Microsurgical Resection of a Parachiasmatic Craniopharyngioma via a Left-Sided Pterional Transsylvian Approach.

Craniopharyngiomas are histologically benign tumors that originate from squamous rests along the pituitary stalk. They make up approximately 1.2% to 4.6% of all intracranial tumors and do not show significant differences in occurrence based on sex. Adamantinomatous craniopharyngiomas have 2 peaks of incidence, commonly observed in patients from ages 5 to 15 years and again from 45 to 60 years. In contrast, papillary craniopharyngiomas mainly affect adults in their fifth and sixth decades of life.1 The "malignancy" of craniopharyngiomas is attributed to their location and the challenges associated with achieving complete removal because they can manifest in the sellar, parachiasmatic, and intraventricular regions or a combination of these.2,3 Various approaches have been used to resect these tumors.4,5 Radical resection offers the most promising option for disease control, potential cure, and the ability to transform the disease from lethal to survivable in children, allowing for a functional adult life.2,3 Meticulous evaluation is crucial to determine the appropriate approach and side, with particular emphasis on closely examining the relationship between the tumor and optic pathways (nerve, chiasm, tract), which are frequently involved. This assessment should also include the tumor's relationship with other crucial structures, such as the hypothalamus and adjacent arteries, to ensure that the strategy is adjusted accordingly to further minimize the risk of postoperative morbidity. Video 1 demonstrates a left-sided pterional transsylvian approach to remove a parachiasmatic craniopharyngioma involving the left optic chiasm and tract.

Optic pathway tumor in children: Toward a new classification for neurosurgical use.

OBJECT: Optic pathway tumors (OPT) represent a challenge for pediatric neurosurgeons. Role of surgery is debated due to the high risk of iatrogenic damage, and in lasts decades it lost its importance in favor of chemotherapy. However, in some cases surgery is necessary to make biomolecular and histological diagnosis, to manage intracranial hypertension (IH) and to cooperate with medical therapies in controlling tumor relapse. With the aim to standardize selection of surgical OPT cases, we propose a simple, practical and reproducible classification. METHODS: We retrospectively analyzed data of 38 patients with OPT treated at our institution (1990-2018). After careful analysis of MRI images, we describe a new classification system. Group 1: lesion limited to one or both optic nerve(s). Group 2: chiasmatic lesions extending minimally to hypothalamus. Group 3: hypothalamo-chiasmatic exophitic lesions invading the third ventricle; they can be further divided on the base of concomitant hydrocephalus. Group 4: hypothalamo-chiasmatic lesions extending widely in lateral direction, toward the temporal or the frontal lobes. Patients' data and adopted treatment are reported and analyzed, also depending on this classification. RESULTS: Twenty children were operated on for treatment of OPT during the study period. Permanent clinical impairment was noted in 5 (25%) of operated patients, while visual improvement was noted in 1 patient. OS rate was 100% at 5 years, with a median follow up of 9 years (ranging from 2 to 23). Prevalence of intracranial hypertension and proportion of first-line surgical treatment decision were significantly higher in groups 3-4 compared to groups 1-2 (P<0.001 for both tests). CONCLUSION: Surgery can offer a valuable therapeutic complement for OPT without major risk of iatrogenic damage. Surgery is indispensable in cases presenting with IH, as in groups 3 and 4 lesions. Eligibility of patients to surgery can be based on this new classification system.

Complete Microsurgical Resection of Large Retrochiasmatic Hypothalamic Craniopharyngioma by Transpetrosal Approach: 2-Dimensional Operative Video.

This two-dimensional video shows the technical nuances of complete microsurgical resection of a hypothalamic craniopharyngioma located in the retrochiasmatic region by the transpetrosal approach. This 49-yr-old man presented with progressive fatigue, excessive sleepiness, and difficulty in vision in both eyes. He was found to have right CN 3 paralysis and bitemporal hemianopsia on neurological examination. Further workup revealed panhypopituitarism. Brain magnetic resonance imaging (MRI) demonstrated a large solid retrochiasmatic hypothalamic lesion with homogeneous contrast enhancement, measuring 2.1 × 2.6 × 2.4 cm. Optic chiasm was prefixed, and the tumor was just posterior to the pituitary stalk area. The preoperative differential diagnosis included hypothalamic astrocytoma, craniopharyngioma, germinoma, and histiocytosis. Because of the prefixed chiasm, a presigmoid, transpetrosal approach was performed. Our initial plan was a large biopsy, but based on frozen section histology, we decided to excise the tumor completely. The tumor had a pseudocapsule, which was firm and yellowish. It was debulked, dissected from the surrounding hypothalamus, and removed completely. The pituitary stalk was found at the anterior and inferior ends of the tumor and was preserved. Postoperatively, the patient developed diabetes insipidus and requires desmopressin replacement, which was gradually tapered. For panhypopituitarism, he is receiving thyroxine, hydrocortisone, and testosterone. Postoperatively, patient had an improvement in vision in his left eye and ptosis was improving in the right eye with mRs 1- at 10-wk follow-up. An informed consent was obtained from the patient prior to the surgery, which included videotaping of the procedure and its distribution for educational purposes. All relevant patient identifiers have also been removed from the video and accompanying radiology slides.

