A VAC system can be successfully used for wound management in the control of fistula effluent in patients with an EAF in an open abdomen until spontaneous fistula closure occurs or definitive fistula surgery can be performed.
BACKGROUND: Endoscopic retrograde cholangiopancreatography (ERCP)-related perforation is an infrequent complication. It is associated with significant morbidity and mortality. The present study is an evaluation of experience with management and outcomes of ERCP-related perforations and a review of relevant literature.
Sclerosing encapsulating peritonitis (SEP)/abdominal cocoon syndrome is a rare condition that is generally identified in young females. The exact cause is still unknown. Timely and accurate imaging and diagnosis play a critical role for morbidity and mortality. It is usually diagnosed during surgery. The initial treatment should be conservative, and aggressive surgical approach should be avoided as much as possible. Herein, we aimed to review the clinical features of SEP based on a patient who underwent surgery in our clinic.
Peutz-Jeghers syndrome is an autosomal dominant disorder characterized by mucocutaneous hyperpigmentation, and intestinal and extraintestinal multiple hamartomatous polyps. Development of gastrointestinal and extragastrointestinal cancer risk is markedly increased in patients with Peutz-Jeghers syndrome. We analyzed five patients from two families diagnosed with Peutz-Jeghers syndrome between 1999 and 2012. This study confirms the actual malignancy potency of PJS. Therefore, we suggest a close follow-up of patients with Peutz-Jeghers syndrome for the risk of malignancy.Keywords: Peutz-Jeghers syndrome, hamartomatous polyp, intussusception
INTRODUCTIONPeutz-Jeghers syndrome (PJS) is an autosomal dominant disorder that is characterized by mucocutaneous hyperpigmentation, and intestinal and extraintestinal multiple hamartomatous polyps. It usually occurs in infancy and late adolescence. Although most of the polyps are encountered in the jejunum, they can occur in any other part of digestive system. Development of gastrointestinal and extragastrointestinal cancer risk is markedly increased in patients with PJS (1, 2). We analyzed five patients from two families diagnosed with PJS between 1999 and 2012. There were three male and two female patients, and their ages at the initial diagnosis ranged from 2 to 38 years. At the time of diagnosis, all patients had characteristic mucocutaneous hyperpigmentations and multiple polyps in the digestive system. Gastrointestinal cancer occurred in four of the five patients, three of whom developed colon cancer and one of whom developed small intestinal cancer at 32 years of age. One female patient with colon cancer also developed bilateral breast cancer. Three of these patients died within one month to one year after being diagnosed with colorectal cancer. Thus, we aim to present some new clinical features of PJS that have not previously been described in the literature and to discuss again the relationship between PJS and the development of cancer.
CASE PRESENTATIONS Case 1:The male patient was born in 1970. He was 32 years old when colon cancer was first diagnosed. His father had been diagnosed with colon cancer at 54 years of age, and he died two years later. The patient had four siblings, two of whom were males at 14 and 22 years of age who do not show any signs or symptoms of PJS. The other two siblings described below, one male and one female, had PJS. The patient had undergone partial jejunectomy due to jejunal intussusception at the age of 17. He was admitted to our hospital in 2004 with subileus complaints and apparent perioral and oral hyperpigmentations on inspection. Detailed analysis revealed small intestinal intussusception at two locations and concomitant rectum cancer. The patient then underwent polyp excision (a total of 13 polyps were excised) and Miles operation in the same session. Histopathological examination of the resected specimen revealed signet ring carcinoma, regional lymph node metastasis (27/28), perinodal infiltration, and 13 hamartomatous pedunculat...
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