Nasal Encephalocele Research Articles (Page 1)

Introduction Endoscopic endonasal approach(EEA) techniques have been increasingly utilized and have been associated with development of cerebrospinal fluid(CSF) leak, meningitis, diabetes insipidus post‐operatively. Cerebral vasospasm following EEA has rarely been described. Here, we describe the clinical course and management of a patient who underwent EEA for encephalocele repair whose course was complicated by postoperative subarachnoid hemorrhage(SAH) and cerebral vasospasm. Methods n/a Results Case Presentation: 40 year old female with past medical history significant for cerebral venous sinus stent on aspirin, CSF rhinorrhea secondary to right nasal encephalocele s/p endoscopic transnasal transethmoidal repair with nasoseptal flap reconstruction and lumbar drain placement eleven days prior presented with speech difficulties and right sided weakness. On exam, patient was noted to have mild right hemiparesis and expressive aphasia. MRI brain showed subacute infarcts in left greater than right frontal lobes, corpus collosum and right anterior perforated substance. MRA head demonstrated multifocal arterial stenosis. In light of postoperative CT head showing SAH in the basilar, perimesencephalic, prepontine cisterns, interhemispheric fissure and right frontal sulci as well as intraventricular hemorrhage in fourth ventricle, her presentation was thought to be secondary to cerebral vasospasm in the setting of postoperative SAH. She was treated with intravenous hydration, permissive hypertension with head of bed in flat position and transferred for further evaluation. On arrival, she continued have mild right hemiparesis and aphasia. Repeat CTA head/neck and CT perfusion showed severe stenosis of bilateral M1 segments and left greater than right A1 segments as well as ischemic penumbra in left ACA/MCA watershed territory. Diagnostic cerebral angiogram showed bilateral severe A1 stenosis and mild to moderate bilateral M1 and supraclinoid ICA stenosis which improved with intra‐arterial verapamil. She was started on nimodipine. Systolic blood pressure was augmented with vasopressors for goal of 150‐180mmHg. Daily TCDs were followed. She developed worsening right leg weakness following day, thus she was taken for repeat diagnostic cerebral angiogram during which time she was re‐administered intra‐arterial verapamil with improvement in vasospasm. After the second treatment, she had improvement in speech and motor strength. Systolic blood pressure goal was gradually normalized. She was noted to have incidental left internal jugular (IJ) vein thrombosis for which anticoagulation was held in the setting of recent neurosurgical procedure and SAH. Workup for vasculitis and hypercoagulability was unrevealing. Lumbar puncture demonstrated 13 WBC/cu mm with lymphocytic predominance(90%), 23 RBC/cu mm, glucose 89mg/dl, protein 30mg/dl. CSF cultures were negative. Pleocytosis in CSF was attributed to recent neurosurgical procedure. Her neurological exam improved to baseline on hospital day(HD) 9.On HD12, she was found to have left common femoral deep venous thrombus in addition to the left IJ thrombus. Anticoagulation with low dose heparin infusion was started which was transitioned to apixaban on discharge. She was discharged home on HD20. Conclusion We report a case of EEA associated with severe multifocal cerebral vasospasm secondary to postoperative SAH that was successfully treated with induced hypertension, oral nimodipine and intra‐arterial verapamil as an adjunct therapy with an excellent outcome.

Snoring is noisy breathing during sleep and is a symptom of sleep disordered breathing. Persistent snoring is an alarming symptom requiring extensive examination of the oral and respiratory tract. There are various causes of nasal obstruction in children. External nasal examination with nasal speculum and nasal endoscopy helps to identify the origin and nature of nasal mass. We report a case of nasal encephalocele in a 5-year-old male child who presented with snoring.

Congenital midline nasal masses are rare. Nasal dermoid sinus cysts（NDSC） are the most common type of the congenital midline nasal masses in childhood. Clinical manifestations are midline nasal cysts, fistula and intracranial attachments. Nasal encephalocele and glioma should be included in the differential diagnosis. Radiologic images are instructive. NDSC are easily misdiagnosed, leading to recurrence and surgical trauma affecting the face. Early appropriate surgical excision is recommended. This article reviews the embryology pathogenesis, progress of diagnosis and treatment of congenital NDSC.

