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Current status of intestinal transplantation in children.

PURPOSE: A clinical trial of intestinal transplantation (Itx) under tacrolimus and prednisone immunosuppression was initiated in June 1990 in children with irreversible intestinal failure and who were dependent on total parenteral nutrition (TPN). METHODS: Fifty-five patients (28 girls, 27 boys) with a median age of 3.2 years (range, 0.5 to 18 years) received 58 intestinal transplants that included isolated small bowel (SB) (n = 17), liver SB (LSB) (n=33), and multivisceral (MV) (n=8) allografts. Nine patients also received bone marrow infusion, and there were 20 colonic allografts. Azathioprine, cyclophosphamide, or mycophenolate mofetil were used in different phases of the series. Indications for Itx included: gastroschisis (n=14), volvulus (n=13), necrotizing enterocolitis (n=6), intestinal atresia (n=8), chronic intestinal pseudoobstruction (n=5), Hirschsprung's disease (n=4), microvillus inclusion disease (n=3), multiple polyposis (n=1), and trauma [n=1). RESULTS: Currently, 30 patients are alive (patient survival, 55%; graft survival, 52%). Twenty-nine children with functioning grafts are living at home and off TPN, with a mean follow-up of 962 (range, 75 to 2,424) days. Immunologic complications have included liver allograft rejection (n=18), intestinal allograft rejection (n=52), posttransplant lymphoproliferative disease (n=16), cytomegalovirus (n=16) and graft-versus-host disease (n=4). A combination of associated complications included intestinal perforation (n=4), biliary leak (n=3), bile duct stenosis (n=1), intestinal leak (n=6), dehiscence with evisceration (n=4), hepatic artery thrombosis (n=3), bleeding (n=9), portal vein stenosis (n=1), intraabdominal abscess (n=11), and chylous ascites (n=4). Graft loss occurred as a result of rejection (n=8), infection (n=12), technical complications (n=8), and complications of TPN after graft removal (n=3). There were four retransplants (SB, n=1; LSB n=3). CONCLUSIONS: Intestinal transplantation is a valid therapeutic option for patients with intestinal failure suffering complications of TPN. The complex clinical and immunologic course of these patients is reflected in a higher complication rate as well as patient and graft loss than seen after heart, liver, and kidney transplantation, although better than after lung transplantation.

Child, Preschool↗

Intestinal metastasis and operation in ovarian cancer: a report on 62 cases.

Among numerous sites of metastasis in ovarian cancer, the most common and serious one is the intestinal tract. However, reports on ovarian malignancy with special reference to intestinal metastasis and its surgical treatment have been few. This paper is a retrospective analysis of cases of ovarian cancer with intestinal metastasis treated at the Peking Union Medical College Hospital. Sixty-two cases of ovarian cancer with intestinal metastatic tumours larger than 2 cm in diameter were treated between January 1982 and October 1987, accounting for 28.2% of a total of 221 cases of ovarian cancer admitted during the same period. Twenty-seven patients were operated on for the first time, 24 were referred from other hospitals where the cancer was considered 'inoperable' during the initial surgery, and 11 were recurrent cases. Of the 62 cases, 51 had epithelial cancer and 11 had germ-cell tumours. Metastasis to the large intestine was observed in all 62 cases, of which the rectosigmoid was involved in 59 (95.2%). Metastasis to the small bowel was observed in 26 cases (41.9%). Superficial or serosal invasion occurred in 40 cases (64.5%), and deep invasion, in which the muscularis or both muscularis and mucosa were involved, occurred in 22 cases. A complete or optimal resection of the intraperitoneal tumours was achieved in 46 cases (74.2%). Resection of metastatic tumours of the intestine was performed in 40 cases, of which a repair of the perforated intestinal wall was required in 19. Resection of the bowel was carried out in 22 cases, and a colostomy was done in 6. Postoperatively, all patients were followed up for at least 6 months. During follow-up, 17 patients (27.4%) survived, 39 died and 6 had recurrent disease. The mean survival time for 17 patients achieving complete remission was 30.3 months. Of these, ten have survived for more than 2 years, and seven for more than 3 years. The survival rate was higher in patients with germ-cell tumours and in those having superficial invasion of the intestinal wall, optimal cytoreduction of tumour and relatively sufficient postoperative chemotherapy. However, only the latter two prognostic factors were found to bear statistical significance. The role and feasibility of bowel surgery in ovarian cancer are discussed.

Adolescent↗

[Intestinal ischemia--non-surgical approach].

