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Encephalocele (Encephalocele, Frontal)

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Brain tissue herniation through a congenital or acquired defect in the skull. The majority of congenital encephaloceles occur in the occipital or frontal regions. Clinical features include a protuberant mass that may be pulsatile. The quantity and location of protruding neural tissue determines the type and degree of neurologic deficit. Visual defects, psychomotor developmental delay, and persistent motor deficits frequently occur.

Also Known As:

Encephalocele, Frontal; Encephaloceles; Acquired Encephalocele; Bifid Cranium; Cephalocele; Cerebellar Hernia; Cerebellar Herniation; Cranial Meningoencephalocele; Craniocele; Cranium Bifidum; Encephalocele, Acquired; Encephalocele, Occipital; Encephalocele, Sincipital; Notoencephalocele; Sincipital Encephalocele; Tonsillar Hernia; Tonsillar Herniation; Acquired Encephaloceles; Bifid Craniums; Bifidum, Cranium; Bifidums, Cranium; Cephaloceles; Cerebellar Hernias; Cerebellar Herniations; Cerebral Hernia; Cerebral Hernias; Cranial Meningoencephaloceles; Cranioceles; Cranium Bifidums; Cranium, Bifid; Craniums, Bifid; Encephaloceles, Acquired; Encephaloceles, Frontal; Encephaloceles, Occipital; Encephaloceles, Sincipital; Frontal Encephaloceles; Hernia, Cerebellar; Hernia, Tonsillar; Hernias, Cerebellar; Hernias, Cerebral; Hernias, Tonsillar; Herniation, Cerebellar; Herniation, Tonsillar; Herniations, Cerebellar; Herniations, Tonsillar; Meningoencephalocele, Cranial; Meningoencephaloceles, Cranial; Notoencephaloceles; Occipital Encephaloceles; Sincipital Encephaloceles; Tonsillar Hernias; Tonsillar Herniations; Frontal Encephalocele; Hernia, Cerebral; Occipital Encephalocele

Networked: 430 relevant articles (11 outcomes, 13 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Spinal Dysraphism (Spina Bifida)
2.	Cleft Palate (Palate, Cleft)
3.	Syringomyelia (Morvan's Disease)
4.	Neoplasms (Cancer)
5.	Cholesteatoma

Experts

1.	Sípek, A: 8 articles (10/2012 - 12/2000)
2.	Gregor, V: 8 articles (10/2012 - 12/2000)
3.	Horácek, J: 7 articles (12/2008 - 12/2000)
4.	Johnson, Colin A: 6 articles (01/2014 - 02/2006)
5.	Bolger, William E: 5 articles (06/2006 - 08/2002)
6.	Olney, Richard S: 4 articles (11/2014 - 11/2009)
7.	National Birth Defects Prevention Study: 4 articles (11/2014 - 11/2009)
8.	Wang, Zhi-Ping: 4 articles (03/2014 - 09/2011)
9.	Zhao, Zhong-Tang: 4 articles (03/2014 - 09/2011)
10.	Woodworth, Bradford A: 4 articles (11/2013 - 06/2009)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Encephalocele:

