Nasal encephaloceles presenting at later ages: experience of otorhinolaryngology department at a tertiary care center in Karachi, Pakistan.

Encephaloceles are anomalous herniations of the meninges, with or without brain matter. Globally the incidence of encephalocele is about 1 per 35,000 births, but it is more frequently reported in Southeast Asia. As the defect is more pertinent to embryological development, an encephalocele is a more common entity in an infant with a mean age of presentation ranging between 15.5 and 21 months; making an encephalocele presenting for the first time in a relatively, older individual a rare occurrence. Consequently a surgeon might not consider an encephalocele among his differentials. Here we present a series of encephaloceles that presented at a later than usual age as nasal masses to the otorhinolaryngology department of our hospital, and recommend that the differential of encephalocele be entertained for nasal masses as proceeding with routine procedures may result in potentially lethal complications.

Transsellar transsphenoidal encephalocele: a series of four cases.

Transsellar transsphenoidal encephalocele is the least common type of basal encephalocele. We present a series of four cases of transsellar transsphenoidal encephalocele. Clinical findings, imaging reviews, surgical repair techniques and postoperative morbidity are discussed with the relevant literature. Non contrast CT scan head with 3D reconstruction and magnetic resonance imaging should be done in all patients of transsphenoidal encephalocele. Endocrine assessment is also essential. Repair of a transsphenoidal encephalocele should be coordinated between a team of neurosurgeons and ENT surgeon. Our surgical outcome supports the transpalatal/ transnasal approach over the transcranial approach.

Hydrocephalus and Chiari type 1 malformation in macrocephaly-cutis marmorata telangiectatica congenita: a case-based update.

BACKGROUND: Macrocephaly-cutis marmorata telangiectatica congenita (M-CMTC) is a newly described condition characterized by macrocephaly (megalencephaly), cutis marmorata telangiectatica congenita, macrosomia and/or asymmetric growth, central nervous system abnormalities and neurological manifestations. DISCUSSION: This condition is usually documented in the Genetics' literature but it has been scarcely disseminated among neurosurgeons. Sudden death of uncertain origin has been reported in children with M-CMTC. Hydrocephalus and tonsillar herniation often occur in this syndrome. The appearance of symptoms and signs of intracranial hypertension or of brain stem compression in M-CMTC patients may herald the development of irreversible neurological damage or death. ILLUSTRATIVE CASES: We report two children diagnosed with M-CMTC and hydrocephalus who were given a ventriculo-peritoneal shunt. In addition, one of them exhibited tonsillar herniation. CONCLUSIONS: Given the high incidence of hydrocephalus and/or tonsillar herniation that occurs in M-CMTC, serial neuroimaging studies and neurosurgical referral seem to be warranted in children with this syndrome.

Survival with good outcome after cerebral herniation and Duret hemorrhage caused by traumatic brain injury.

Brainstem hemorrhage can occur as a primary or secondary event in traumatic brain injury (TBI). Secondary brainstem hemorrhage that evolves from raised intracranial pressure and transtentorial herniation is referred to as Duret hemorrhage. Duret hemorrhage following TBI has been considered an irreversible and terminal event. The authors report on the case of a young adult patient with TBI who presented with a low Glasgow Coma Scale score and advanced signs of cerebral herniation. She underwent an urgent decompressive hemicraniectomy for evacuation of an acute epidural hematoma and developed a Duret hemorrhage postoperatively. In accordance with the family's wishes, aggressive TBI monitoring and treatment in the intensive care unit was continued even though the anticipated outcome was poor. After a lengthy hospital course, the patient improved dramatically and was discharged ambulatory, with good cognitive functioning and a Glasgow Outcome Scale score of 4. Duret hemorrhage secondary to raised intracranial pressure is not always a terminal event, and by itself should not trigger a decision to withdraw care. Aggressive intracranial monitoring and treatment of a Duret hemorrhage arising secondary to cerebral herniation may enable a good recovery in selected patients after severe TBI.

