Sincipital Encephalocele Research Articles

Periorbital Mass in a 2-month-old Girl.

Periorbital Mass in a 2-month-old Girl.

Sincipital Encephalocele, a Rare Anomaly Diagnosed Antenatally in the Late Pregnancy

AbstractA case of sincipital encephalocele with intracranial cyst is presented which was diagnosed at 35 weeks gestational age in a patient who had undergone no ultrasound examination earlier. The purpose of this article is to make the radiologists and the obstetricians aware of this condition, prognosis, and complications.

Sincipital Encephaloceles: A Study of Associated Brain Malformations.

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.

Endoscopic Endonasal Repair of Congenital Defects of the Anterior Skull Base: Developmental Considerations and Surgical Outcomes

Techniques of endoscopic endonasal surgery, initially developed primarily for intracranial neoplasms, have been adapted to treat a wide variety of pathologies previously approached with open craniotomy, including congenital and acquired defects of the anterior skull base. Congenital defects can lead to herniation of leptomeninges containing CSF alone or with brain tissue. Categories of encephalocele can be defined based on associated abnormal bony anatomy. The most widely used classification scheme divides anterior fossa encephaloceles into sincipital and basal categories. Sincipital encephaloceles, which are associated with a skull defect at the foramen cecum, are anterior to the cribriform plate. These can be nasofrontal, nasoethmoidal, nasoorbital, or interfrontal. Basal encephaloceles, protruding through the cribriform plate or planum sphenoidale, are classified as sphenopharyngeal, spheno-orbital, sphenomaxillary, or sphenoethmoidal.

Antenatal Diagnosis of a Rare Neural Tube Defect: Sincipital Encephalocele.

Context. Fetal sincipital encephalocele is one of the most serious congenital neural tube defects with a high risk of mortality and neonatal morbidity. Prenatal diagnosis of this malformation is important in fetal medicine. Case Report. We report a case of prenatal diagnosis of sincipital encephalocele using ultrasound and MRI imaging. The diagnosis was done at 25 weeks of gestation by identifying an anterior cephalic protrusion through a defect in the skull. Conclusion. Through this case, we discuss the differential diagnosis, management, and prognosis of such lesions.

Naso-ethmoidal encephalocele with bilateral orbital extension: report of a case in a western country

Encephalocele is a rare congenital malformation of the central nervous system with protrusion of cranial content (meninges, brain, and ventricles in different combinations) beyond the normal confines of the skull. Anterior encephaloceles occur with a high frequency in Southeast Asia, while in the Western countries occipital encephaloceles prevail. The treatment of an anterior (naso-ethmoidal) encephalocele involves a neurosurgeon or a multidisciplinary team (neurosurgeon, maxillofacial surgeon, plastic surgeon, and ENT surgeon) dealing with craniofacial surgery. Goals of surgery include removal or repositioning of nonfunctional cerebral tissue, closure of the dura, and reconstruction of skeletal and cutaneous structures. The prognosis depends from the anatomical site, volume of neural contents, and the presence of coexisting malformations. We report the case of an Italian child suffering from a naso-ethmoidal encephalocele with bilateral orbital extension. The surgical treatment was performed in two steps. Sincipital encephalocele is a complex pathology without a unique standardized surgical treatment. Its low incidence in Western countries can make its management particularly tricky.

Coexistence of dermal sinus tract, dermoid cyst, and encephalocele in a patient presenting with nasal cellulitis

Dermoid cysts, encephaloceles, and dermal sinus tracts represent abnormalities that develop during the process of embryogenesis. The elucidation of the precise timing of formation for these malformations has remained elusive at the molecular level of study. Yet, clinical experience has demonstrated that these malformations do not all occur in the same patient, suggesting a shared pathway that goes awry at distinct points for different patients, resulting in 1 of the 3 malformations. Herein the authors describe a case in which all 3 malformations were present in a single patient. This is the first description in the English literature of a sincipital encephalocele occurring with a dermoid cyst and a dermal sinus tract.