An Unusual Presentation of Unilateral Nasal Mass in Infant: Congenital Nasal Encephalocele

Encephalocele is most commonly a defect in the neural tube characterised by herniation of the brain and the membranes in form of protusions that cover it through openings in the skull. It is mostly congenital but can be acquired following trauma. It usually presents with cerebrospinal fluid leak, which may get overlooked because of their subtle or intermittent course. High level of suspicion is required for early diagnosis and treatment. We hereby present a rare scenario, the patient presented more than 12 years after trauma with complaint of unilateral nasal blockage and discharge. The diagnostic investigations and treatment options are discussed. Patient was treated by endonasal endoscopic resection of mass and multilayered repair of skull base defect and is without recurrence in last more than 1 year follow up.

Nasal meningoencephalocele: A retrospective study of clinicopathological features and diagnosis of 16 patients

To describe the clinical presentation, diagnosis, and management of pediatric nasal encephaloceles. Encephaloceles and meningoencephaloceles are on the differential diagnosis for congenital midline nasal masses. These lesions are treated surgically to prevent craniofacial deformities and meningitis and to address symptoms such as a nasal obstruction, feeding difficulties, and cerebrospinal fluid (CSF) rhinorrhea. With the advent of endoscopic skull base approaches and instruments, surgical treatment of nasal encephaloceles have transitioned from large bicoronal external approaches to more minimally invasive endonasal endoscopic approaches. Endoscopic surgical approaches are safe in pediatric patients. Pediatric intranasal encephaloceles are more frequently managed endoscopically. However, due to the rarity of this condition, further multi-institutional research is needed to examine long-term outcomes for this surgical approach.

To retrospectively analyse surgical management of clinico-radiologically proven nasal meningoencephalocele amongst children and results of repair with single-layer septo-mucosal flap at a tertiary skull base surgery centre in north India. Fifteen children with clinic-radiological nasal meningoencephaloceles with or without CSF rhinorrhoea were included. Radiological scans included CT scans and MRI scans for all cases to delineate soft tissue and bony architecture. All children underwent endoscopic excision of encephaloceles and repair of the skull base defect. The authors were successful in fourteen cases. Successful cases included 4 children less than 6months of age who underwent single-layer rotated septo-mucosal flaps, thereby avoiding donor site morbidity. As endoscopic excision of nasal encephalocele is an established management approach, the authors take the concept of minimally invasive surgery a step further by successfully managing these lesions with single-layer repair. Vascularized septo-mucosal flap seems to be the only determining factor for a successful repair for congenital meningoencephalocele. However, a prospective study comprising a larger subset of patients would substantiate the assumption.

BackgroundCri-du-chat syndrome (CdCS; OMIM#123450) is a classic contiguous gene syndrome caused by chromosome 5p terminal deletion (5p-), which characterized by a high-pitched cat-like cry, developmental delay, severe psychomotor, mental retardation, and dysmorphic features in infancy. Prenatal diagnosis of CdCS is difficult due to the non-specific ultrasound features. And reports using array analysis are rare. This study presented the first retrospective analysis of prenatal series of CdCS fetuses diagnosed by single nucleotide polymorphism (SNP) array in China.Case presentationA total of 35,233 pregnant women were enrolled from Jan 2014 to April 2019 in our center, there are twelve 5p- cases with abnormal sonographic signs revealed by SNP array, giving an incidence of 0.034% (12/35,233). Clinical information and molecular basis included: maternal demographics, indications for invasive testing, sonographic findings and SNP array results. Among all the 5p- cases revealed, nine cases were diagnosed by both karyotyping and SNP array, three cases were detected only by SNP array. Half of our cases (6/12) had an isolated 5p terminal deletion, which sizes ranged from 9.0 Mb to 30 Mb. The other half of cases (6/12) characterized by unbalanced translocation, with sex ratio 7:5 (female: male), when combine the clinical features observed from this study and available literature, the most frequent anomaly observed in prenatal ultrasound examination of CdCS was cerebral abnormalities, accounted for 44.4% (16/36) of the existing cases. Features that are less consistent included: choroid plexus cyst (13.8%, 5/36), single umbilical artery (13.3%, 4/30), ventricular septal defect (11.1%, 4/36), hydrops fetalis (8.3%, 3/36), ascites (8.3%, 3/36), increased NT/NF (8.3%, 3/36), absent/severely hypoplastic nasal bone (5.5%, 2/36), in order.ConclusionPrenatal findings such as cerebral abnormalities, absent/hypoplastic nasal bone, hydrops fetalis, ascites or encephalocele may act as suggestive signs of CdCS or other microdeletion/duplication syndromes. Combining typical karyotyping with chromosomal microarray analysis (CMA) is a definitive method for a precise diagnosis of CdCS and provides more accurate results in order to offer genetic counseling to families which need to deal with cryptic aberrations.

