Giant Encephalocele Research Articles

Giant encephaloceles: Are always challenging task?

Giant encephaloceles: Are always challenging task?

Giant Occipital Meningoencephaloceles: Challenges in Management and Our Experience

Introduction: Giant encephalocele are encephalocele more than the size of head from which it arises. They pose a management challenge in view of risk of associated anomaly, challenges in anaesthesia and surgical management. There is no literature available from peripheral tertiary care centres from India apart from few case reports. Methods: This case series comprises of seven cases operated at our institute from 2014 to 2021. After routine clinical examination, patients subjected to recommended radiological investigations. Patients were anaesthetised with standard protocol with intubation in lateral or supine position with head hanging at edge of table and supported at table. Surgery carried out in lateral position with complete excision of sac with CSF diversion if pre existing hydrocephalus was present. Results: Out of seven cases, 3 were males and four female with age range from 12 days to 14 Mo. All patients were anaesthetised with standard protocol and had excision of sac with CSF diversion if required with satisfactory outcome in post operative phase and follow up. No death was recorded. Conclusion: With careful assessment and all recommended investigation with standard practice of anaesthesia and surgery, acceptable outcomes can be expected. Emphasis should be laid for good antenatal folate supplementation and possible antenatal diagnosis of meningoencephaloceles.

Hydrocephalus and occipital encephaloceles: presentation of a series and review of the literature.

Encephaloceles are rare congenital malformations of the central nervous system in which brain tissue is extruded from a defect in the skull. Hydrocephalus can occur in 60 to 90% of patients with posterior encephaloceles when compared to other types of this malformation. This article aims to present a series of posterior encephaloceles and its association with hydrocephalus as well as promote a review of the pertinent literature. A retrospective study of our series based on hospital charts of 50 patients with posterior encephaloceles was performed. Data on sex, location of encephalocele, presence of associated malformations, presence of neural tissue within the malformation, presence of hydrocephalus and microcephaly were recorded. There were 29 females and 21 males. There were 25 (50%) supratorcular, 8 (16%) torcular, and 17 (34%) infratorcular lesions. Mean age of encephalocele primary repair was 8days (range 2-120days). Hydrocephalus was diagnosed in 25 (50%) of the cases. Ventriculoperitoneal shunt was inserted in 24 patients. The mean age at VP shunt insertion was 1.3months (range 0.3-9months). Endoscopic third ventriculostomy was successfully performed in one patient. Dandy-Walker malformation and ventriculomegaly prior to encephalocele surgical correction were positively associated with hydrocephalus (p values 0.05 and 0.01, respectively). Chiari III malformation was found in 2 cases, both requiring CSF shunt for treatment of hydrocephalus and are stable in follow-up. Microcephaly was present in 9 cases. The known mortality rate was 8%. Hydrocephalus is common in patients with posterior encephaloceles, being more frequent in the supratorcular type, especially when associated to Dandy-Walker, Chiari III malformation, and pre-existing ventriculomegaly. The severity of giant encephaloceles, when associated to torcular types and microcephaly, is a limiting factor for development of hydrocephalus, due both to the rapid evolution of natural history and the structural changes in microcephaly.

Repair of Giant Anterior Skull Base Encephalocele Containing Intralesional Eloquent Brain: Technical Note.

Encephaloceles are herniations of intracranial neural tissue and meninges through defects in the skull. Basal encephaloceles are rare anterior skull base defects incident in 1 in 35,000 live births. Sphenoethmoidal encephaloceles are even more uncommon, with an incidence of 1 in 700,000 live births. Anterior skull base encephaloceles may be life-threatening in infants, presenting as airway obstruction and respiratory compromise. They can also present with cerebrospinal fluid (CSF) rhinorrhea, purulent nasal drainage, or meningitis. To report a novel technique for repairing a giant sphenoethmoidal encephalocele containing eloquent neural tissue. A 16-mo-old girl presented with progressive airway obstruction from a giant sphenoethmoidal encephalocele that filled her oral cavity. She had multiple congenital anomalies including agenesis of the corpus callosum and cleft lip and palate. Computed tomography showed complete absence of the bony anterior cranial base, and magnetic resonance imaging demonstrated the presence of the pituitary gland and hypothalamus in the hernia sac. We repaired the encephalocele using a combined microsurgical and endoscopic multidisciplinary approach working through transcranial, transnasal, and transpalatal corridors. The procedure was completed in a single stage, during which the midline cleft lip was also repaired. The child made an excellent neurological and aesthetic recovery with preservation of pituitary and hypothalamic function, without evidence of CSF fistula. The authors describe a novel multidisciplinary technique for treating a giant sphenoethmoidal encephalocele containing eloquent brain. The cleft lip was also repaired at the same time. The ability to work through multiple corridors can enhance the safety and efficacy of an often-treacherous operative endeavor.