[Optochiasmatic tuberculomas as a paradoxical reaction to treatment for meningeal tuberculosis]. / Tuberculomas optoquiasmaticos como reaccion paradojica al tratamiento de tuberculosis meningea.

TITLE: Tuberculomas optoquiasmaticos como reaccion paradojica al tratamiento de tuberculosis meningea.

Nieve visual / Visual snow

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Optic pathway/hypothalamic gliomas: a dilemma in management.

Optic pathway gliomas follow an unpredictable course. Some remain static for years; others increase rapidly in size and often lead to death. This unpredictability, along with the histological similarity of these tumors, has resulted in controversy about their management. We have reviewed the results of management of all 62 patients with a diagnosis of optic pathway/hypothalamic glioma treated at The Hospital for Sick Children during the years 1976-1990. Twelve patients received no direct treatment, and 3 only a biopsy. Six patients were treated with radiotherapy alone. Eight patients received radiotherapy following a biopsy. Seventeen patients were treated by resection alone and 16 had a resection followed by radiotherapy. Eight patients received chemotherapy in addition to other therapy, and in 5 of them the chemotherapy was given as an initial therapy. Forty-eight patients are well with their visual deficits but 7 of them are receiving hormone replacement therapy. Six patients have significant neurologic deficits and 8 have died.

Large hypothalamic and optic chiasm gliomas in infants: difficulties in distinction.

Hypothalamic and optic chiasm gliomas may be indistinguishable clinically, radiographically, and pathologically. Ten children with giant gliomas of the hypothalamus and optic pathway, all under age 2 years, had masses greater than 3 cm in diameter. Pathologically all proven cases (seven) were cytologically benign fibrillary astrocytomas. Previous authors have recognized the difficulty in distinguishing these lesions; in this series, using previously suggested criteria, masses of optic chiasm could not be differentiated from hypothalamic origins. Likewise, at surgery and autopsy, the origin of these large masses was indeterminate. These tumors were more aggressive, invasive, and less responsive to therapy than the relatively benign orbital and optic nerve gliomas of older children and adults.

Computed tomography of intra- and juxtasellar lesions. A radiological study of 108 cases.

During the first two years with the 160 X 160 matrix EMI scanner at Rigshospitalet, Copenhagen 108 consecutive patients referred with the suspicion of intra- or juxtasellar tumor were subjected to 166 computed tomography (CT) examinations. The X-ray attenuation and contrast enhancement patterns of the various lesions were analyzed. In general, it was difficult to correlate these parameters with the histopathological features. Arachnoid cysts, however, had typical low preinjection attenuation and no contrast enhancement. Chromophobe and eosinophilic pituitary adenomas rarely contained calcium and only in minute amounts, hardly visible on the polaroid pictures. Craniopharyngiomas and low grade suprasellar gliomas frequently contained large calcifications. Grade I gliomas, when located in the optic nerves or hypothalamus, showed significantly higher contrast enhancement than elsewhere in the brain. Three purely intrasellar adenomas were demonstrated with CT only. The diagnostic accuracy of CT was compared to that of carotid angiography, PEG and plain skull films in the lesions verified by initial operation (n = 32). CT gave the highest accuracy of the four methods, but the accuracy of CT differed statistically only from that of carotid angiography.

[Tumours of chiasmatic and hypothalamic regions in children (author's transl)]. / Tumores de la región quiasmática e hipotalámica. A propósito de 31 casos infantiles.

31 cases with tumours in chiasm and hypothalamus are presented. 21 cases had glioma of chiasma (the tumor continued along one or both optic nerves), six cases had craniopharyngioma and four cases had astrocytoma in the hypothalamus. All of these tumours represent 19% of intracranial tumours observed in our service over a period of eleven years. Russell's syndrome was usually seen in cases of gliomas of chiasm. Visual alterations were observed in a high average of patients with any type of the tumours. Skull in lateral view was a positive proof in all the three types of tumours showing "omega" sella turcica in gliomas of chiasm, supra or retro-sellar calcifications in croniopharyngiomas and large sella turcica in astrocytomas of the hypophalamus. Neumoencephalography and angiography were very positives proofs in all types of tumours. Gammagraphy was specially possitive in gliomas of chiasm. Computed axial tomography is very useful in all types of tumour, but our experience is still limited in comparison with other methods. Most effective treatment for gliomas of the chiasm was radiotherapy with shunt in cases with hydrocephalus and total or partial extirpation in astrocytomas of the hypothalamus and in craniopharyngiomas. Survival rate among patients with these types of tumour is high but presence of visual and endocrinological secuelae is very high too.

Diagnosis of radiosensitive hypothalamic tumors without craniotomy: endocrine and neuroradiologic studies of intracranial atypical teratomas.

Three patients with intracranial atypical teratomas presented with symptoms of endocrine dysfunction (diabetes insipidus, hyperprolactinemia, and anterior hypopituitarism) suggestive of a hypothalamic disorder. Computer-assisted tomography done in two of the three cases enabled us to document the presence of a mass and institute radiotherapy, without prior neurosurgical exploration. Computer-assisted tomography also provided a safe and effective method of assessing the effects of radiotherapy.