Sellar–suprasellar lesions are most commonly pituitary adenomas; however, rare vascular tumors such as hemangioblastomas (HBs) may closely mimic them, posing significant diagnostic challenges. HBs are typically associated with von Hippel–Lindau (VHL) disease and are seldom encountered in the sellar region, with only a few cases documented. We report a 37-year-old male presenting with chronic headache and progressive visual impairment characterized by bitemporal hemianopia. Neuroimaging revealed a heterogeneously enhancing sellar–suprasellar mass with bony erosion, initially suggestive of pituitary macroadenoma. Hormonal evaluation was unremarkable, and endoscopic biopsy proved inconclusive. The patient underwent right pterional craniotomy, during which a highly vascular tumor limited complete excision. Histopathological analysis confirmed HB (World Health Organization Grade I). Post-operative evaluation excluded associated VHL disease. This case underscores the importance of including HB in the differential diagnosis of hypervascular sellar lesions. Pre-operative recognition is crucial to anticipate intraoperative hemorrhagic risk and optimize surgical planning.
Background: High-dose chemotherapy followed by autologous hematopoietic stem cell transplantation (HSCT) is used in selected children with relapsed solid tumors. In heavily pre-treated children, cumulative nephrotoxic exposure, reduced renal mass, and tubular dysfunction may contribute to renal vulnerability that is not reflected by glomerular filtration rate (GFR) alone. Aims and Objectives: This study aimed to characterize renal vulnerability beyond GFR in heavily pre-treated children undergoing autologous HSCT and to explore a multidimensional assessment incorporating structural renal reserve, cumulative nephrotoxic exposure, glomerular function, and tubular phenotype. Materials and Methods: Three children with relapsed pediatric solid tumors underwent autologous HSCT following modified thiote-pamelphalan (TT–MEL) conditioning. We reviewed clinical records for renal anatomy, cumulative nephrotoxic treatment exposure, GFR, and tubular electrolyte abnormalities before and during transplantation. Results: We observed three distinct renal phenotypes despite preserved GFR. Case 1 had a solitary kidney, substantial platinum/ifosfamide exposure, and pre-existing mild hypomagnesemia and hypokalemia, followed by severe renal magnesium and potassium wasting during transplantation. Case 2 had a solitary kidney and substantial nephrotoxic exposure without documented tubular dysfunction. Case 3 had extensive platinum/ifosfamide exposure with preserved GFR and no documented dyselectrolytemia. All three received modified TT–MEL and achieved timely engraftment without dialysis. Conclusion: Renal vulnerability may extend beyond a measurable reduction in GFR. Assessment of structural renal reserve, cumulative nephrotoxic exposure and tubular phenotype may complement conventional GFR-based assessment before high-dose conditioning. These observations support a conceptual multidimensional model requiring prospective validation.
An adult Bochdalek hernia (BH) is a rare congenital diaphragmatic defect caused by incomplete fusion of the pleuroperitoneal membrane during fetal development. Although congenital, it may remain asymptomatic until childhood and often presents with non-specific respiratory or gastrointestinal symptoms that delay diagnosis. Once identified, timely surgical repair is essential to prevent life-threatening complications. This case highlights the key anesthetic considerations and perioperative management strategies for successful repair of an adult BH.
Background: Severe pre-eclampsia is a leading cause of maternal and perinatal morbidity and mortality. Although magnesium sulfate is a standard anticonvulsant for seizure prophylaxis, some patients still progress to eclampsia. Aims and Objectives: This study determined the incidence of breakthrough eclampsia and evaluated the factors associated with its occurrence in women receiving the Zuspan regimen. Materials and Methods: A prospective observational study was conducted over a period of 1 year, from February 2021 to January 2022, among 224 patients with severe pre-eclampsia admitted to the Labour and Delivery Unit, IOG and ISO-KGH, Chennai. Following ethical approval and informed consent, all patients received a Zuspan regimen of magnesium sulfate and were monitored throughout their hospital stay. Demographic, obstetric, maternal, and neonatal data were recorded until delivery, and progression to eclampsia was assessed. Results: Of the 224 patients, 14 (6.3%) developed eclampsia, whereas 210 (93.8%) remained seizure-free. The mean age and body mass index (BMI) were 27.29±5.60 years and 30.11±3.77 kg/m2, respectively. Rural residence, gestational age >36 weeks, and mild anemia were observed in 45.5%, 56.7%, and 80.4% of patients. Hypothyroidism and gestational diabetes mellitus (GDM) were present in 31.3% and 10.3%, respectively. Eclampsia was significantly associated with GDM (P=0.01), fetal growth restriction (P=0.03), end-organ damage (P=0.01), neonatal intensive care unit admission (P=0.001), and obstetric score (P=0.044), but not with age, BMI, anemia, residence, hypothyroidism, or mode of delivery. Conclusion: Most women with severe pre-eclampsia remained seizure-free during Zuspan magnesium sulfate prophylaxis, although 6.3% developed breakthrough eclampsia. Early recognition of high-risk features and careful clinical monitoring may further reduce breakthrough seizures and improve maternal and neonatal outcomes.
Background: Monoclonal gammopathy of renal significance (MGRS) comprises renal lesions caused by nephrotoxic monoclonal immunoglobulins produced by small B-cell/plasma-cell clones without overt hematological malignancy. Renal biopsy is essential for diagnosis and characterization. Aims and Objectives: This study aimed to determine the prevalence and histomorphological spectrum of MGRS among renal biopsies and assess clinical outcomes. Materials and Methods: This retrospective and prospective renal biopsy-based study was conducted at the Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences. Biopsies from January 2012 to March 2021 were evaluated using light microscopy, immunofluorescence, and electron microscopy where available. Clinical outcome data were analyzed using statistical tests; P<0.05 was considered significant. Results: Among 7717 renal biopsies, 109 were diagnosed as monoclonal gammopathy-associated kidney disease, of which 62 fulfilled criteria for MGRS, yielding a prevalence of 0.8%. The mean age was 55.6±10.7 years, with male predominance (M=1.8:1). AL amyloidosis was the most common lesion (34; 54.8%), followed by monoclonal immunoglobulin deposition disease (12; 19.3%), fibrillary glomerulonephritis (5; 8.0%), and proliferative glomerulonephritis with monoclonal immunoglobulin deposits (4; 6.4%). Other lesions included light chain proximal tubulopathy, C3 glomerulonephritis, membranous nephropathy, and acute tubulointerstitial nephritis. Among 52 patients with follow-up data, mean follow-up duration was 13.8±17.3 months; 18 (35%) became dialysis-dependent, and 34 (65%) died. No significant differences were found between the AL and non-AL groups for age, dialysis dependence, or mortality. Conclusion: MGRS was uncommon but clinically important, with considerable morbidity and mortality. AL amyloidosis predominated. Early biopsy-based diagnosis and multidisciplinary nephrology and hematology evaluation are essential for timely management and better outcomes.
