Surviving against the odds: exploring the clinical and radiological features of iniencephaly compatible with life. Illustrative case.

BACKGROUND: Iniencephaly is a rare neural tube defect (NTD) characterized by deformities in the occiput and inion, along with rachischisis in the cervical and thoracic spine, resulting in the head appearing in retroflexion. OBSERVATIONS: This report details the case of a female newborn who underwent surgery for an encephalocele. She survived up to 6 months, exhibiting good overall health, although she displayed physical abnormalities, including facial deformity, a short neck, and minor spasms in all limbs. Both cardiovascular and abdominal assessments remained stable, and imaging revealed defects in the occipital bone, a large cephalocele, and spinal dysraphism. LESSONS: Although iniencephaly is generally incompatible with life, a few cases have been reported otherwise. Our patient, one of these notable exceptions, remains alive at 6 months old, possibly due to the lack of major vascular deformities. However, she does exhibit significant psychomotor retardation.

Giant supra and retrosellar glioependymal cyst presenting with only precocious puberty. Clinical study and review of the literature.

INTRODUCTION AND IMPORTANCE: Intracranial glioependymal cysts are an uncommon type of neuroepithelial cyst and are encountered much less frequently than arachnoid cysts. These cysts primarily manifest within the parenchyma of the brain, although exceedingly rare instances have been reported in the lateral ventricles. CASE PRESENTATION: We present a highly unusual case of a glioependymal cyst in a 7-year-old girl. The glioependymal cyst was located in the midline in the suprasellar region and extended to the upper clivus region. Its only manifestation was precocious puberty. We performed endoscopic fenestration of the cyst, leading to a return of hormonal levels to normal and a slight reduction in cyst size. CLINICAL DISCUSSION: A comprehensive search of the Medline database revealed only a few documented cases of glioependymal cysts (fewer than 30 cases). Remarkably, the majority (if not all) were located laterally rather than in the midline of the brain. Endoscopic fenestration and biopsy are effective and confirm the diagnosis. CONCLUSION: This instance of a rare glioependymal cyst located in the midline, spanning the suprasellar and retrosellar regions, is an uncommon occurrence. Its sole presentation was precocious puberty. The successful management of this condition was achieved through an endoscopic approach, leading to the normalization of endocrine abnormalities.

A rare entity of primary hydatid cyst located between the two layers of the intracranial dura in a child: a case report.

INTRODUCTION: Hydatid disease is a parasitic infection caused by the tapeworm Echinococcus granulosus. Intracranial locations are rare and account for less than 3% of all cases. Typically, these cysts are found in the intracerebral spaces. However, this study presents an extremely rare intradural hydatid cyst. To our knowledge no similar case has been previously reported. CASE PRESENTATION: This study presents the case of an 8-year-old boy presented with a 3-month history of headache and vomiting without any neurological deficit. Full radiological investigations were performed, the brain MRI showed a large cerebral hydatid cyst located within the dura layers between the periosteal and the endosteal layers. Surgery was performed without cyst rupture, confirming the intracerebral intradural location. CONCLUSION: Early diagnosis and treatment for intracranial hydatid cysts are crucial to prevent complications such as neurological deficits, seizures, and even death. In this case, the intracerebral intradural location of the cyst is extremely rare.

Primary non-Hodgkin lymphoma of the chiasm and optic tract in a nonimmunocompromised patient: illustrative case.

BACKGROUND: Chiasmatic and optic track lymphoma as the primary lesion of the central nervous system (CNS) is extremely rare. OBSERVATIONS: The authors report a case of a previously healthy 62-year-old woman who presented with quick and progressive visual impairment leading to bilateral blindness. Brain imaging studies suggested glioma or lymphoma of the chiasm and the posterior visual pathway. Postoperative examination revealed low-grade malignant B-cell lymphoma. No evidence of extracranial lymphoma was found, so a final diagnosis of primary CNS lymphoma (PCNSL) was made. LESSONS: To the authors' knowledge, PCNSL confined to the optic chiasm has rarely been reported in nonimmunocompromised patients. The present case of lymphoma affecting the optic chiasm and optic tract is extremely rare.

Bobble-head doll syndrome in an infant with an arachnoid cyst: a case report.

