Transsphenoidal Meningoencephalocele Research Articles

Transalar transsphenoidal meningoencephalocele presenting in the form of recurrent meningitis: Report of two cases and discussion of the diagnosis and treatment

IntroductionSkull base meningoencephaloceles are rare congenital malformations. The two cases of transalar transsphenoidal malformation reported here differ from the classical transsphenoidal meningoencephalocele. Case reportsCase 1 was a three-and-a-half-year-old boy and case 2 was a 36-year-old man. Both cases presented with clinical features of recurrent meningitis. Surgical management of case 1 was performed via an intradural infratemporal fossa craniotomy with reoperation 2years later. In the second case, surgery was initially performed via an endonasal approach and then via pterional craniotomy. Reoperation via Sekhar's transpetrosal approach was also a failure. Only closure of the trigeminal-pontine angle via a suboccipital retrosigmoid approach allowed repair of the defect. DiscussionSurgical access to the pterygopalatine fossa is complex due to its anatomical position and its anatomical relations with nerves and vessels. An endoscopic approach appears to be a valuable alternative to classical craniotomy. ConclusionIn the two cases reported here, neurosurgery allowed lasting closure of the defect.

Endoscopic repair of transsellar transsphenoidal meningoencephalocele; case report and review of approaches

Abstract We present an extremely rare case of transsellar transsphenoidal meningoencephalocele in a 36-year-old woman with pituitary dwarfism complaining of nasal obstruction. Imaging studies showed a bony defect in the sellar floor and sphenoid sinus with huge nasopharyngeal mass and 3rd ventricle involvement. Using endoscopic endonasal approach the sac was partially removed and the defect was reconstructed with fat and fascial graft, and buttressed with titanium mesh and septal flap. Visual field improvement was noticed post-operatively and no complication was encountered during follow-up. So, endoscopic endonasal approach with partial resection of the sac is a safe and effective treatment for this disease

Transsphenoidal meningocele: an anatomical study using human fetuses including report of a case

An asymptomatic transsphenoidal meningoencephalocele was discovered incidentally by fiber laryngoscopic examination in a 62-year-old man suffering from hoarseness due to dysplasia of the vocal cord epithelium. To provide a better understanding of the pathogenesis of this anomaly, we performed histologic observations of paraffin-embedded specimens of 42 human fetal heads at 12-16 weeks of gestation. At these stages, ossification had started in the clivus but the sphenoid sinus was not developed. In contrast to the very low incidence of the intra- or trans-sphenoidal remnant of Rathke's pouch after birth, we found (1) the typical mid-line cleft of the sphenoid body in two specimens (2/42 or 4.8 %) and (2) a duct-like, sellar inferior protrusion ending in the sphenoid body in 12 specimens (12/42 or 28.6 %). The cyst-like structure in the protrusion (two specimens) seemed to be composed of obstructed veins. The intra- and trans-sphenoidal anomalies were observed more frequently in specimens without ossification of the sphenoid body than in those with ossification. However, irrespective of ossification, a cyst-like remnant of the most upper part of Rathke's pouch was always seen between the anterior and posterior lobes of the developing pituitary gland. In addition, the bursa pharyngea was seen in four specimens and we confirmed that the notochord was attached to the bursa in each case. The consistent remnant of the intrasellar Rathke's pouch appeared to explain the high incidence of Rathke's cleft cyst in adults. The relatively high incidence of intrasphenoidal anomalies in fetuses (14/42) suggested that the intra- or trans-sphenoidal remnant of Rathke's pouch was physiologically closed by ossification of the sphenoid body.

Rathke’s pouch remnant and its regression process in the prenatal period

During ontogeny, part of Rathke's pouch, a physiologically superior pouching of the stomodeal ectoderm, may remain, forming the usual anomaly known as Rathke's cleft cyst. More rarely, however, the entire pouch (i.e., the craniopharyngeal canal) remains, resulting in transsphenoidal meningoencephalocele. This study is aimed to provide a better understanding of the pathogenesis. We histologically evaluated the inferior protrusion of the putative hypophysial fossa in 35 embryonic and fetal heads, respectively (15 at 5-8weeks and 20 at 12-16weeks of gestation). In 3 of the 35 specimens, we observed a complete cleft originating from the adenohypophysis, passing through the sphenoid and connecting with the pharyngeal epithelium or pharyngobasilar fascia. In another 18 specimens, we observed a duct-like structure protruding from the fossa and ending in the sphenoid. The sellar protrusion contained vein-like structures and debris of red blood cells. The protrusion was located on the anterior or anterosuperior side of the notochord. No ossification center was observed around the sellar region of the embryos. Although ossification occurred in 12 of the 20 fetuses, nine of the latter showed no evidence of cleft or protrusion, indicating that the incidence of Rathke's pouch remnant was lower in the fetuses (11/20) than in the embryos (11/15). Rathke's pouch may be closed by ossification of the sphenoid, but increased cell proliferation and/or large amounts of degenerated veins may provide a structure resistant to the mechanical pressure caused by ossification.

