Rare Brain Tumors with Infrequent Clinical Manifestations: Illustrative Cases

This chapter describes the epidemiology, clinical and neuroimaging features, histological characteristics, surgical approach, outcomes, and prognostic factors of different cases of very rare intracranial tumors, associated with complex clinical syndromes. Highlighting the important aspects in the diagnosis and management that were considered relevant through the experience of our center. Here we included an intracranial Rosai-Dorfman disease manifested as an apparent multiple meningiomatosis, a choroid plexus papilloma clinically manifested as a hemifacial spasm originated by a compression of the facial colliculus, and a neuroenteric cyst associated with Klippel-Feil syndrome. This type of tumor presents a challenge to the neurosurgeon, originating various questions about its management. In this chapter, we present the experience we had with these pathologies to establish the most appropriate management decisions.

Tetrad of Split Cord Malformation I with Neuroenteric Cyst, Dermoid Cyst, and Thickened Filum Terminale in a 2-Year-Old Child: A Case Report

<b><i>Introduction:</i></b> Split cord malformations (SCMs) are developmental anomalies that are associated with a number of congenital defects. However, a combination of SCM I with a neuroenteric cyst (NEC) is extremely rare, and only 11 cases have been described in the literature. To the best of authors’ knowledge, the combination of the above two with dermoid cyst and thickened filum terminale has never been reported in the literature. <b><i>Case Presentation:</i></b> We present a case of the above combination in a 2-year-old child who underwent microsurgical excision of all 4 pathologies and complete recovery. <b><i>Conclusion:</i></b> NEC and dermoid should be considered in the differential diagnosis when imaging reveals cystic pathology along with SCM. Expeditious surgical repair resulted in an outstanding functional outcome at 1-year follow-up.

Anesthetic management of cesarean section of a wheelchair bound parturient with myelomeningocele

Neural tube defects (NTD) affect 1-2 in 1000 pregnancies over the world. The most frequent spinal dysraphisms are meningocele and myelomeningocele while the spina bifida occulta, tethered cord, lipomyelomeningocele, split cord malformations, neuroenteric cyst, and several other conditions occur less frequently. Open spinal dysraphisms (meningocele and myelomeningocele) are compatible with postnatal survival, but they can result in severe neurologic impairment as inability to ambulate, urinary incontinence, hydrocephalus, scoliosis, and gastrointestinal (GI) disorders. Closed spinal dysraphisms (spina bifida occulta, tethered cord, lipomyelomeningocele, split cord malformations, neuroenteric cyst) are often less severe and sometimes asymptomatic but may result in severe neurologic impairment secondary to spinal cord tethering. Modern health care facilities has resulted in a significant decrease in the mortality rate in patients with SB2 along with improved survival rate up to 80%. Due to the neurologic complexity and severity of these conditions, it is paramount to understand the challenges and offer the best anesthetic approach for these patients. To the best of our knowledge, this is the first case of SB going under caesarean delivery in our hospital. We wish to highlight our experience of perioperative management of this patient. Key words: Spina bifida, anesthetic management Citation: Lal S, Aisa T, Akram U, Sher A, Memon Z, Moriarty RM. Anesthetic management of cesarean section of a wheelchair bound parturient with myelomeningocele. Anaesth. pain intensive care 2020;24(5): Received: 13 June 2020, Reviewed: 24 June 2020, Revised: 18 July 2020, Accepted: 8 August 2020

A RARE CASE OF NEUROENTERIC CYST INVOLVING THE ANTERIOR FOSSA; REPORT OF A CASE AND REVIEW OF LITERATURE

Neuroenteric cysts, are rare benign endodermal lesions which mostly occur in the central nervous system. We report a case of a neuroenteric cyst in a 30-year-old man who presented with rhinoliquorrhea at the ENT department. After clinical examination, a semisolid mass was revealed in the left nostril. The Computer Tomography Scan revealed a frontal ethmoidal nasal meningoencephalocele with inferior extension into the left nostril. Gadolinium-enhanced T1-weighted MR images showed a well-defined frontal mass with ring-like enhancement and extension into the cribriform plate of the ethmoid bone and into the left nostril. The lesion measured 10/10/20 mm. The tumor was totally resected using a unilateral subfrontal approach. At five months’ follow-up, the patient showed significant amelioration of symptoms and remission of cerebrospinal fluid leakage. Native and Contrast-enhanced Cerebral Computer Tomography, as well as Magnetic Resonance Imaging showed total surgical resection of the cyst. Supratentorial neurenteric cysts involving the anterior fossa are rare. Intracranial neurenteric cysts should be differentiated by any well-demarcated cystic tumors. The gold standard treatment remains complete surgical resection with favorable outcome.

Resection of a Neuroenteric Cyst Using a Far Lateral Approach

This operative video highlights a rare case of a neuroenteric cyst at the ventral craniocervical junction. The case involved a 30-year-old man who initially presented 13 years earlier with acute onset of headache and visual changes. At that time, he was found to have a small, enhancing ventral intradural extramedullary mass at the rostral aspect of C1 thought to be a meningioma. The lesion was managed conservatively, and surveillance imaging tracked its slow progressive enlargement to a size of 1.4 cm (Fig. 1A, B). Although he remained asymptomatic, nonurgent elective resection was recommended because of his age and mass progression. The patient underwent a left far lateral approach to the craniocervical junction for resection of the mass. This involved dissection of the suboccipital musculature to expose the C1 transverse process in the suboccipital triangle and ultimately the vertebral artery. After a small craniectomy and C1 hemilaminectomy, the dura was opened and a cystic lesion encountered (Fig. 2). The cystic contents were debulked and the capsule resected. Histopathologic examination revealed abundant goblet cells consistent with a neuroenteric cyst. Dural closure was bolstered with fascia lata and autologous fat graft. Postoperative magnetic resonance imaging (MRI) was consistent with gross total resection (Fig. 1C, D). The patient tolerated the procedure well with no new postoperative neurological deficits and was discharged home on postoperative day 2. On completing a 3-day decadron taper, he developed steroid-responsive symptoms consistent with aseptic meningitis, possibly related to cerebrospinal fluid contamination with the cyst contents during resection.The link to the video can be found at: https://youtu.be/SskETPe5PXQ.