Large supra- and infra-tentorial occipital encephalocele encompassing posterior sagittal sinus and torcular Herophili.

Occipital encephaloceles are the most common type of encephalocele encountered in the Western Hemisphere. The occipital encephalocele is typically classified according to the relationship of the herniated tissue to the torcular. We report the unusual case of a premature infant with a rare, large occipital encephalocele which encompassed the posterior superior sagittal sinus and torcular. We discuss the variable venous anatomy with occipital encephaloceles and review the different options to employ in their surgical repair.

[Early epidural hematoma after CSF shunt for obstructive hydrocephalus]. / Hématome extradural précoce après dérivation interne du liquide céphalorachidien pour hydrocéphalie non communicante.

We present the first case of early epidural hematoma after CSF shunt probably caused by defective material. A 26-year-old man was treated for obstructive hydrocephalus associated with a tonsillar herniation, revealed by headaches and papillary edema. Ventriculoperitoneal shunt was preferred to endoscopic ventriculostomy. Three hours after the operation, the patient fell into a coma, developing a voluminous bifrontal epidural hematoma that was evacuated immediately. The patient completely recovered neurologically. One month later, to treat persistent hydrocephalus, endoscopic ventriculostomy was performed without incident. Then the shunt was removed and an opening threshold close to zero was discovered. Distant MRI showed a reduction in ventricular size, normalization of the tonsils' position and a tumor of the tectal plate. To our knowledge, this is the only case of early epidural hematoma after ventriculoperitoneal shunt. We discuss the choice of treatments for obstructive hydrocephalus and its risks and complications.

Comparison of Dural Splitting and Duraplasty in Patients with Chiari Type I Malformation: Relationship between Tonsillo-Dural Distance and Syrinx Cavity.

AIM: To compare the clinical and radiological results of dural splitting and duraplasty in patients with Chiari Type I Malformation. MATERIAL AND METHODS: This study includes 113 adult patients with Chiari Type I malformation treated between 2009 and 2013. The patients were divided into two groups according to the surgical method (Group 1: dural splitting, Group 2: duraplasty). Neurological examinations and magnetic resonance imaging (MRI) scans were recorded periodically on 3rd, 6th,and 12th months at the postoperative period. The tonsillo-dural distance (TDD) and regression rate of the syrinx cavity were measured on T1 and T2 weighted sagittal MRI scans at the postoperative period. RESULTS: The ratio of syrinx regression was %49.6 in the Group 1 and %54.6 in the Group 2. This result was statistically significant. The TDD increased in Group 2 and this result was statistically significant (p < 0.05). Postoperative pain and numbness decreased in both groups and no statistically significant difference was detected. CONCLUSION: There was no relationship between tonsillar herniation length and the width of syringomyelic cavity. The syrinx cavity more regressed in the group 2 than group 1. There was no relation between the TDD and the ratio of syrinx regression.

[Endoscopic treatment of cerebral rhinorrhea]. / Endoskopowe leczenie plynotoku nosowego.

INTRODUCTION: The frequency of different factors causing cerebrospinal fluid rhinorrhea (CFR) has lately changed. The incidence of iatrogenic CFR has reached 10% of all cases of CFR, due to an increasing number of endoscopic operations of the sinuses and skull base, while idiopathic CFR is nowadays very rare. The current treatment method for CFR is surgical repair of the fistula. Endoscopic surgery of the anterior skull base has become the standard procedure for the repair of cerebrospinal fluid (CSF) leaks of various origins. The aim of this study was to analyse results of endoscopic surgical technique used in our department for the treatment of CFR. MATERIAL AND METHODS: Records of 5 patients aged from 46 to 69 (mean 58.2) years treated in the department between April 2004 and March 2006 were analysed retrospectively. 4 individuals had underwent endoscopic sinus surgery for sinus problems which resulted in iatrogenic CSF leak. One patient had idiopathic CFR. 3 fistulas localised in the neighbourhood of the cribriform plate were closed using an "underlay" technique with synthetic dura, and covered with free mucosal grafts from the nasal septum, kept in place by fibrin glue. The fistula in the neighbourhood of the sphenoid sinus posterior wall was closed using an ,,overlay" technique with surgical, covered with synthetic dura. RESULTS: In the 3 patients with cribriform plate fistulas the closure was successful and CFR did not recur during 6 to 9 month's follow-up. In the patient with sphenoid sinus fistula CFR recurred on exertion after 4 months. In one patient with cribriform plate fistula, CFR resolved spontaneously during preparation to surgery. CONCLUSIONS: Endoscopic closure of the skull base fistula represents a minimally invasive and highly successful procedure. Our experience suggests thet the optimal surgical technique in the region of cribriform plate consists in performing an "underlay" procedure with synthetic dura and covering the graft with free mucosal grafts from the nasal septum.