Temporobasal, Transsphenoidal Meningoencephalocele Becoming Symptomatic with Spontaneous Cerebrospinal Fluid Rhinorrhea: Diagnostic Work-up and Microsurgical Strategy

We report the rare case of an adult transsphenoidal meningoencephalocele and outline the microneurosurgical strategy. Clinical history, the findings of computerized tomography (CT) scans and magnetic resonance imaging (MRI), the microsurgical procedure, and histopathology are reported. A 54-year-old female patient complained about cerebrospinal fluid (CSF) rhinorrhea; a transnasal biopsy of a mass in the maxillar sinus prior to diagnostic work-up was performed elsewhere. Persisting CSF leakage prompted CT and MRI, which showed brain tissue extending from the left middle cranial fossa into the left sphenoid sinus through several bony defects. The diagnosis of a transsphenoidal meningoencephalocele was made, and the lesion was targeted via a subtemporal intradural approach with resection of the herniated brain tissue and closure of the bony as well as of dural defects. The postoperative course was uneventful without recurrence of the CSF fistula. The transsphenoidal subtype of basal meningoencephaloceles is exceedingly rare. Nonetheless, it has to be considered as a differential diagnosis if a nasal or intrasphenoidal mass is diagnosed. Otherwise, unjustified biopsy or unsuccessful management of CSF leakage could not be avoided. The intradural subtemporal approach is effective to treat the transsphenoidal type of basal meningoencephaloceles.

Transsphenoidal Meningoencephalocele: Challenge of Early Transsphenoidal Repair

Background: Transsphenoidal meningoencephalocele is a rare congenital anomaly, reported in fewer than 50 patients. It may be subdivided into intrasphenoidal, extending into the sphenoid sinus, and true transsphenoidal, traversing the floor of the sinus and protruding into the nasal cavity or nasopharynx. Surgical treatment of true transsphenoidal meningoencephalocele is debated.

Transsphenoidal meningoencephalocele associated with cleft lip and palate: challenges for diagnosis and surgical treatment

The aim of this study was to highlight the challenges for early diagnosis and the difficulties observed in surgical treatment of patients with transsphenoidal meningoencephalocele associated with cleft lip and/or palate. We evaluated six male patients treated over the course of 4 years. Five patients presented encephalic herniation with nonfunctional brain tissue; one of these presented herniation of the pituitary gland and cerebral ventricles. All the patients received surgical treatment for the cleft lip and/or palate. Only one patient underwent repair of the meningoencephalocele, via nasal endoscopy. There were no postprocedural clinical or surgical complications. The tendency is to avoid neurosurgery, opting for periodic follow-up with magnetic resonance imaging. In the presence of cleft palate, palatoplasty is essential to protect the meningoencephalocele.

Optic disc coloboma (the morning glory syndrome) and optic nerve coloboma associated with transsphenoidal meningoencephalocele

The association optic disc coloboma (the morning glory syndrome), optic nerve coloboma, and transsphenoidal meningoencephalocele is extremely rare. We describe an original case of optic disc coloboma in association with optic nerve coloboma, microphthalmos, optic nerve hypoplasia, and transsphenoidal meningoencephalocele. There were no midfacial anomalies (hypertelorism, broad nasal root, cleft lip, and cleft plate) and pituitary dysfunction. For patients who had optic disc-nerve coloboma without midfacial anomalies, neuroimaging should be prompted to look for transsphenoidal encephalocele/meningoencephalocele and other anomalies. We present demonstrative fundoscopic pictures, ultrasound and magnetic resonance imaging of this original case of a 7-year-old patient.

Transsphenoidal meningoencephalocele in adults

BACKGROUND Transsphenoidal meningoencephalocele is a rare congenital anomaly, reported only in 14 adult patients. It may be subdivided into intrasphenoidal, extending into the sphenoid sinus, and true transsphenoidal, traversing the floor of the sinus and protruding into the nasal cavity or nasopharynx. METHODS We present the eighth case of true transsphenoidal meningoencephalocele reported in an adult. We review the literature and highlight the differences in clinical presentation and therapeutic management between patients with intrasphenoidal and true transsphenoidal meningoencephalocele. CONCLUSION Adult patients with intrasphenoidal meningoencephalocele often present with rhinorrhea and should be considered for transsphenoidal repair, whereas patients with true transsphenoidal meningoencephalocele should not undergo surgery, in view of the complex anatomy and the slow progression of symptoms.