in these patients included episodic tachypnea and irregular breathing, opsoclonus, ataxia, marked hypotonia of the limbs, coloboma, and polydactyly. All four patients had midline occipital encephalocele. [ncbi.nlm.nih.gov]

  • Hyperpnea

    A syndrome of episodic hyperpnea, abnormal eye movements, ataxia and retardation. Neurology 19: 813 – 825, 1969. Google Scholar 8. [journals.sagepub.com]

Eyes

  • Strabismus

    Nystagmus, strabismus, small optic discs, glaucoma, and cataracts have been reported. Poor vision and progressive loss of acuity are common. [disorders.eyes.arizona.edu]

    Conversely, early developmental disturbances of vision often disrupt ocular motor control systems, giving rise to complex disorders such as nystagmus, strabismus, and torticollis. [books.google.com]

    Strabismus and lacrimal obstructions, resulting in epiphora and/or dacryocystitis can be observed.[ 4, 5 ] Table 1 Suwanwela and Suwanwela classification[ 3 ] CASE REPORT Indication of surgical repair of the lesion (anterior encephalocele) is usually [ncbi.nlm.nih.gov]

Psychiatrical

  • Psychomotor Retardation

    The four main findings in this syndrome may be accompanied by severe psychomotor retardation, blindness, hypotonia, convulsions, and facial dysmorphism. [ncbi.nlm.nih.gov]

Neurologic

  • Screaming

    Nathan came out screaming and kicking. Ironically he peed on the pediatrician who had told us there was no point in the c-section. His apgars were 7 & 9 (like a healthy baby). After birth I was able to hold and nurse him, an answer to prayer. [fivemonkeysmama.blogspot.com]

Treatment

The age of the patient, size of the sac, pathologies that accompanied the condition, and treatments applied were assessed. [ncbi.nlm.nih.gov]

After we carefully evaluate your baby, we tailor a treatment plan to their needs. [seattlechildrens.org]

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]

Prognosis

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]

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]

[…] 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]

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]

They are organised into groups, and further divided into clinical, etiological or histopathological sub-types. [orpha.net]

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]

The etiology is controversial. [fetalsono.com]

Encephalocele Causes The exact etiology of this condition has not been determined by the medical experts yet. As aforementioned, the neural tube fails to close completely during fetal development, causing sac-like protrusions of the meninges. [hxbenefit.com]

Epidemiology

Aims and Objectives: To determine the epidemiological features, patterns of encephalocele, and its postsurgical results. Materials and Methods: The study was carried from year July 2012 to June 2015. [pediatricneurosciences.com]

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]

Most registries and epidemiological studies classify encephaloceles using broad categories like frontal, parietal, occipital and sphenoidal. [rarediseases.org]

Epidemiology [ edit ] Encephaloceles occur rarely, at a rate of one per 5,000 live births worldwide. [en.wikipedia.org]

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]

No drains: Wound dressings under slight pressure are usually sufficient to prevent subcutaneous blood collections. Scroll back to top [ispn.guide]

If brain tissue is present, it may be referred to as a meningoencephalocele. [2] When the head size or occipitofrontal circumference is smaller than the herniating sac, then it is termed as giant encephalocele. [3] Prevention [ edit ] It is recommended [en.wikipedia.org]

The IV provides fluids, medicines, nutrients, and antibiotics to prevent dehydration and infection. [nationwidechildrens.org]

Encephalocele Prevention Although the condition cannot be prevented completely, certain steps can be taken to decrease the chances of development. [hxbenefit.com]

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