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Tail-like caudal appendages are rare and have been reported to be associated with spinal dysraphism, especially with spinal lipomas or lipomyelomeningoceles. We present the case of an 11 year-old girl with human tail and lipomeningocoele.
Citations
... Other locations include cerebellopontine angle cisten (9%), Sylvian cistern (5%) and rarely on the surface of the cerebral hemispheres 8,11,12,13 . The callosal lipomas can be divided into two types: a bulky tubulonodular anterior variety which is associated with forebrain and rostral callosal anomalies and a ribbon-like curvilinear posterior lipoma with a normal or nearly normal corpus callosum 14 . ...
Intracranial lipomas are rare, frequently asymptomatic, congenital malformations. They are most commonly located in the pericallosal region and are often detected incidentally during neuroimaging studies or postmortem examinations. While other associated brain malformations, most notably callosal agenesis, are frequently reported, association with a subcutaneous scalp lipoma is extremely rare. We present a case of pericallosal lipoma associated with callosal agenesis and subcutaneous lipoma over the anterior fontanelle in a 6-month-old female infant who had excision of only the extracranial mass and has remained asymptomatic from the intracranial mass for the 3 years of follow up.
... Encephaloceles are generally classified based on the anatomical location where 75% of encephaloceles are located in the occipital region, 13-15% are situated in the frontal ethmoidal region, and 10-12% in the parietal or the sphenoidal region [5]. Infants with encephaloceles may often have associated malformations, reported to occur in 36-60% of children [6] and include anomalies such as microcephaly, agenesis of the corpus callosum, holoprosencephaly, arachnoid cyst, Dandy-Walker malformation, Chiari malformation, craniosynostosis, cleft lip or palate, Klippel-Feil malformation, myelomeningocele, hydrosyringomyelia, and optic nerve abnormalities [5][6][7][8]. ...
... For a newborn with an encephalocele, the prognosis depends on many factors including the size and contents of the sac, presence of microcephaly, hydrocephalus, and associated anomalies [5,8,9]. A significant proportion of these children lack normal developmental milestones and may have mental and growth retardation, seizures, ataxia, and visual impairment [6]. ...
Background
Encephaloceles are cystic congenital malformations in which central nervous system (CNS) structures, in communication with cerebrospinal fluid (CSF) pathways, herniate through a defect in the cranium. Hydrocephalus occurs in 60–90% of patients with occipital encephaloceles.
Objective
Assessment of the surgical management of hydrocephalus associated with occipital encephalocele and its effect on the clinical outcome.
Methods
Between October 2015 and October 2019, a retrospective study was conducted on seventeen children with occipital encephaloceles who were operated upon. The presence of progressive hydrocephalus was determined by an abnormal increase in head circumference and an increase in the ventricular size on imaging studies. A ventriculoperitoneal (VP) shunt was applied in patients who had hydrocephalus. The clinical outcome was graded according to the developmental milestones of the children on outpatient follow-up visits.
Results
The mean age at surgery was 1.6 (range, 0–15) months. There were ten girls (58.8%) and seven boys (41.2%). Ten encephaloceles (58.8%) contained neural tissue. Ten patients (58.8%) had associated cranial anomalies. Eleven children (64.7%) had associated hydrocephalus: four of them (36.4%) diagnosed preoperatively, while seven children (63.6%) developed hydrocephalus postoperatively. Ten of them (90.9%) were managed by VP shunt. All children with hydrocephalus had some degree of developmental delay, including six (54.5%) with mild/moderate delay and five (45.5%) with severe delay. Half of the patients (50%) of the children with occipital encephalocele without hydrocephalus had normal neurological outcome during the follow-up period ( p value= 0.034).
Conclusions
Occipital encephalocele is often complicated by hydrocephalus. The presence of hydrocephalus resulted in a worse clinical outcome in children with occipital encephalocele, so it can help to guide prenatal and neonatal counseling.
... The most common associated malformation with sincipital encephaloceles is the agenesis or hypogenesis of the corpus callosum. [11] Corpus callosum is the largest cerebral commissure connecting neocortical areas and develops between 12 and 20 weeks of gestation. Development occurs from front to back with the exception of the rostrum which develops after the splenium. ...
Objective:
The aim of this study was to evaluate the associated intracranial malformations in patients with sincipital encephaloceles.
Materials and methods:
A hospital-based cross-sectional study was conducted over 8 years from June 2007 to May 2015 on 28 patients. The patients were evaluated by either computed tomography or magnetic resonance imaging whichever was feasible. Encephaloceles were described with respect to their types, contents, and extensions. A note was made on the associated malformations with sincipital encephaloceles.
Results:
Fifty percent of the patients presented before the age of 3 years and both the sexes were affected equally. Nasofrontal encephalocele was the most common type seen in 13 patients (46.4%), and corpus callosal agenesis (12 patients) was the most common associated malformation. Other malformations noted were arachnoid cyst (10 patients), hydrocephalus (7 patients), and agyria-pachygyria complex (2 patients).
Conclusion:
Capital Brain malformations are frequently encountered in children with sincipital encephaloceles. Detail radiological evaluation is necessary to plan treatment and also to prognosticate such rare malformations.
... The most common associated malformation with sincipital encephaloceles is the agenesis or hypogenesis of the corpus callosum. [11] Corpus callosum is the largest cerebral commissure connecting neocortical areas and develops between 12 and 20 weeks of gestation. Development occurs from front to back with the exception of the rostrum which develops after the splenium. ...
The subject of this report is a rare case of naso-orbital meningocele concurrent with right cerebral hemisphere hydranencephaly and agenesis of corpus callosum in a newborn lamb. Our necropsy findings revealed that the bulging sac containing CSF extends into the skull bony and dural defects, through which the cyst protrudes into the orbital and nasal cavities. Also, histological and immunohistochemical demonstrations of the cyst wall showed that the inner layer is composed of meningeal components. These findings support the diagnosis of a naso-orbital meningocele. This case is an unusual presentation of a rare congenital skull defect and associated anomalies in veterinary literature.
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.
