RESUMO
INTRODUCTION: Hirschsprung's disease (HSCR) is congenital aganglionosis affecting the hindgut and presents with constipation. Surgical pull-through is the current standard treatment but causes morbidity. Per-rectal endoscopic myotomy is a novel third-space endoscopy technique for treating short-segment (SS)-HSCR. METHODS: Retrospective study of SS-HSCR patients diagnosed on history, contrast enema, rectal biopsies, and anorectal manometry, and treated by PREM. The aganglionic segment was mapped before PREM was performed using third-space endoscopy principles. Stool frequency and laxative usage before and after PREM were compared. RESULTS: Nine patients (age 7.5 [±â5.2] years; 7 male) underwent PREM during a 4-year period. Mean aganglionic segment length was 6.3âcm, mean procedure time 96.1 minutes, and mean length of hospital stay 2.5 days. Median follow-up was 17 months (range 9-58 months). Stool frequency was 1/4.4 days before vs. 1/1.2 days after PREM (Pâ=â0.0004). Mean laxative usage was 5.4 units of laxative (UL) before vs. 0.4 UL after PREM (Pâ=â0.0002). No laxatives were used by 6/9 patients after PREM. The single adverse effect seen (anal stenosis) was treated with dilatation. CONCLUSIONS: PREM is a safe and effective minimally invasive procedure to treat SS-HSCR and results in long-term response.
Assuntos
Procedimentos Cirúrgicos do Sistema Digestório , Doença de Hirschsprung , Miotomia , Criança , Endoscopia , Doença de Hirschsprung/cirurgia , Humanos , Masculino , Estudos RetrospectivosRESUMO
BACKGROUND: Microsatellite instability (MSI) accounts for 15-20% of colorectal cancer (CRC) and is considered to have favorable stage-adjusted prognosis compared to Microsatellite stable (MSS) CRCs. Determination of MSI in stage II CRC is important for management decisions regarding adjuvant chemotherapy administration. The aim of this study was to determine the prognostic and predictive significance of MSI in stage 2 CRC in the Indian scenario. MATERIALS AND METHODS: A total of 195 patients who underwent curative surgery for stage II CRC from 2010 to 2017 were included. MSI testing by immunohistochemistry (DNA MisMatch Repair proteins) was performed in all. Various clinicopathological factors and disease-free survival and overall survival were assessed between MSI and MSS groups. The effect of treatment in terms of survival benefits with adjuvant therapy in the MSI group was also assessed. RESULTS: 27.1% of the CRCs' showed MSI. Younger age (<50 years), family history of cancer, synchronous/metachronous malignancies, proximal (right sided) location, poor morphological tumour differentiation, mucin production, and presence of peritumoral (Crohn's-like) lymphocytic response showed statistically significant association with MSI. Majority (56%) of our patients showed combined loss of MLH1 and PMS2. Overall, survival among the MSI patients was significantly higher (76.6 ± 4.149 months) than the MSS patients (65.05 ± 3.555)P= 0.04. MSI patients did not show any differences in survival with or without treatment. CONCLUSION: This study highlights the distinct clinicopathological features of MSI-related CRC and the relevance of MSI testing of stage II CRC for management decisions and prognostication.