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- Miniprobe endoscopic ultrasonography for the diagnosis of colon hemangiolymphangiomaPublication . Castro-Poças, Fernando M; Bernardo, Sónia; Lima, Olinda M G; Santos, Marisa D.Hemangiolymphangioma is a very rare benign vascular tumor that affects the gastrointestinal tract in less than 1% of cases. We present the case of an asymptomatic 52-year-old female referred for endoscopic colorectal cancer screening. A sub-epithelial pediculated polypoid lesion in the splenic angle of the colon was identified. An endoscopic ultrasonography with a miniprobe was performed, which identified an anechoic lesion in the submucosal layer. Surgery was performed and the histologic findings were compatible with two hemangiolymphangioma lesions. This is one of the few cases reported in the literature of hemangiolymphangioma diagnosed by miniprobe endoscopic ultrasonography and the first to describe two lesions in the same patient.
- Perianal Paget Disease: Different Entities With the Same NamePublication . Santos, Marisa D.; Soares, Filomena; Presa-Fernandes, José M; Silva, Donzília SousaExtramammary Paget disease (EMPD) is a rare form of cancer that involves skin areas rich in apocrine glands. The common sites of occurrence include the vulva, perianal region, perineum, and axilla. Perianal Paget disease (PPD) is a subset of EMPD, which arises in the perianal skin. The disease commonly presents with a thickened plaque-like lesion with erythema or white scaly appearance. It is generally classified into two categories based on the origin of the tumor cells: (1) primary PPD if the tumor arises from intraepidermal cells and (2) secondary PPD if cancer originates from the underlying colorectal or urinary tract neoplasm. Due to its rarity, only a few sporadic case reports have been published in the literature, and treatment methods are yet to be standardized. In light of this, we report two PPD cases with different etiopathogenesis and staging: one involved only the perianal skin without regional or metastatic disease, and was not accompanied by visceral adenocarcinomas although there was a previous history of sigmoid adenocarcinoma; the other was probably secondary to an anal canal tumor spreading with disseminated disease involving the perianal and perineum area with bilateral inguinal, pelvic lymph node, and liver metastasis. The treatment plans and the outcomes of both cases were necessarily different from each other.
- Intussusception in a pregnant womanPublication . Silva, Cristina; Moreira, Pedro Soares; Simões, Vitor Costa; Sampaio, Mónica; Santos, Marisa D.Abdominal pain in a pregnant woman with a history of laparoscopic Roux-en-Y gastric bypass (LRYGB) in the emergency department is challenging. Intussusception is a rare cause of small bowel obstruction after LRYGB and can lead to intestinal necrosis, perforation, sepsis and death. The authors report a case of a 34-week pregnant patient, previously submitted to LRYGB, presenting to the emergency department with abdominal pain and vomiting. A computed tomography scan suggested the presence of ileoileal intussusception. So, an emergent laparotomy was performed with invagination reduction. The postoperative period was uneventful, as well as pregnancy and caesarian performed 4 weeks after surgery. At the 45-month follow-up, there was no recurrence of intussusception.
- Extensive colectomy in colorectal cancer and hereditary nonpolyposis colorectal cancer – long-term resultsPublication . Santos, Marisa D.; Silva, C.; Oliveira, J.; Brandão, P.; Sampaio, M.; Silva, A.; Rocha, A.; Matos, E.; Marcos-Pinto, R.Background: Colorectal cancer survival is better in hereditary nonpolyposis colorectal cancer patients than in sporadic colorectal cancer patients and even for hereditary nonpolyposis colorectal cancer with colorectal cancer is not consensual that extensive colectomy is preferable to partial colectomy. This study analyzes and compares the long-term results of these two groups of patients submitted to curative subtotal colectomy or total colectomy. Methods: Between 2002 and 2018, 68 patients with colorectal cancer without familial adenomatous polyposis were submitted to a total or subtotal colectomy in a single tertiary center. The patients were divided in two groups: hereditary nonpolyposis colorectal cancer patients (with Amsterdam criteria) and sporadic colorectal cancer patients (the others). The presence of Amsterdam criteria for hereditary nonpolyposis colorectal cancer and germline mutation for mismatch repair genes was confirmed by clinical records. Results and survival were analyzed following surgery. Results: We obtained a sporadic colorectal cancer group with 31 patients and a hereditary nonpolyposis colorectal cancer group with 37 patients. The two groups differ in age but not in gender, tumor stage or surgical morbidity. The overall survival and disease-free survival were good in both groups but even better for hereditary nonpolyposis colorectal cancer group with statistical significance when comparing the two groups. Conclusion: Total or subtotal colectomy for colorectal cancer provides a good survival. These surgical procedures should be considered the first option for colorectal cancer in young hereditary non polyposis colorectal cancer patients. In those cases, they provide good long-term results, avoiding the risk of metachronous colorectal cancer and the surveillance is restricted only to the remaining need for rectum.