To identify and determine variations on eye distance in patients with bilateral nasal polyposis (BNP) compared to a healthy control group. This is a case-control study that included 20 BNP patients and 40 healthy controls. We included all patients with BNP confirmed by pathology and a computed tomography scan. A healthy control group was admitted, filtered by the exclusion criteria of nasal polyposis, craniofacial malformations, and encephalocele. Paranasal sinus CT scans were performed in all participants, and two measures were evaluated, the interoptic (soft tissue) and the interzygomatic (bone structure) distances. A total of 20 BNP subjects, 13 (65%) male and 7 (35%) female, with a mean age of 38.8years, and 40 healthy controls, 16 (40%) male and 24 (60%) female with a mean age of 43.2years, were included. The mean interoptic distance was 69.7mm (71.9mm men, 66.4mm women) and interzygomatic distance was 103.1mm (104.5mm men, 100.6mm women). A significant increase of the interoptic (p < 0.001) and interzygomatic (p < 0.002) measurements was found in patients with polyposis compared to the controls. In the receptor operative curve analysis, the interoptic distance had an area under a curve of 96% and the threshold that maximizes the sensitivity and specificity was 59.85mm (sensitivity 90%, specificity 95%, PPV 90%, NPV 95%). An increase in ocular and orbital distances was identified in patients with BNP. Polyposis may be identified by measuring eye separation. The established cut point distance identifies patients that may benefit from follow-up. Further research in this study line is suggested.

Infants with an extremely low birth weight, who receive intensive unit care in the neonatal period may have severe unrecognized neurologic sequelae. We describe a 3-year-old boy, born preterm small for gestational age, who presented with food aversion, failure to thrive, and recurrent upper respiratory tract infections. Because of persistent rhinorrhea, nasal endoscopy was performed revealing a gray polypoid mass in the right nasal cavity. β-trace protein concentration in the nasal secretions confirmed cerebrospinal fluid (CSF) leakage. Magnetic resonance imaging showed perforation of the lamina cribrosa with a large nasal encephalocele. The clinical presentation of growth retardation, recurrent infections, hyponatremia, and hypoalbuminemia could be explained by persistent loss of CSF. Following surgical correction of the defect, gradual recovery of the overall condition, including catch-up growth occurred. Reconstruction of perinatal care in this patient revealed complicated nasotracheal intubation immediately after birth, providing an adequate explanation for an acquired perforation of the lamina cribrosa.

Endoscopic management of nasal encephalocele in a 4-month-old baby

ABSTRACT A modified subcranial approach based upon the procedure first described by Joram Raveh is a viable alternative to standard craniofacial resection for treating pathology of the anterior skull base. It is basically an extradural approach providing wide exposure to the anterior skull base with minimal brain retraction; thus decreasing significantly the morbidity. We present here two cases of a rare benign pathology of anterior skull base-naso-orbito-ethmoid encephalocele wherein this technique was used successfully. How to cite this article Nagpal T. A Subcranial Approach for Anterior Skull Base Pathology (Nasal Encephalocele). Clin Rhinol An Int J 2014;7(1):20-22.

Nasal encephaloceles (meningoceles or meningoencephaloceles) are rare and not reported to be infected or coupled with a facial deformity in dogs. This report describes an older dog with acute worsening of seizures due to suppurative meningoencephalitis with coexisting suppurative rhinitis and infection of a meningoencephalocele. Additionally, the dog had a facial deformity for at least 5 years. The results of necropsy, computed tomography, and postmortem magnetic resonance imaging are compared. The development of nasal encephaloceles is discussed, including the potential role of early trauma, and whether separation of neural ectoderm from the surface ectoderm is part of the pathogenesis.

