Endoscopic endonasal transplanum transtuberculum approach for retrochiasmatic craniopharyngiomas: Operative nuances.

The surgical treatment of craniopharyngiomas is challenging. An optimal surgical approach is extremely important to achieve complete removal of the tumor, which is often the goal of treatment. Conventionally, the endoscopic transsphenoidal approach is used for resection of craniopharyngiomas that are essentially confined to the sellar cavity, or have smaller suprasellar extension. However, the tumors located in the retrochiasmatic space are difficult to remove surgically due to a poor access. Traditionally, various transcranial microsurgical routes have been employed with limited success for resection of retrochiasmatic craniopharyngiomas. The transcranial approaches generally do not provide adequate exposure of the tumors originating in the space under the optic chiasm and nerves. Recently, the extended endonasal endoscopic surgical route, obtained by removal of the tuberculum sellae and planum sphenoidale, has been used with great success in the surgical management of tumors lying ventral to the optic chiasm, including craniopharyngiomas. It offers a direct midline access to the retrochiasmatic space and provides excellent visualization of the undersurface of the optic chiasm. It also allows extracapsular dissection using binostril-bimanual technique and facilitates complete removal of these formidable tumors. In this report, we describe step-by-step, the technical details of the endonasal endoscopic transplanum transtuberculum approach with emphasis on the operative nuances for removal of retrochiasmatic craniopharyngiomas.

Surgical management of selected pituitary macroadenomas using extended endoscopic endonasal transsphenoidal approach: early experience.

BACKGROUND AND OBJECTIVES: The endoscopic transsphenoidal approach is commonly used surgical approach for pituitary adenomas. However, adenomas with dumbbell configuration, pure suprasellar location, and fibrous consistency are difficult to remove by this approach. Recently, the extended endoscopic endonasal approach (EEEA) has been utilized to excise this subgroup of pituitary adenomas successfully. MATERIALS AND METHODS: Between January 2009 and December 2011, 13 patients with pituitary macroadenomas were treated with EEEA. The tumor subgroups included: Dumbbell tumor configuration (4), pure suprasellar tumor location (2), and large suprasellar tumors with subfrontal extension (2). Five patients had fibrous/recurrent tumors and required addition of transtubercular-transplanum extension to the standard endoscopic endonasal exposure for radical resection. RESULTS: The tumor removal was gross total in 8 (61.5%) patients, subtotal in 4 (30.7%), and partial in 1 (7.7%) patient. Clinical improvement was observed in almost all patients, immediate relief in headaches in 88% and normalization of vision in 90% of patients with pre-operative visual disturbances. Three patients with secreting adenomas, two with growth hormone-secreting adenomas and one with prolactin-secreting adenoma, had normalization of hormonal status. Three patients developed temporary diabetes insipidus two patients suffered transient ischemic attacks and one patient with a recurrent giant pituitary adenoma experienced a serious injury to the perforating artery. Four patients (30.7%) developed post-operative cerebrospinal rhinorrhea and two patients required surgical repair. CONCLUSIONS: Our early experience suggests that the EEEA offers a potentially viable treatment option in certain pituitary tumors which are difficult to remove by the standard endoscopic approaches. However, longer follow-up and larger series are needed to establish the efficacy of this approach.

Endoport-Assisted Endoscopic Surgery for Removal of Lateral Ventricular Tumors: Our Experience and Review of the Literature.

Background: Endoscopic surgery has emerged in recent years as an alternative to conventional microsurgical approaches for removal of intraventricular tumors. Endoports have enhanced tumor access and visualization with a significant reduction in brain retraction. Objective: To evaluate the safety and efficacy of endoport-assisted endoscopic technique for the removal of tumors from the lateral ventricle. Methods: The surgical technique, complications, and postoperative clinical outcomes were analyzed with a review of the literature. Results: Tumors were primarily located in one lateral ventricular cavity in all 26 patients, and extension to the foramen Monro and the anterior third ventricle was observed in seven and five patients, respectively. Except for three patients with small colloid cysts, all other tumors were larger than 2.5 cm. A gross total resection was performed in 18 (69%), subtotal in five (19%), and partial removal in three (11.5%) patients. Transient postoperative complications were observed in eight patients. Two patients required postoperative CSF shunting for symptomatic hydrocephalus. All patients improved on KPS scoring at a mean follow-up of 4.6 months. Conclusions: Endoport-assisted endoscopic technique is a safe, simple, and minimally invasive method to remove intraventricular tumors. Excellent outcomes comparable to other surgical approaches can be achieved with acceptable complications.

Surgical Management of Tuberculum Sellae Meningioma: Our Experience and Review of the Literature.

BACKGROUND: Tuberculum sellae (TS) meningiomas are benign lesions that produce neurological deficits through a mass effect on vital neurovascular structures. The treatment of choice is surgical removal with the ultimate goal to improve vision and achieve total tumor removal. OBJECTIVE: We analyzed clinical features, tumor characteristics, and surgical approaches in 62 consecutive patients with TS meningiomas to identify factors influencing the postoperative outcome. METHODS: The authors reviewed the medical records of all patients of TS meningiomas who underwent surgery at our institute between 2005 and 2018. Pre- and postoperative clinical data, including the operative findings, of these patients were analyzed. RESULTS: Sixty-two patients who underwent surgery for removal of TSM, transcranial (TCA) in 38, and endoscopic endonasal (EEA) in 24, were identified. Gross total resection was performed in 84% and 87.5% patients in the TCA and EEA groups, respectively. A significantly higher rate of visual improvement was observed in the EEA group as compared to TCA (96% versus 79%). Visual worsening after surgery was greater with TCA than EEA (21% versus 4%). Fourteen (37%) patients in the TCA group experienced different postoperative complications, and CSF leak was the main complication in the EEA group. CONCLUSIONS: TCA and EEA are both associated with higher rates of GTR and visual improvement without compromising the safety of the procedure. An optimal outcome, however, depends on the careful selection of cases based on the tumor location and morphology, and the surgeon's familiarity with the surgical approach.