Giant Encephalocele in Sokoto, Nigeria: A 5-Year Review of Operated Cases

Encephalocele is a common congenital malformation of the central nervous system; however, giant encephaloceles are rare. The use of folic acid supplementation and termination of pregnancies, which are prenatally diagnosed with encephaloceles and other congenital malformation of the central nervous system, has significantly reduced the occurrence of this type of congenital malformation, especially in developed countries. This was a retrospective review over a 5-year period from January 2006 to December 2010 at the Department of Neurosurgery, Regional Centre for Neurosurgery, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria. The records of patients with giant encephalocele were retrieved from the case notes of patients who had excision and repair for encephalocele. Seventy-three patients had excision and repair of encephalocele over the study period. However, the records of only 50 patients were retrieved. Fourteen (28%) of the 50 whose records were retrieved had giant encephalocele. There were 4 male and 10 female patients (1:2.5). Thirteen (92.9%) had the lesion located in the occipital region, whereas in 1 patient (7.1%) the lesion was at the vertex. Three (21%) of the cases had microcephaly, 1 (7.1%) had macrocephaly, and 1 (7.1%) developed postoperative hydrocephalus. The average size of defect was 2.43 cm, and the size of the lesion ranged from 12× 6 cm to 40× 50 cm. The average maternal age was 20.3 years (n= 6), and the paternal age was 29 years (n= 4). Four out of 7 (57%) mothers had febrile illness in early pregnancy. Seven out of 10 patients (70%) did not have antenatal care. This condition is more common in children conceived during the period when farm products are yet to be harvested, and whose mothers did not attend antenatal care visits. It is recommended that mothers should be educated on the necessity of preconception folic acid. Implementation of a national strategy on food fortification is also advised. We recommend surgery after the first month of life to reduce poor early postoperative outcomes. In developing countries and centers with suboptimal pediatric intensive care units, surgical intervention is preferred after the first month of life with good temperature control, adequate fluid replacement following rupture of the sac, blood transfusion availability, and, only if necessary, complex cranial reconstruction.

Occipito-suboccipital neonatal giant encephalocele

Occipito-suboccipital neonatal giant encephalocele - IJASHNB- Print ISSN No: - 2581-5210 Online ISSN No:- 2581-5229 Article DOI No:- 10.18231/j.ijashnb.2019.006, IP Indian Journal of Anatomy and Surgery of Head, Neck and Brain-IP Indian J Anat Surg Head Neck Brain

Perioperative Management of Children With Giant Encephalocele: A Clinical Report of 29 Cases.

Giant encephalocele, a rare entity, makes anesthesiologists wary of challenging anesthetic course. Apart from inherent challenges of pediatric anesthesia, the anesthesiologist has to deal with unusual positioning, difficult tracheal intubation, and associated anomalies during the perioperative course. Medical records of 29 children with giant encephalocele, who underwent excision and repair, during a period of 13 years, were retrospectively analyzed. Data pertaining to anesthetic management, perioperative complications, and outcome at discharge were reviewed. The average age at admission was 164 days. Hydrocephalus and delayed milestones were present in 19 (65.5%) and 7 (24.1%) children, respectively. Difficulty in tracheal intubation was encountered, in 15 (51.7%) children. Tracheal intubation was attempted with direct laryngoscopy, most often, in lateral position (24 [82.8%]). Intraoperative hemodynamic and respiratory complications were observed in 9 (31.0%) and 5 (17.2%) children, respectively. Intraoperative hypothermia was observed in 4 (13.8%) children. The average stay in the intensive care unit was 2.7 days and average hospital stay was 11.5 days. The condition at discharge remained same as the preoperative period in 24 children (82.7%), deteriorated in 2 (6.9%), and 3 children (10.3%) died. Management of children with giant encephalocele requires the updated knowledge on possible difficulties encountered during the perioperative period. They need specialized anesthetic care for dealing with difficult tracheal intubation, associated congenital anomalies, unusual positioning, electrolyte abnormalities, hypothermia, and cardiorespiratory disturbances. For securing the airway, we suggest the practice of direct laryngoscopy in lateral position after inhalational induction. Muscle relaxant should be administered only after visualization of the glottis.

Incidence of Secondary Hydrocephalus after Excision of Huge Encephaloceles in Neonates: Case Study.