1.	Neural tube defects X-linkedIBA 12/01/2000 - "A significant reduction of the incidence of anencephaly, spina bifida and encephalocele in the Czech Republic was found during the last decade of the investigation period (1961-1999). " 06/01/2015 - "The cases were 547 women who gave birth to a child with an NTD (anencephaly, spina bifida, or encephalocele); the controls were 543 women who gave birth to a full-term healthy infant. " 06/01/2014 - "A case-control study was designed to compare the frequencies of the polymorphism at four sites in the SUFU gene in control and NTDs group, as well as in subtype groups, including anencephaly, spina bifida and encephalocele. " 07/01/2013 - "Differences in factors and their effects on NTDs were found for the three subtypes of NTDs: anencephaly, spina bifida and encephalocele. " 06/01/2013 - "Only Anencephaly, Spina bifida and Cephalocele were considered as NTD. "
2.	Folic Acid (Vitamin M)FDA LinkGeneric 09/01/2006 - "Additional studies should be done to explore the etiologic heterogeneity of encephalocele using better markers of folate status and a wider range of risk factors." 03/01/2006 - "Infants with encephalocele had a statistically nonsignificant increase in survival rates, ie, 79.1% (adjusted hazard ratio: 0.76; 95% confidence interval: 0.51-1.13) with folic acid fortification, compared with 75.7% for earlier births. " 03/01/2006 - "First-year survival rates for both spina bifida and encephalocele cohorts were measured with Kaplan-Meier estimation; factors associated with improved chances of first-year survival, including birth before or during folic acid fortification, were measured with Cox proportional-hazards regression analysis. " 03/01/2006 - "Our aim was to determine whether survival among infants born with spina bifida and encephalocele has improved since folic acid fortification and to measure the effects of selected maternal, pregnancy, and birth characteristics on first-year (infant) survival rates. " 04/01/2009 - "Neural tube defects in Australia: trends in encephaloceles and other neural tube defects before and after promotion of folic acid supplementation and voluntary food fortification."
3.	Cerebrospinal fluid leakIBA 01/01/2014 - "[Endoscopic treatment using ethmoidal artery as pedicle of the septum flap repair for iatrogenic meningeal encephalocele with cerebrospinal fluid leak: a case report]." 09/01/2013 - "Here, authors report two such cases, one presented with ruptured encephalocele with cerebrospinal fluid leak and another case presented six months after birth due to financial constraints. " 01/01/2013 - "Postoperative cerebrospinal fluid leak after septoplasty: A potential complication of occult anterior skull base encephalocele." 07/01/2010 - "This is a retrospective study of eight patients with spontaneous cerebrospinal fluid leak and six patients with cerebrospinal fluid leak or encephalocele discovered during chronic ear surgery who were treated in a tertiary medical center over a 5-year period. " 03/01/2006 - "Otolaryngologists are increasingly being called upon to treat patients with cerebrospinal fluid leak and encephaloceles. "
4.	Morphine (MS Contin)FDA LinkGeneric 06/01/1997 - "To determine the efficacy of lumbar intrathecal (i.t.) morphine in a dose of 0.02 mg/kg in providing analgesia following repair of frontal encephaloceles. " 06/01/1997 - "Lumbar IT morphine provides effective analgesia following repair of frontal encephaloceles in children and adolescents." 06/01/1997 - "Intrathecal morphine for postoperative analgesia following repair of frontal encephaloceles in children: comparison with intermittent, on-demand dosing of nalbuphine."
5.	2'- deoxythymidylyl- (3'- 5')- 2'- deoxyadenosine (d(AT))IBA 02/01/2009 - "Most of the encephalocele patients manage d at BKKH had good outcomes and proceeded to live normal or near-normal lives. "
6.	Hereditary renal agenesisIBA 01/01/2000 - "We report a female fetus with occipital encephalocele, dysraphism of the cervical spine, right renal agenesis and Mullerian agenesis. " 04/01/2015 - "Here, we describe a case of 26-year primigravida with 23 weeks pregnancy whose detailed sonographical examination of the fetus revealed large encephalocele through the posterior fontanelle microcephaly, anhydramnios, unilateral left enlarged polycystic kidney and right sided renal agenesis. " 10/01/2001 - "Malformations incompatible with life (anencephaly, encephalocele, hypoplastic lungs, renal agenesis, and trisomies 13 and 18) were the cause of one-third of all malformation deaths. " 02/01/1993 - "Several fetuses with cleft palate, renal agenesis, exencephaly/encephalocele, and/or Di-George sequence were noted at the high dose, the latter a previously undescribed finding. " 07/01/2003 - "More precise anatomy and location of fetal pathology (20 of 40 cases) and additional etiologic information (8 of 40 cases) were substantial advantages in cerebrospinal abnormalities [ventriculomegaly, encephalocele, vein of Galen malformation, callosal malformations, meningo(myelo)cele], in retroperitoneal abnormalities (lymphangioma, renal agenesis, multicystic renal dysplasia), and in neck/thoracic pathology [cervical cystic teratoma, congenital hernia diaphragmatica, congenital cystic adenomatoid lung malformation (CCAM)]. "
7.	VitaminsIBA 05/15/2001 - "They compared data on 1,242 infants with NTDs (spina bifida, anencephaly, and encephalocele) with data from a control group of 6,660 infants with malformations not related to vitamin supplementation. " 07/20/1991 - "A randomised double-blind prevention trial with a factorial design was conducted at 33 centres in seven countries to determine whether supplementation with folic acid (one of the vitamins in the B group) or a mixture of seven other vitamins (A,D,B1,B2,B6,C and nicotinamide) around the time of conception can prevent neural tube defects (anencephaly, spina bifida, encephalocele). "
8.	TeratogensIBA 09/01/1997 - "RESEARCH OBJECT: This study intends to consider an encephalocele experimental model, obtained in embryonate eggs, treated in a post-neurulation phase with chemical teratogens. "
9.	Niacinamide (Nicotinamide)FDA LinkGeneric 07/20/1991 - "A randomised double-blind prevention trial with a factorial design was conducted at 33 centres in seven countries to determine whether supplementation with folic acid (one of the vitamins in the B group) or a mixture of seven other vitamins (A,D,B1,B2,B6,C and nicotinamide) around the time of conception can prevent neural tube defects (anencephaly, spina bifida, encephalocele). "
10.	MelatoninIBA 11/01/2015 - "The aim of this study was to investigate the effects of melatonin on the incidence of NTDs (including anencephaly, encephalocele, and spina bifida) of offspring from diabetic pregnant mice as well as its underlying mechanisms. "