Holocord Tanycytic Ependymoma: An Interesting Case with Unusual Presentation, Challenging Histological Diagnosis, and Single-staged Complete Tumor Resection.

An interesting case of intramedullary holocord cystic tumor in an 11-year old boy is reported, who presented with unusual clinical manifestations and radiological features. Gross total resection of the tumor was performed in a single-staged surgery. A rare combination of unusual presentation, uncommon histopathological findings, and challenges in the selection of surgical options were discussed with a detailed review of the literature.

Biallelic mutations in the LPAR6 gene causing autosomal recessive wooly hair/hypotrichosis phenotype in five Pakistani families.

BACKGROUND: Autosomal recessive wooly hair/hypotrichosis is an inherited disorder of hair characterized by less dense, short, and tightly curled hair on the scalp and sometimes less dense to complete absence of eyebrows and eyelashes. Autosomal recessive wooly hair/hypotrichosis phenotypes are mostly associated with pathogenic sequence variants in LIPH and LPAR6 genes. METHODS: To find out the molecular basis of the disease, five families with autosomal recessive wooly hair/hypotrichosis were recruited for genetic analysis. Direct Sanger sequencing of LIPH and LPAR6 genes was carried out using BigDye chain termination chemistry. P2RY5 protein homology models were developed to study the effect of mutation on protein structure in a family having novel mutation. RESULTS: Sanger sequencing revealed a novel homozygous missense mutation (c.47A>T) in the LPAR6 gene in family A, while recurrent mutation (c.436G>A) was detected in the rest of the four families (B-E). Protein homology models for both native and mutant P2RY5 protein were developed to study the difference in subtle structural features because of Lys16Met (K16M) mutation. We observed that P2RY5K16M mutation results decrease in the number of ionic interactions detrimental to the protein stability. Protein modeling studies revealed that the novel mutation identified here decreased the number of ionic interactions by affecting physicochemical parameters of the protein, leading to an overall decrease in protein stability with no major secondary structural changes. CONCLUSION: The molecular analysis further confirms the frequent involvement of LPAR6 in autosomal recessive wooly hair/hypotrichosis, while the bioinformatic study revealed that the missense mutation destabilizes the overall structure of P2RY5 protein.

Extended endoscopic endonasal transsphenoidal approach for retrochiasmatic craniopharyngioma: Surgical technique and results.

OBJECTIVE: Surgical treatment of retrochiasmatic craniopharyngioma still remains a challenge. While complete removal of the tumor with preservation of the vital neurovascular structures is often the goal of the treatment, there is no optimal surgical approach available to achieve this goal. Transcranial and transsphenoidal microsurgical approaches, commonly used in the past, have considerable technical limitations. The extended endonasal endoscopic surgical route, obtained by removal of tuberculum sellae and planum sphenoidale, offers direct midline access to the retrochiasmatic space and provides excellent visualization of the undersurface of the optic chiasm. In this report, we describe the technical details of the extended endoscopic approach, and review our results using this approach in the surgical management of retrochiasmatic craniopharyngiomas. METHODS: Fifteen children, including 9 girls and 6 boys, aged 8 to 15 years underwent surgery using extended endoscopic transsphenoidal approach between 2008 and 2014. Nine patients had a surgical procedure done previously and presented with recurrence of symptoms and regrowth of their residual tumors. RESULTS: A gross total or near total excision was achieved in 10 (66.7%) patients, subtotal resection in 4 (26.7%), and partial removal in 1 (6.7%) patient. Postoperatively, headache improved in 93.3%, vision recovered in 77.3%, and the hormonal levels stabilised in 66.6%. Three patients (20%) developed postoperative CSF leaks which were managed conservatively. Three (20%) patients with diabetes insipidus and 2 (13.3%) with panhypopituitarism required long-term hormonal replacement therapy. CONCLUSIONS: Our early experience suggests that the extended endonasal endoscopic approach is a reasonable option for removal of the retrochiasmal craniopharyngiomas. Compared to other surgical approaches, it provides better opportunities for greater tumor removal and visual improvement without any increase in risks.

Pharmacognostic standardization, antioxidant and free radical scavenging activity of the seeds of Triticum aestivum L - A dietary staple.

AIMS: To establish the Quality standards of Triticum aestivum L, seeds as per WHO guidelines. To study the antioxidant and hepatoprotective profile of T. aestivum L. seeds. METHODS: Pharmacognostic studies like morphological, microscopical, physico-chemical, phytochemical evaluation, fluorescence analysis, TLC, HPTLC, phytochemical analysis etc. of various extracts of the seeds of T. aestivum were carried out as per established methods. The ethanolic extract was evaluated for antioxidant and hepatoprotective activity using rat model. RESULTS: Preliminary phytochemical analysis mainly revealed the presence of carbohydrates, phenolics, proteins, resins, lipids and flavonoids. T. aestivum at different doses, i.e. 5-45 µg/ml showed free radical scavenging activity in dose dependent manner. The amount of phenolic components was found to be 313.5 µg/mg indicating considerable antioxidant activity. The ethanolic extract of T. aestivum was administered at dose level of 100 mg/kg/day, every day for 21 days along with CCl4. Biochemical and histopathological results conclude that the seeds have hepatoprotective activity.