BACKGROUND: Bobble-head doll syndrome is a rare and unique movement disorder most commonly affecting children younger than 5 years of age. It is characterized by continuous or episodic movement at the frequency of 2-3 Hz. The exact mechanism of bobble-head doll syndrome has not been elucidated. Endoscopic ventriculocisternostomy is the optimal treatment option. In a literature review, there were less than 75 cases of bobble-head doll syndrome with suprasellar arachnoid cyst. CASE PRESENTATION: We report a case of a 1.5-year-old Asian-Syrian girl who presented with a history of excessive head nodding for 3 months that increased with walking, emotions, and stress; decreased during periods of concentration; and was absent during sleep. On physical examination, she was alert and normal, with no medical history. Laboratory assessment and ophthalmological examination were normal. Cranial magnetic resonance imaging demonstrated a well-defined thin-walled suprasellar arachnoid cyst measuring 3 × 5 × 7 cm that obstructed the foramina of Monro, with resulting hydrocephalus ventriculomegaly. The patient underwent endoscopic cystoventriculostomy and cystocisternostomy for the suprasellar arachnoid cyst. During the 6 months of follow-up, the head bobbing disappeared completely, and her growth was normal. CONCLUSION: Despite the rareness of bobble-head doll syndrome, it is considered an important condition that must be investigated early to detect the cause and treated promptly to avoid potential complications.

The incidence, timing, and predisposing factors of germinal matrix and intraventricular hemorrhage (GMH/IVH) in preterm neonates.

BACKGROUND: Germinal matrix and intraventricular hemorrhage (GMH/IVH) is a known complication occurring in the first week of life in preterm neonates. However, the precise time of its occurrence and the ideal time to perform diagnostic imaging studies remain controversial. The purpose of this paper is to address these two issues in our patient population to allocate our resources to those at highest risk. MATERIALS AND METHODS: This study included 282 premature newborns (under 37 weeks of gestation) that were admitted to our neonate ICU in a year's time and screened for GMH/IVH. They were grouped in four categories according to their weight at birth, and according to their gestational age. All patients had a daily cranial ultrasound during the first week. It was then repeated once in the second week and once in the third. RESULTS: We found that the incidence of GMH/IVH among preterm neonates was 44.68%. It was inversely related to the weight and the age of the newborn. The onset of bleeding coordinated with the occurrence of hypoxia and respiratory distress requiring mechanical ventilation. The majorities occurred in the first 7 days of life; they were mostly grade I and II according to the Papule classification and silent for the most part. Complications were present in 41% of the survivors.

Incidence of childhood brain tumors in Syria (1993-2002).

OBJECTIVE: To determine whether the incidence and location of childhood CNS tumors in Syria follows the same pattern described in Western and Far Eastern countries. PATIENTS AND METHODS: We analyzed the data compiled from 367 children with brain tumors operated on in our Department of Neurosurgery between 1993 and mid-2002. We excluded all vascular and metastatic lesions and adopted the latest WHO classification in grouping all glial tumors. RESULTS: We found that 47% of brain tumors were located in the supratentorial, and 53% in the infratentorial region. The ratio of male to female occurrence was 1:1.2 (52% males, 48% females). For lesions in the supratentorial space, the distribution was 56% males and 44% females, while in the posterior fossa, the distribution was 61% males and 39% females. Low-grade tumors (WHO I/II) constituted 53.5% of all lesions, and the rest were high grade tumors (WHO III/IV) 46.5%. The most common tumor found in our childhood population was medulloblastoma (27.5%), followed by astrocytoma (25.8%), then craniopharyngioma (14.1%). The most common tumor in the posterior fossa was medulloblastoma (53.5%), followed by astrocytoma (22.5%), then ependymoma (17%). The most common tumors in the supratentorial space were astrocytoma and craniopharyngioma. CONCLUSIONS: In our patient population, the incidence and distribution of CNS tumors were somehow different than those reported by authors from the Western and Far Eastern countries. Whether these results are unique to Syria, or reflect a regional difference in the disease distribution between the Middle East region and the rest of the world, remains to be determined.

Late appearance of hydrocephalus associated with posttraumatic intradiploic arachnoid cyst.

CASE REPORT: We present a case of a posttraumatic intradiploic arachnoid cyst associated with hydrocephalus in a 16-year-old boy. The diagnosis was missed 2 years previously, when an MRI following a transient gait disturbance failed to show the intradiploic extension of the cyst. He presented to us with hydrocephalus and a CT scan clearly identified the intradiploic position of the cyst. OUTCOME: A fenestration between the lateral ventricle and the cyst with aqueductoplasty and ETV were performed with good results.