Predictors of surgical approaches for the repair of anterior cranial base encephaloceles

Surgical approaches to the anterior cranial base have changed considerably with the introduction of endonasal endoscopic surgery. This study aims to define the factors which help in selecting the optimal surgical approach for the treatment of anterior cranial base encephaloceles. Patients who received treatment for anterior cranial base encephaloceles at our department between 1996 and 2011 were included in the study. Patients' charts were reviewed retrospectively to collect the necessary data. Treatment periods were classified as before 2000, between 2000 and 2005, and after 2005. The relationship between the treatment period, localization of encephalocele, symptoms related with the lesion, size of skull base defect, and selected treatment modality were investigated. Twenty-five patients, aged between 1 and 61 years with anterior encephaloceles were included in the study. Patients with small asymptomatic frontonasal and trans-ethmoidal encephaloceles (n = 5) were followed without surgery. An external approach with or without subfrontal craniotomy was mainly preferred for resection of sincipital encephaloceles (n = 10), especially with facial deformity. A subfrontal craniotomy approach was used for resection of basal encephaloceles in two cases before 2000. Two cases with sincipital encephaloceles and six cases with basal encephaloceles underwent pure endonasal endoscopic surgery after 2000. Cranial base defects of every size could be repaired using the endoscopic approach. Hydrocephalus and meningitis were the two complications seen after craniotomy in a follow-up period of 13-26 (mean 14.5) months. An external approach with or without craniotomy is needed for encephaloceles with external mass and facial deformity. Otherwise, sincipital and basal encephaloceles can be repaired successfully using the endonasal endoscopic approach.

Sincipital Encephaloceles

An encephalocele is a protrusion of the cranial contents beyond the normal confines of the skull through a defect in the skull and the facial bones. Encephaloceles are classified according to their contents, site of exit through the skull/facial bones, and the path traversed through the face. Sincipital encephaloceles are the most common variety seen in the Asian population. Sincipital encephaloceles have been classified by Suwanwela and Suwanwela (1972) into frontoethmoidal, interfrontal, and those associated with craniofacial clefts. Correction of encephaloceles can be done in 1 stage or multiple stages and consists of excision of the encephalocele sac, repair of the bony defect, correction of hypertelorism/telecanthus, and correction of associated deformities such as trigonocephaly and the "long nose."

Rhabdomyolysis after transnasal repair of anterior basal encephalocele

Background Postoperative Rhabdomyolysis (RM) is rare after neurosurgical procedures. Furthermore, it has not been observed after transnasal approaches. The authors report a case of idiopathic RM occurring after transnasal resection of a sincipital encephalocele. Case Description A 32-year-old woman underwent a transnasal resection of a sincipital encephalocele after 6 years of intermittent clear nasal drainage. Postoperatively, she experienced severe back pain, peripheral neuropathy, associated with a markedly elevated creatinine kinase, and severe RM. The patient was treated with hydration and forced urine alkalization and treated symptomatically for her pain and neuropathy. She ultimately made a full recovery without complication. Conclusion Rhabdomyolysis is a rare but known complication of neurosurgical procedures. We report the first known case report of RM after a transnasal procedure. Furthermore, a review of documented postneurosurgical cases of RM is presented and reveals that the causes and risk factors for this complication after neurosurgery are similar to those in other surgical subspecialties.

Surgical management and reconstruction of sincipital encephalocele presenting in adulthood

Sincipital encephaloceles presenting in adulthood are misdiagnosed until they present with persistent cerebrospinal fluid (CSF) rhinorrhea or, in extreme cases, meningeal infection. The basic approach to their repair is a frontal craniotomy and bandeau-type removal of the orbital framework, and reconstruction using dural patch for watertight dural closure, split calvarial bone graft fixation for rigid repair of the bony framework, and a vascularized pericranial flap to provide the soft tissue interposition. We emphasize the pitfall of biopsy when encephalocele is included in the differential diagnosis of a nasal mass and include a step-by-step operative technique that ensures safe and effective reconstruction of the skull base defect.

What is the Incidence of Hypertelorism in Filipino Frontonasal Encephalocele Patients?

It is unclear whether Filipinos with sincipital encephaloceles have true orbital hypertelorism or just telecanthus. Knowing this determines whether orbital osteotomies or medial canthoplasty are more appropriate corrective procedures. To evaluate this, 56 sequential Filipino sincipital encephalocele patients (28 female and 28 male, average age 66.6 months, age range 4 days to 21.8 years) were evaluated for orbital width. Soft tissue measurements of inner and outer canthal position and CT measurements of medial and lateral orbital wall position were obtained. Normative data curves by age and sex for orbital bony and soft tissue distances in normal Filipino children were developed. Data was plotted on these curves and analyzed to determine if encephaloceles were associated with medial widening alone or true hypertelorism. Inner canthal measurements for male were approximately +2.8 standard deviation (SD) and female +2.3 SD wider than average. Medial orbital wall dimensions were wider for male +1.5 SD and female +1.4 SD. Lateral orbital measurements for both male and female clustered around the normative mean (males -0.5 SD and females -0.8 SD). Female outer canthal measurements also clustered around the normative mean (+0.6 SD wider) while male outer canthal measurements averaged approximately +1.1 SD wider than mean. As expected, measurements were wider medially when compared to normative data. However, lateral bony and soft tissue widening was minimal or approached normative averages. This suggests the problem is medial widening only and less invasive procedures such as medial canthoplasty or nasal bony contouring may be the most appropriate surgical management.