[Intradiploic encephalocele in an adult. A case report and review of the literature]. / Encéphalocèle intra-diploïque de l'adulte. A propos d'un cas et revue de la littérature.

We describe a rare case of parietal intradiploic encephalocele in a 51-year-old woman with no history of head trauma. The patient presented with a 1-month history of left hemiparesthesia. A hard indolent scalp lesion was palpable on examination in the right parietal area. Skull x-rays and cranial computed tomography examination demonstrated a lytic lesion that was consistent with a malignant osteolytic skull lesion. Magnetic resonance imaging of the head revealed an intradiploic cyst that included a round tissue mass contiguous with the parietal cortex. Surgery confirmed the diagnosis of encephalocele and the patient underwent surgical resection of the herniated brain, duraplasty, and cranioplasty. The presenting hemiparesthesia persisted at the 6-month follow-up. Encephaloceles of the cranial vault are a rare complication of skull fractures and rarely occur in adults. These lesions can be difficult to distinguish from congenital encephaloceles in patients with no history of head trauma. The pathogenesis, clinical and radiological nuances and the role of surgery are discussed.

Parietal atretic cephalocele: Associated cerebral anomalies identified by CT and MR imaging.

We describe a case of atretic cephalocele (AC) characterized by the presence of various cerebral anomalies of different midline structures. In our patient the presence of a parietal AC was associated with an embryonic position of the straight sinus, fenestration of the superior sagittal sinus, an abnormal insertion of the cerebellar tentorium with prominence of the superior cerebellar cistern and a septum pellucidum cyst. These findings, associated with AC, could lead to a worse prognosis with regard to neurodevelopmental milestones. This suggests that even if AC is a benign lesion, a complete evaluation of the brain structures should always be performed in these young patients.

Spontaneous delayed brain herniation through a subdural membrane after tumor surgery.

BACKGROUND AND IMPORTANCE: We report on a rare case of spontaneous cerebral herniation through a subdural membrane in a 54-year-old patient. Brain herniation in adults as a complication of chronic subdural hematomas shortly after a neurosurgical intervention is rare. We are the first to report a case of delayed local herniation in an adult patient more than 1 year after a neurosurgical procedure. CLINICAL PRESENTATION: The patient suffered from a low-grade oligodendroglioma since 1993. Radiotherapy was then applied, followed by resective surgery and chemotherapy in 2008 because of tumor progression. Subsequently, he developed a symptomatic subdural hygroma treated with a subduro-atrial cerebrospinal fluid shunt. In January 2010, the shunt was occluded. Follow-up brain imaging showed a stable situation after tumor resection, with a cyst in the temporal resection cavity and a stable subdural hygroma. In February 2011, the patient visited the emergency department because of an acute right hemiparesis and progressive motor aphasia. Urgent magnetic resonance imaging was suspicious of a herniation of brain parenchyma in the left middle cranial fossa. Explorative surgery showed a locally incarcerated brain herniation through a membrane with a ring-like aperture. Resection of this membrane led to normalization of the position of the brain tissue and to clinical improvement. CONCLUSION: Brain herniation through a subdural membrane is an extremely rare complication, but must be a differential diagnosis in patients with a known chronic subdural hematoma or hygroma and clinical deterioration, even in the absence of recent surgery. Urgent surgical intervention of the herniated brain is recommended to reduce the risk of permanent neurological damage.