- Preoperative Enteral Nutrition and Surgical Outcomes in Adults with Crohn's Disease: A Systematic ReviewPublication . Rocha, A.; Bessa, I.; Lago, P.; Santos, Marisa D.; Leite, J.; Castro-Poças, F.Background and aim: Enteral nutrition (EN) is applicable to adult Crohn's disease (CD) in treating malnutrition and in inducing remission - here as a less effective alternative than corticosteroids. The purpose of this review is to determine whether preoperative EN impacts postoperative complications of adult CD, either by means of nutritional or therapeutic effects. Summary: A systematic review of English written full-text research articles published between January 1990 and November 2017, including adult patients undergoing abdominal surgery for complicated CD after EN, was performed. Four studies out of 22 were selected, all of which institutional, retrospective, case-control cohorts, one classified as "good quality" and three as "poor quality," as rated by the Newcastle-Ottawa Scale. The application of inclusion and exclusion criteria resulted in a non-intentional absence of studies referring to supplemental EN among those reviewed. The reduced number of heterogeneous eligible studies impeded meta-analysis. In all studies, exclusive EN (EEN) was used and well tolerated, allowing to defer or even avoid surgery altogether, improving patients' global state. The two studies with the greatest number of patients found preoperative EEN to be an independent factor against infectious and non-infectious complications in 219 patients and against anastomotic leaks or abscesses in 38 patients. Also, in univariate analysis, EEN was found to increase preop-erative immunosuppressant-free intervals and to protect against anastomotic dehiscences, intra-abdominal abscesses, surgical wound infections, ileus, stomas, and reoperations in the largest study; in another study it was related to fewer intra-abdominal septic complications. Key messages: All reviewed studies are retrospective and, consequently, of limited relevance. Nonetheless, all of them call the attention of the scientific community to the potential benefits of preoperative EEN on postoperative outcomes in adult CD, calling for prospective multi-institutional studies and randomized controlled trials.
- Cecum perforation in intestinal malrotation setting in a patient with chromosome 12p deletion syndrome: A case reportPublication . Oliveira, João T.; Marques, Paula; Preza Fernandes, J.M.; Teixeira, Tânia; Santos, Marisa D.; Povo, Ana; Castro Alves, EuricoIntroduction: Intestinal malrotation results from failure of the normal gut rotation during embryological development. It is usually diagnosed in early childhood when it becomes symptomatic. Aetiology of intestinal malrotation has been scarcely addressed although relevant roles have been attributed to a few genes involved in gastrointestinal formation and association with certain syndromes has been suggested. Presentation of case: We describe the case of a 23-year-old woman with 12p deletion syndrome who presented with clinical symptoms of occlusion to the emergency department. Analytically, an elevation of inflammatory parameters was confirmed and imaging revealed pneumoperitoneum originated on cecum perforation. The patient was submitted to surgery with favorable evolution. Discussion: Clinical manifestation of intestinal malrotation is uncommon in the adult population but can have severe consequences if not diagnosed early. The abnormal positioning of the duodenojejunal loop compressed by Ladd's bands, can lead to obstruction and ischemia. Surgery via Ladd's procedure commonly applies and elective treatment may prevent added morbidity. Intestinal malrotation has been associated to certain syndromes but no prior association to chromosome 12p deletion has been described. Occlusion in a patient with 12p chromosome deletion should raise prompt suspicion for intestinal malrotation. Moreover, diagnosis of 12p chromosome deletion should increase attention towards gastrointestinal changes since elective surgery may diminish morbidity. Conclusion: Intestinal malrotation results from abnormal embryological rotation of the midgut and is associated with certain syndromes. This paper firstly associates intestinal malrotation to chromosome 12p deletion. The possibility to address it electively may prevent morbidity in patients with this syndrome.