O.090 Intraoral distraction osteogenesis to lengthen the ascending ramus. Experiences with seven patients

Anomalies of the face may occur in its lower or middle segments. Anomalies of the lower face generally involve the derivatives of the branchial apparatus and therefore manifest as defects in the mandible, pinnae, external auditory canals, and portions of the middle ears. These anomalies are occasionally isolated, but most of them occur in combination with systemic syndromes. These anomalies generally do not occur with respiratory compromise. Anomalies of the midface may extend from the upper lip to the forehead, reflecting the complex embryology of this region. Most of these deformities are isolated, but some patients with facial clefts, notably the midline cleft syndrome and holoprosencephaly, have anomalies in other sites. This is important because these patients will require detailed imaging of the face and brain. Anomalies of the midface tend to involve the nose and its air-conducting passages. We prefer to divide these anomalies into those with and without respiratory obstruction. The most common anomalies that result in airway compromise include posterior choanal stenoses and atresias, bilateral cysts (mucoceles) of the distal lacrimal ducts, and stenosis of the pyriform (anterior) nasal aperture. These may be optimally evaluated with computed tomography (CT) and generally require immediate treatment to ensure adequate ventilation. Rare nasal anomalies that also result in airway obstruction are agenesis of the pharynx, agenesis of the nose, and hypoplasia of the nasal alae. Agenesis of the nasopharynx and nose are complex anomalies that require both CT and magnetic resonance imaging (MRI). The diagnosis of hypoplasia of the nasal alae is a clinical one; these anomalies do not require imaging studies. Besides facial clefts, anomalies of the nose without respiratory obstruction tend to be centered around the nasofrontal region. This is the site of the most common sincipital encephaloceles. Patients with frontonasal and nasoethmoidal encephaloceles require both CT and MRI. In these patients, imaging of the brain is essential. Patients with suspected extranasal, intranasal, or mixed heterotopia may need images with CT or MRI or both. The last of the anomalies in this region is the presence of ectodermlined tracts and sinuses that may or may not be accompanied with masses. When these masses are located in the midline, they are almost always dermoids, whereas masses located off-midline may also be epidermoids. This latter group of anomalies is important because of its potential communication with the intracranial contents and the possibility of infection. Adequate imaging may require both CT and MRI. Midline facial clefts may be divided into common and complex. Common clefts represent the great majority and are isolated cosmetic defects that generally do not require imaging studies. Complex facial clefts with hypertelorism are almost always a part of the holoprosencephalies; imaging of the brain is more important than is imaging of the cleft proper. The studies should be obtained with MRI. Complex facial clefts in the presence of hypertelorism are most likely related to the midline cleft syndromes. This very rare and intricate group of disorders is generally accompanied with severe brain and eye anomalies. Imaging of these patients is better performed with both CT and MRI. We review common and uncommon facial anomalies. We have tried to approach them according to their clinical importance by dividing them into those with and without airway compromise.

Sincipital encephalocele mismanaged as a facial hemangioma

A 3-month-old male infant was found to have a protruding mass with irregular contour at the root of his nose since birth. The mass protruded more when he cried. It was diagnosed and treated as a hemangioma by laser surgery in another hospital. However, the lesion recurred 1 week later. Reviewing the previous computed tomographic study of the brain, it revealed that the protruding mass communicated with the brain. Sincipital encephalocele was diagnosed, and craniotomy for excising the encephalocele and repair of the dura were undertaken successfully. We report this patient with sincipital encephalocele that was misdiagnosed and treated as a vascular malformation on the face. Fortunately, there was no complication. The authors stress the importance of accurate diagnosis of a lesion near the craniofacial junction to avoid unnecessary surgery and possible catastrophes.

