Clinical predictors of developmental outcome in patients with cephaloceles

Lo, Benjamin; Kulkarni, Abhaya V.; Rutka, James T.; Jea, Andrew; Drake, James M.; Lamberti-Pasculli, Maria; Dirks, Peter B.; Thabane, Lehana

doi:10.3171/ped.2008.2.10.254

Cited by 102 publications

(118 citation statements)

References 7 publications

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“…Our review has confirmed previous findings that hydrocephalus is significantly associated with cognitive deficit in patients with encephaloceles. 8,9,19 While patients with posterior encephaloceles had a higher rate of progressive hydrocephalus (15/56) and developmental delay (35/56) than those with anterior lesions (2/14 and 7/14, respectively), there was no statistically significant difference between these rates, confirming the finding by Lo et al 19 A sample of large reviews is shown in Table 5 for comparison with our results.…”

Section: Discussionsupporting

confidence: 80%

Risk factors for hydrocephalus and neurological deficit in children born with an encephalocele

Silva

Jeelani

Dang

et al. 2015

PED

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OBJECT There is a known association of hydrocephalus with encephaloceles. Risk factors for hydrocephalus and neurological deficit were ascertained in a series of patients born with an encephalocele. METHODS A retrospective analysis was undertaken of patients treated for encephaloceles at Children's Hospital Los Angeles between 1994 and 2012. The following factors were evaluated for their prognostic value: age at presentation, sex, location of encephalocele, size, contents, microcephaly, presence of hydrocephalus, CSF leak, associated cranial anomalies, and neurological outcome. RESULTS Seventy children were identified, including 38 girls and 32 boys. The median age at presentation was 2 months. The mean follow-up duration was 3.7 years. Encephalocele location was classified as anterior (n = 14) or posterior (n = 56) to the coronal suture. The average maximum encephalocele diameter was 4 cm (range 0.5–23 cm). Forty-seven encephaloceles contained neural tissue. Eight infants presented at birth with CSF leaking from the encephalocele, with 1 being infected. Six patients presented with hydrocephalus, while 11 developed progressive hydrocephalus postoperatively. On univariate analysis, the presence of neural tissue, cranial anomalies, encephalocele size of at least 2 cm, seizure disorder, and microcephaly were each positively associated with hydrocephalus. On multivariate logistic regression modeling, the single prognostic factor for hydrocephalus of borderline statistical significance was the presence of neural tissue (odds ratio [OR] = 5.8, 95% confidence interval [CI] = 0.8–74.0). Fourteen patients had severe developmental delay, 28 had mild/moderate delay, and 28 were neurologically normal. On univariate analysis, the presence of cranial anomalies, larger size of encephalocele, hydrocephalus, and microcephaly were positively associated with neurological deficit. In the multivariable model, the only statistically significant prognostic factor for neurological deficit was presence of hydrocephalus (OR 17.2, 95% CI 1.7–infinity). CONCLUSIONS In multivariate models, the presence of neural tissue was borderline significantly associated with hydrocephalus and the presence of hydrocephalus was significantly associated with neurological deficit. The location of the encephalocele did not have a statistically significant association with incidence of hydrocephalus or neurological deficit. In contrast to modestly good/fair neurological outcome in children with an encephalocele without hydrocephalus, the presence of hydrocephalus resulted in a far worse neurological outcome.

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Section: Discussionsupporting

confidence: 80%

Risk factors for hydrocephalus and neurological deficit in children born with an encephalocele

Silva

Jeelani

Dang

et al. 2015

PED

View full text Add to dashboard Cite

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“…Severe microcephalus was seen in 3 infants, which was accompanied by a poor prognosis, compatible with previous reports [16,40]. Naidich et al [21] have proposed that a lack of associated microcephaly, holoprosencephaly, or ventriculomegaly is a criterion for a good long-term prognosis in cephalocele-affected children.…”

Section: Discussionsupporting

confidence: 74%

“…A birth survey in Bangkok reported an incidence of 1 in 6,000 [15]. Lo et al [16] reported 40 frontal lesions out of 85 cephaloceles in one of the largest series in North America, contrasting previous reports. This fact may be a consequence of ethnic changes, due to an increasing rate of people immigrating to Canada during the past few years.…”

Section: Discussioncontrasting

confidence: 40%

“…Another determinant of poor prognosis in our study was ventriculomegaly. This condition demonstrated the poorest outcome, while in a report by Lo et al [16], hydrocephalus and intracranial abnormalities were the variables with cumulative predictive effects on developmental delay. Univariate analysis in this series has been in line with previous studies where the presence of gross brain tissue in the sac of encephalocele [16] and the size of the sac were important factors leading to poorer outcome [41].…”

Section: Discussionmentioning

confidence: 99%

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Cephalocele: Report of 55 Cases over 8 Years

et al. 2009

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Objective: Cephalocele is a central nervous system (CNS) birth defect. Various CNS and extra-CNS anomalies, as well as prognostic factors have been reported with cephalocele. The aim of this study was to discuss prognostic factors and current possible theories explaining associated anomalies seen in a series of 55 patients with cephalocele. Methods: A retrospective study was performed using the records of 55 children with cephalocele at the Children’s Hospital Medical Center in Tehran, Iran, from October 2000 through October 2008. Patients’ sex, age at the operation time, characteristics of the lesion including location, size, cerebrospinal fluid leakage, radiological assessments and intraoperative findings (sac contents), intracranial and extracranial associated anomalies, and the last situation of the children were reviewed. Conclusion: A genetic role can be proposed while a female predominance is seen in our results as well as in other reports. CNS and extra-CNS anomalies, as well as several genetic syndromes are not explicable as a causal consequence of cephalocele and the probability of a mere coincidence cannot be ruled out; therefore, we propose that the combination of these anomalies is rather a low-frequency association. Ventriculomegaly was the only poor prognostic determinant in our study, which can compel more severe neuroradiologic studies in such patients as a reasonable prognostic evaluation.

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“…The prognosis for patients with a cephalocele depends primarily on site, content of the sac, operability, hydrocephalus, and additional malformations [16,25,29,30,31,34,36,48,53].…”

Section: Discussionmentioning

confidence: 99%

Recurrence of atretic parietal cephalocele in adult: a case report and review of literature

et al. 2018

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Common presentation of atretic parietal cephalocele (APC) is mostly seen in infants and young children. And, it is a palpable midline parietal soft tissue mass which is thought to represent involuted true cephalocele (meningocele or encephalocele) connected to dura mater via a fibrous stalk. The incidence of intracranial anomalies have increased with APC. Parietal cephaloceles comprise about 1% of all cerebrospinal congenital malformations and 10% of cephaloceles. We report here the case of an atretic parietal cephalocele with no associated brain malformations in adult.

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Clinical predictors of developmental outcome in patients with cephaloceles

Cited by 102 publications

References 7 publications

Risk factors for hydrocephalus and neurological deficit in children born with an encephalocele

Risk factors for hydrocephalus and neurological deficit in children born with an encephalocele

Cephalocele: Report of 55 Cases over 8 Years

Recurrence of atretic parietal cephalocele in adult: a case report and review of literature

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