A 9-month-old baby boy presented with his parents to the emergency department with the inability to follow objects, repeated vomiting, and irritability for three days. He is an outcome of uneventful full-term gestation. His postnatal history was unremarkable and neurodevelopmental milestones were normal.

On examination, he was dehydrated and frequently cried. His head circumference was in the upper 90 percentiles for age, but the anterior fontanel was small with no evidence of increased intracranial pressure. There were no stigmata of neurofibromatosis type 1 (NF1). The neurological examination revealed adequate power and tone in all extremities. There is a poor pupillary response to light and no clear visual recognition of moving objects. There was no ophthalmoplegia or facial weakness, and the corneal and gag reflexes were brisk.

Urgent computed tomography (CT) scan revealed a large hyperdense suprasellar lesion and mild ventricular dilatation (Figure 1). A magnetic resonance imaging (MRI) scan revealed large sellar and suprasellar cystic and solid lesions containing subacute components of blood products. The lesion has marked heterogeneous enhancement following intravenous contrast administration, and there is marked mass effect and mild ventriculomegaly (Figure 2). Magnetic resonance angiography (MRA) revealed no vascular abnormality.

The baby had normal routine laboratory workup with particularly no coagulopathy or pituitary dysfunction on admission. He was admitted to the pediatric intensive care unit (PICU) to optimize her general condition. Ophthalmological consultation documented his poor visual response. He was started on dexamethasone (2 mg intravenous injection followed by 0.5 mg hourly). The surgery was performed the next day on the right frontotemporal craniotomy. The dura was open curvilinear and reflected anteriorly. There was no significant brain swelling, and there was no need to insert a ventricular drain. The optic-carotid cistern and sylvian fissure were widely opened, which made more brain relaxation. The tumor was yellowish firm, with areas of soft cystic consistencies. Intratumoral microscopic decompression of the suprachiasmatic and hypothalamic solid and cystic parts was achieved using an ultrasonic aspirator. There were mixed-blood products of recent and old components within cystic regions. After achieving adequate decompression, craniotomy closure was done in layers. The patient was transferred to PICU in stable condition with an estimated blood loss of around 120 ml. He received an intraoperative blood transfusion of 100 ml.

The patient had an uneventful postoperative period; he was extubated three days later. There was no postoperative endocrinopathy, and his visual status was the same. Postoperative brain MRI revealed adequate decompression of 50% of the tumor, and he was discharged in stable condition 12 days after surgery.

The histopathology examination was consistent with pilomyxoid astrocytoma with hemorrhagic background and necrosis (Figure 3).

He was evaluated at outpatient clinics by pediatric oncology service and started on MOB chemotherapy protocol (including nitrogen mustard, vincristine, and procarbazine for one year). At the age of 5 years, his brain MRI scan revealed a good response to stable residual tumor treatment (Figure 4). His visual evaluation revealed a blind left eye, but he could see and recognize objects from the right eye.

A one-year-old baby girl, with a normal state of health until two weeks from admission, presented with decreased activity and oral intake and repeated vomiting 4–5 times a day. No seizures, trauma, or loss of consciousness. She was delivered at 35-week gestation by cesarean section (CS) due to uncontrolled gestational diabetes mellitus. Her postnatal history was unremarkable and neurodevelopmental milestones were within normal.

On examination, she was awake but irritable. General physical examination revealed head circumference within the 90 percentiles for age with normal small anterior fontanel. There were multiple cafe au lait spots (more than six) in the trunk and extremities, measuring greater than 5 mm. Neurological examination revealed normal tone and power in all extremities. Her pupils were sluggish to light with decreased response to external stimuli and moving objects, but further tests could not be assessed due to the baby’s cooperation. The optical coherence tomography test was difficult to perform due to the infants’ clinical condition. However, there was no ophthalmoplegia, and her corneal response, facial movement, and gag reflex were within normal.

On admission, her laboratory workup was normal, with no evidence of coagulopathy. A brain CT scan was performed in the emergency department, revealing a sizeable suprasellar lesion with an internal hemorrhagic component: mild ventriculomegaly (Figure 5). A brain MRI scan demonstrated a large suprasellar mass with mixed solid and cystic components representing subacute hemorrhages, a heterogeneous enhancement following intravenous contrast administration. The ventricular systems were mildly increased in size, and MRA revealed no vascular abnormalities (Figure 6).

The patient was admitted to PICU well hydrated and started on dexamethasone (2 mg intravenous injection followed by 0.5 mg hourly). She underwent right frontotemporal craniotomy the following day. The dura was open curvilinear and reflected anteriorly. There was no significant brain swelling or edema. The sylvian fissure and optic-carotid cistern were opened, which made more brain relaxation. The tumor was yellowish soft, with areas of firm and areas of soft cystic consistencies. Intratumoral microscopic decompression of the suprachiasmatic and hypothalamic solid and cystic parts was achieved using an ultrasonic aspirator. There were old and new blood products within the large cystic component. After achieving adequate decompression, craniotomy closure was done in layers, and the patient was transferred to PICU in stable condition with an estimated blood loss of around 200 ml. She received an intraoperative blood transfusion of 150 ml.

The patient remained ventilated in PICU for two days after surgery. Her postoperative course revealed transient diabetic insipidus, and she received two doses of desmopressin for two days. Her neurological examination was unchanged. A postoperative brain MRI scan (within 24 h) revealed 60%–70% adequate decompression. The patient was evaluated by pediatric oncology and endocrinology services and discharged on Day 10. She was up to date on her neurodevelopmental milestones: able to cruise on furniture, sit without support, has a good pincer grasp in both hands, able to say 2–3 words, recognize faces and follow objects, and interact with her parents and her elder sister. Pediatric neurology discharged her on clobazam 2.5 mg at bedtime for insomnia and myoclonic seizures and levothyroxine 0.25 mg as per pediatric endocrinology advice.

Histopathology examination of the specimen showed astrocytoma with myxoid features suggestive of pilomyxoid astrocytoma with a background of hemorrhage and necrosis (Figure 7). Therefore, genetics was done and a WES study was recommended to rule out NF1.

A pediatric oncology service followed the patient. She was labeled NF1 as she met the following two criteria: cafe au lait and positive family history (her father and elder sister had cutaneous stigmata of NF1). The patient started on chemotherapy protocol Cog a9952, Carboplatin Plus Vincristine for one year. At six months, an early brain MRI scan revealed a good response to the treatment (Figure 8).

An extensive literature search was conducted to include English-published case reports discussing optic pathway/hypothalamic glioma hemorrhage, covering the time from January 1970 (date of first reported case) up to January 2022. Several databases were utilized, including PubMed, Google Scholar, and Embase. Several related keywords were used, such as “optic glioma,” “chiasm,” “hypothalamus,” “hemorrhage,” “apoplexy,” and “bleeding.”

All case reports or case series of the optic pathway (optic nerve and optic chiasm) or hypothalamic glioma associated with hemorrhage for pediatric and adult populations were included from the first reported case (1970) to January 2022. Cases dealing with other hemorrhagic lesions and vascular lesions in the optic pathway/hypothalamus were excluded.

Abstracts were reviewed by two authors (RM and YM), inclusion criteria were applied, and any disagreement was resolved by discussion and review with the senior author (SB). Full-text abstracts that met the inclusion criteria were accessed and reviewed. For all included cases, the following variables were collected: author/year, age in years, gender, history of neurofibromatosis type 1 (NF1), pertinent medical history, clinical features, location of the hemorrhage, treatment received, histological diagnosis, and outcome. All variables were collected using a Microsoft Excel Sheet.