Tectocerebellar dysraphism with vermian encephalocele.

Tectocerebellar dysraphism is a very rare malformation associated with encephalocele and tectal deformity. This article presents tectocerebellar dysraphism with a solely vermian content of encephalocele and tectal beaking defined by magnetic resonance imaging (MRI) in a 5-month-old girl who was successfully treated surgically and demonstrated excellent prognosis at the 4-year follow-up.

Occult middle fossa encephaloceles in patients with temporal lobe epilepsy.

OBJECTIVE: Middle fossa encephaloceles are rare structural defects previously reported to cause complex partial seizures. Their debated etiology is either by failed union of temporal and sphenoid bone ossification centers or by erosion of the middle fossa floor secondary to pressure phenomena. Although magnetic resonance imaging (MRI) often reveals abnormalities, the actual encephalocele may not be identified preoperatively. METHODS: We present three cases of middle fossa encephaloceles that were identified intraoperatively and provide a review of the relevant literature. RESULTS: All three of our case presentations demonstrate patients with medically intractable epilepsy and intraoperative findings of middle fossa encephaloceles. In all patients, careful retrospective analysis of preoperative imaging provided clues to these encephaloceles, although none were suspected before surgery. After resection of the area all patients had improved seizure outcome. CONCLUSION: Middle fossa encephaloceles should be recognized as a potential source of epileptic pathology in patients with complex partial seizures. Although only 12 cases are reported in the literature, we believe that this phenomenon may be more common than previously recognized. We suggest that simple resection of the encephalocele alone may result in long-lasting, excellent seizure outcomes without amygdalohippocampectomy.

Syrinx disappearance following laminoplasty in cervical canal stenosis associated with Chiari malformation--case report.

A 50-year-old woman presented with nuchal pain, clumsiness in both hands, and gait disturbance. Cervical magnetic resonance (MR) imaging showed cervical canal stenosis at C3-C7 levels associated with a syrinx and cerebellar tonsillar herniation. The patient underwent C3-C7 laminoplasty. Her symptoms improved completely, and the patient was discharged 10 days after operation. Postoperative MR imaging showed disappearance of the syrinx. This case suggests that cerebellar tonsillar herniation may not cause syringomyelia. Posterior decompression, such as laminectomy and laminoplasty, without foramen magnum decompression may be an appropriate surgical strategy for diffuse cervical canal stenosis associated with syringomyelia below the narrow canal, even in the presence of co-existing Chiari malformation.

Transethmoidal meningoencephalocele involving the olfactory bulb with enlarged foramina of the lamina cribrosa--case report.

A 3-year-old girl presented with a transethmoidal meningoencephalocele manifesting as recurrent rhinorrhea. Initially, she developed meningitis, but after treatment she experienced rhinorrhea. Two months later, she again presented with rhinorrhea. Neuroimaging studies revealed a small protrusion (15 mm x 10 mm) at the roof of the ethmoidal sinus. Nasal endoscopy confirmed the diagnosis of meningoencephalocele. The operative findings revealed a small hole in the left olfactory bulb, which had descended into an enlarged foramen along with the arachnoid membrane. The left olfactory bulb was removed, and the enlarged foramina of the lamina cribrosa were covered with a frontal pericranial flap. The defect in the bone was very small, but contributed to the development of meningitis and leakage of the cerebrospinal fluid. Basal cephalocele should be considered in a patient with recurrent rhinorrhea and intracranial infections, even in the absence of any apparent anomaly.

Fourth ventriculoceles with extracranial extension: one case report.

Encephaloceles involving only the herniated fourth ventricle are exceedingly rare. We report a case of such a cranial malformation and review the literature regarding these malformations and their potential embryological derailment.