Nasal encephalocele with herniated anterior cerebral arteries in an adult: A technical case report with special emphasis on technique of management of herniated cerebral vessels

Encephaloceles are considered by most to be a type of neural tube defect characterized by a herniation of the brain and meninges through structural weaknesses in the bony structures of the skull. Many different types of encephaloceles have been classified according to the location of the bony defect. Basal and frontoethmoidal encephaloceles constitute a nasal subclass of encephaloceles, which are herniations from the skull base and ethmoid bone, respectively. Basal encephaloceles are usually occult and can herniate into nasal structures causing obstruction. Frontoethmoidal encephaloceles usually present as a protrusion that is visible at birth and enlarges during crying. Both of these entities are rare with an incidence ranging from 1 in 5,000 to 1 in 40,000 live births around the world with the majority of cases localized in Southeast Asia. Although cases have been recorded since the sixteenth century, there is still a scarcity of knowledge on the exact causes and factors associated with the development of the disease. Many studies have determined these to be caused by a combination of genetic and environmental factors. Most cases are non-life-threatening and the preferred method of treatment is surgical removal after diagnosis is confirmed with computerized tomography or magnetic resonance imaging. Prognosis is generally positive, especially in the subset of patients with frontoethmoidal encephaloceles.

The object of this study was to assess the efficacy and complications of endoscopic management of anterior skull base defects. The authors reviewed the medical records of 28 children (20 boys and 8 girls) undergoing endoscopic repair of anterior skull base defects in their tertiary referral center between 2001 and 2008; 18 cases were congenital and 10 cases posttraumatic. During the endoscopic procedure, rigid telescopes--2.7 or 4 mm in diameter, with 0° or 30° lenses--were used. In 23 patients the anterior skull base defect was sealed with fragments of middle turbinate (bone and mucosa). In the remaining 5 patients it was sealed with cartilage harvested from the nasal septum (3 cases) or from the auricle (2 cases), fibrin glue, and oxidized cellulose. A combined external and endoscopic approach was required in 3 cases because of the size and extensions of the encephalocele. Outcome was primarily assessed by means of clinical examination, nasal fibroscopy, and imaging. The mean duration of follow-up was 26.7 months (range 9-57 months). One patient treated by a combined approach died of meningitis 2 years after surgery. In the remaining 27 patients, there was no recurrence of CSF leak, meningitis, or encephalocele. An iatrogenic frontal or ethmoidal mucocele was observed in 4 cases. The endoscopic approach is a minimally invasive, safe, and efficient technique for removing nasal encephaloceles in children.

Nasal encephalocele: Endoscopic excision with anesthetic consideration

A 6-week-old boy presented with failure to thrive, pulmonary stenosis and stridor, which had worsened since birth. He also had undescended testes. On examination, he was noted to have facial asymmetry and his left eyelid did not close fully. He was noted to have a broad nose with a pit in the skin of his nose at the junction of the nasal bones and upper lateral cartilages in the midline from which hairs grew. His left ear was low set. He required laser laryngoplasty for severe laryngomalacia. Following investigations including computed tomography and MRI of the nose, brain and skull base, this pit was shown to continue up to the anterior skull base as a sinus tract. There was a defect in the skull floor anterior to the crista galli, and the crista galli itself was bifid. These clinical and radiological changes are consistent with the fibrous tract running from the nasal skin to the anterior cranial fossa floor being the embryological remnants of a nasal meningoencephalocele. Beneath the broad nasal bones was a widened nasal septum in the area enclosing the fibrous tract of the atrophied meningoencephalocele. The fibrous tract and nasal pit were removed from below and may require more extensive clearance if cysts or gliomas appear along the tract in the future. On review at 14 months, he had global developmental delay. He subsequently had feeding difficulties with delayed chewing skills and some aspiration. He had developed pigmentary mosaicism along the lines of Blaschko. Trisomy 14 mosaicism was diagnosed on skin biopsy

Nasal glioma (also known as nasal glial heterotopia) has been used to describe a congenital benign tumor of the nasal region containing neural tissue. It arises from failure of closure of foramen caecum at about the third week of gestation. The patient described in this report is an 18 months old girl who presented with a nasal bridge swelling for three months. The differential diagnosis included nasal encephalocele, nasal dermoid and epidermoid cysts. All are due to failure of ectoderm and neuroectoderm embryologic separation. CT scan and MRI imaging can be used to look for probable concomitant intracranial tumors and the existence of a connection between nasal tumor and the brain. Surgical resection is the usual method of managing such pathologies. In this case, an open rhinoplasty approach was used to resect this mass after the radiologic evaluation was complete.