Encephaloceles presents as a protrusion of the cranial contents through a defect in the cranium. The most common sites of occurrence are the occipital and frontonasal regions. The surgical outcome is reported to be satisfactory; however, the incidence of hydrocephalus in patients with encephaloceles is variable in the literature. This study investigated the relationship between the size of the encephaloceles and the occurrence of hydrocephalus. Data of all neonates with encephaloceles who presented to our institution from September 2012 to September 2014 were collected. Surgery was performed during the first 2 weeks of age. Encephaloceles with a maximal diameter > 10 cm were included in the study, and the clinical picture, surgical technique, pre- and postoperative imaging, and follow-up were analyzed. Nineteen cases were included in this study. The mean follow-up period was 7 months. Sixteen cases were occipital; three were frontal. In all patients the maximum diameter was > 10 cm. However, in four patients it was > 18 cm. Postoperative ventriculomegaly occurred in seven cases. Of these, four patients needed a permanent ventriculoperitoneal shunt implantation (21%). Wound dehiscence occurred in two patients who required secondary sutures with a favorable outcome. One patient died 2 weeks after the surgery due to a poor general condition and wound infection. Early surgical excision provides effective treatment of huge encephaloceles. Overall, 21% of cases require cerebrospinal fluid (CSF) diversion afterward depending on associated anomalies. Despite their size, giant encephaloceles can have an excellent prognosis with no need for further treatment or CSF diversion.

Giant Orbital Encephalocele with Proptosis Due to Sphenoid Wing Dysplasia Associated With Neurofibromatosis 1: Soft Tissue Repair

Giant Orbital Encephalocele with Proptosis Due to Sphenoid Wing Dysplasia Associated With Neurofibromatosis 1: Soft Tissue Repair

GIANT FRONTO-PARIETAL ENCEPHALOCELE: A RARE CASE REPORT

Although encephaloceles are common, but giant encephaloceles are rare with only few published short series and are mostly described in occipital location. Giant frontal encephaloceles are very rarely reported with only four published case reports in the world literature and giant encephalocele in fronto-parietal region has not been reported till date. Herein, we report a rare case of giant frontoparietal encephalocele in a six month old girl.

Dev Ensefaloselli Olguda Anestezi Yönetimi

Dev Ensefaloselli Olguda Anestezi Yönetimi

Neuroanaesthetic and perioperative challenges in the management of giant encephaloceles

There are complex issues involved in the surgical management of giant occipital encephaloceles, especially in neonates and young infants. Airway management can cause technical difficulties due to location of lesion, associated abnormalities and the position to be maintained during surgery. We present perioperative challenges we faced in the management of one such case.

Giant Encephalocele: A Study of 14 Patients

Background: Giant encephalocele is a rare condition and few published reports are available in the English literature. It is a challenge to neurosurgeons, even today. This series consists of 14 patients with giant encephaloceles treated at our institute. Material and Observation: Over a period of 8 years, from 2002 to 2009, 110 patients with encephaloceles were managed at our institute. Amongst them, 14 were children with giant encephaloceles. All patients had CT/MRI or both prior to surgery, and all were operated upon. Four patients were neonates, under 1 month of age, and 9/14 patients (64%) were under 3 months. The youngest child was a newborn baby aged 2 days. Except for 1 with an anterior encephalocele, the rest were patients with occipital encephaloceles. A CT scan was performed on 5 and an MRI on 1 patient. Both CT and MRI scans were performed on the other 8 patients. MRI/CT showed hydrocephalus in 10/14 patients. Of these, 7 required ventriculoperitoneal (VP) shunt, and the remaining 3 with mild to moderate hydrocephalus did not. Of the 7 patients who underwent VP shunt, 5 had a shunt during the encephalocele repair and 2 had a postoperative shunt for increasing hydrocephalus. Results: Other associated anomalies recorded were acquired Chiari malformation in 3 patients, secondary craniostenosis with microcephaly in 5, and syringomyelia in 1 patient. All the patients underwent repair of encephalocele and 4 had suturectomy of coronal suture for the secondary craniostenosis. There were 2 postoperative deaths due to hypothermia. Among the 12 surviving patients, 9 had a good outcome and 3 had poor mental development. The present study shows overall good outcomes in 9/14 (66%) patients.

Microvascular reconstruction of a giant encephalocele defect in a 10-week-old infant

Microvascular reconstruction of a cranial defect in a 10-week-old infant, which is the youngest case in the literature, is reported. A latissimus dorsi free muscle flap was transferred to cover the defect and a split thickness skin graft was placed over the muscle flap. Despite the successful flap transfer in this case, microvascular reconstruction in an infant has its own risks and infants should be discussed as a separate entity apart from the other pediatric patients.