Therapies and Procedures

1.	Decompression 09/01/1996 - "The results of the study revealed that encephaloceles could be induced in 78% of chick embryos manipulated at stage 26 that had evidence of marked decompression of the primitive ventricle. " 01/01/1976 - "Contrast studies performed because of their clinical deterioration demonstrated cerebellar tonsillar herniation, and at posterior fossa decompression, this was confirmed in all cases. " 04/01/2014 - "All patients had some degree of tonsillar herniation, with 25 patients treated with foramen magnum decompression. " 09/01/2011 - "Increase in the number of encephalocele was noted, which is to be expected considering that dural decompression is used in DC procedure. " 09/01/2009 - "However, both tonsillar herniation and cervical syrinx may clinically and radiologically resolve following removal of the intracranial lesion and posterior fossa decompression."
2.	Craniotomy 10/01/1989 - "Modification of existing craniofacial surgical approaches in order to avoid a frontal craniotomy, allowed for good repair of the encephalocele together with significant benefits in terms of simplification of the surgical procedure, operating time, blood loss, frontal lobe retraction and complications." 09/01/2015 - "Five patients underwent a craniotomy for resection of a temporal lobe encephalocele with repair of a middle fossa floor defect. " 04/01/2015 - "After frontotemporal craniotomy, it was possible to localize the encephalocele and the underlying bone defect. " 04/01/2015 - "We conducted a retrospective review of 32 patients undergoing combined mastoidectomy and middle cranial fossa craniotomy for the treatment of temporal encephalocele. " 04/01/2014 - "The patient was operated upon with right frontal craniotomy with excision of encephalocele and repair of skull base. "
3.	Ventriculostomy 01/01/2007 - "She underwent endoscopic third ventriculostomy that resulted in a marked decrease in the ventricle size as well as complete resolution of the syrinx and tonsillar herniation within 4 days. " 01/01/2014 - "CSF diversion to parasellar cisterns is not a known iatrogenic cause of basal encephalocele and is not noted elsewhere as a complication of third ventriculostomy. " 09/01/2001 - "Resolution of tonsillar herniation following endoscopic third ventriculostomy." 04/01/2010 - "Jarvis, Chief of Head and Neck Surgery at the now Kenyatta National Hospital, when he operated on anterior encephaloceles, and later also performed anterior third ventriculostomies for hydrocephalus. " 12/01/1981 - "Because the frequency of improvement and the risk of upward cerebellar herniation following ventriculostomy is unknown, immediate surgical evacuation of the hemorrhage should be anticipated."
4.	Endoscopes 06/01/2012 - "However, with advancements in endoscopic skull base surgery, including improved surgical exposures, angled endoscopes and instruments, and novel repair techniques, these encephaloceles can be resected and successfully repaired with purely endoscopic endonasal approaches. " 04/01/2008 - "Meticulous manipulations of the endoscope following precise autograft placement are mandatory for the successful repair of traumatic basal encephaloceles." 12/01/2012 - "An endoscope-assisted trans-sphenoidal approach was performed and, with the aid of image guided surgery, reduction of the encephalocele was obtained and followed by surgical repair of the dural and bony defects. "
5.	Radiotherapy 05/01/2012 - "In 27 patients with benign tumor, 24 were cured, without recurrence during follow-up; in 8 patients with malignant tumor, 6 had recurrence within 18 months and underwent second operation or radiotherapy, 2 relapsed cases died of cerebral hernia and respiratory circulating failure at 24 months after operation. " 09/01/1950 - "[Intrasellar cerebral hernia after prolonged radiotherapy of acromegaly]." 12/01/2008 - "We present a 31-year-old woman with a frontal sinus encephalocele who developed rhinorrhoea after a ventriculo-peritoneal shunt to treat her hydrocephalus and underwent radiotherapy for a tectum tumour."