Prognostic factors and long-term results in the surgical treatment of intraoral cancer

Anomalies of the face may occur in its lower or middle segments. Anomalies of the lower face generally involve the derivatives of the branchial apparatus and therefore manifest as defects in the mandible, pinnae, external auditory canals, and portions of the middle ears. These anomalies are occasionally isolated, but most of them occur in combination with systemic syndromes. These anomalies generally do not occur with respiratory compromise. Anomalies of the midface may extend from the upper lip to the forehead, reflecting the complex embryology of this region. Most of these deformities are isolated, but some patients with facial clefts, notably the midline cleft syndrome and holoprosencephaly, have anomalies in other sites. This is important because these patients will require detailed imaging of the face and brain. Anomalies of the midface tend to involve the nose and its air-conducting passages. We prefer to divide these anomalies into those with and without respiratory obstruction. The most common anomalies that result in airway compromise include posterior choanal stenoses and atresias, bilateral cysts (mucoceles) of the distal lacrimal ducts, and stenosis of the pyriform (anterior) nasal aperture. These may be optimally evaluated with computed tomography (CT) and generally require immediate treatment to ensure adequate ventilation. Rare nasal anomalies that also result in airway obstruction are agenesis of the pharynx, agenesis of the nose, and hypoplasia of the nasal alae. Agenesis of the nasopharynx and nose are complex anomalies that require both CT and magnetic resonance imaging (MRI). The diagnosis of hypoplasia of the nasal alae is a clinical one; these anomalies do not require imaging studies. Besides facial clefts, anomalies of the nose without respiratory obstruction tend to be centered around the nasofrontal region. This is the site of the most common sincipital encephaloceles. Patients with frontonasal and nasoethmoidal encephaloceles require both CT and MRI. In these patients, imaging of the brain is essential. Patients with suspected extranasal, intranasal, or mixed heterotopia may need images with CT or MRI or both. The last of the anomalies in this region is the presence of ectodermlined tracts and sinuses that may or may not be accompanied with masses. When these masses are located in the midline, they are almost always dermoids, whereas masses located off-midline may also be epidermoids. This latter group of anomalies is important because of its potential communication with the intracranial contents and the possibility of infection. Adequate imaging may require both CT and MRI. Midline facial clefts may be divided into common and complex. Common clefts represent the great majority and are isolated cosmetic defects that generally do not require imaging studies. Complex facial clefts with hypertelorism are almost always a part of the holoprosencephalies; imaging of the brain is more important than is imaging of the cleft proper. The studies should be obtained with MRI. Complex facial clefts in the presence of hypertelorism are most likely related to the midline cleft syndromes. This very rare and intricate group of disorders is generally accompanied with severe brain and eye anomalies. Imaging of these patients is better performed with both CT and MRI. We review common and uncommon facial anomalies. We have tried to approach them according to their clinical importance by dividing them into those with and without airway compromise.

Cephaloceles: classification, pathology, and management.

A cephalocele is defined as a herniation of cranial contents through a defect in the skull. Cephaloceles are classified according to their contents and location. We have reviewed a total of 112 patients with cephaloceles, 51 of whom had sincipital meningoencephaloceles (fronto-ethmoidal meningoencephaloceles). This group is distinctive in its demographic distribution, in the effect on growth of other facial structures, and in the combined craniofacial approach needed to treat them. This review is based on the sincipital encephaloceles with the other cephaloceles included for completeness. Despite many theories, the cause of congenital cephalocele is not known. Preoperative work-up includes 3-dimensional computed tomography scan of the facial skeleton, and surgical management is multidisciplinary in nature. The aim is to remove the lesion before the deformity has time to greatly distort facial growth, which appears to realign itself after surgery. The 50 patients who underwent surgery for fronto-ethmoidal encephalocele all survived with minimal complications.

Isotope cisternography and ventriculography in congenital anomalies of the central nervous system.

The author discusses the value of isotope cisternography and ventriculography in the diagnosis of a variety of congenital anomalies of the central nervous system (CNS) associated with either hydrocephalus or a disturbance in cerebrospinal fluid (CSF) flow and absorption. In sincipital encephaloceles the techniques demonstrate CSF communication with the anomalous sac and the frequently associated hydrocephalus with occipital lesions. In porencephaly and cerebral agenesis, the isotope demonstrates ventricular reflux and remains in the cystic dilatation indicating abnormal CSF absorption; isotope ventriculography also reveals a delayed CSF absorption and the possible sites of CSF obstruction in Arnold-Chiari malformations and Dandy-Walker syndrome. In addition to their differential diagnostic value isotope studies can be a helpful guide in the choice of the proper surgical treatment of some CNS anomalies.