Prognosis of acute mesenteric ischemia depends on the quality of the initial management of the treating physician. To reduce the still appallingly high mortality rate of this disease a rapid, invasive and aggressive diagnosis and treatment is necessary. However, early signs and symptoms of mesenteric ischemia as well as laboratory studies are nonspecific. Therefore, definitive diagnosis requires invasive testing using mesenteric angiography. Surgery should not be delayed in patients with peritoneal signs and suspected of having intestinal perforation or gangrene.

Acute Disease↗

Urgent abdominal re-explorations.

BACKGROUND: Treatment of a number of complications that occur after abdominal surgeries may require that Urgent Abdominal Re-explorations (UARs), the life-saving and obligatory operations, are performed. The objectives of this study were to evaluate the reasons for performing UARs, outcomes of relaparotomies (RLs) and factors that affect mortality. METHODS: Demographic characteristics; initial diagnoses; information from and complications of the first surgery received; durations and outcomes of UAR(s) performed in patients who received early RLs because of complicated abdominal surgeries in our clinic between 01.01.2000 and 31.12.2004 were investigated retrospectively. Statistical analyses were done using the chi-square and Fisher exact tests. RESULTS: Early UAR was performed in 81 out of 4410 cases (1.8%). Average patient age was 50.46 (13-81) years with a male-to-female ratio of 60/21. Fifty one (62.96%) patients had infection, 41 (50.61%) of them had an accompanying serious disease, 24 (29.62%) of them had various tumors and 57 (70.37%) patients were operated under emergency conditions during first operation. Causes of urgent abdominal re-explorations were as follows: leakage from intestinal repair site or from anostomosis (n:34; 41.97%); hemorrhage (n:15; 18.51%); intestinal perforation (n:8; 9.87%); intraabdominal infection or abscess (n:8; 9.87%); progressive intestinal necrosis (n:7; 8.64%); stomal complications (n:5; 6.17%); and postoperative ileus (n:4; 4.93%). Two or more UARs were performed in 18 (22.22%) cases, and overall mortality was 34.97% (n:30). Interval between the first laparotomy and UAR averaged as 6.95 (1-20) days, and average hospitalization period was 27.1 (3-78) days.Mortality rate was found to be higher among the patients who received multiple UARs. The most common (55.5%) cause of mortality was sepsis/multiple organ failure (MOF). The rates for common mortality and sepsis/MOF-dependent mortality that occured following UAR were significantly higher in patients who received GIS surgery than in those who received other types of surgeries (p:0.000 and 0.010, respectively). CONCLUSION: UARs that are performed following complicated abdominal surgeries have high mortality rates. In particular, UARs have higher mortality rates following GIS surgeries or when infectious complications occur. The possibility of efficiently lowering these high rates depends on the success of the first operations that the patient had received.

Journal Article↗

Gastrointestinal T cell lymphoma: predominant cytotoxic phenotypes, including alpha/beta, gamma/delta T cell and natural killer cells.

Gastrointestinal T cell lymphoma (TCL) is a rare subset of peripheral TCL, presenting with or without cytotoxic phenotype, a history of coeliac disease (CD) and enteropathy. However, CD is rare in Japan. Here, we describe the clinicopathological features of 18 Japanese cases. Lesions were found in the small intestine (n=13), stomach (n=3) and colon (n=2). Seven patients presented with enteropathy but none had a history of CD. Lymphomas appeared as ulceration (n=11), tumour formation (n=6), or polypoid growth (n=1). Histologically (REAL classification), neoplastic lesions were composed of intestinal type T cell lymphoma (ITCL, n=13, including one case with NK type), anaplastic large cell (ALCL, n=2), adult T cell leukaemia/lymphoma (ATLL, n=2), and lymphoblastic type (n=1). Epstein Barr virus infection was detected by EBER-1 in situ hybridization in 6 of 11 cases with ITCL but not in the other types. ALCL expressed CD30. CD56 was expressed in 3 of 11 cases of ITCL but not in other types. Among the 10 examined cases, 8 were alphabeta T cell type [CD2+, CD3+, T cell receptor (TCR)delta-1-, betaF1+], one was gammadelta T cell type [CD2+, CD3+, TCRdelta-1+, betaF1-], and the remaining case expressed natural killer (NK) cell type [CD2+, CD3-, CD56+, TCRdelta-1-, betaF1-]. Among the 8 examined cases, 3 expressed CD103 molecule, which was associated with extrathymic T cells of intraepithelial lymphocytes. All cases except ATLL expressed the cytotoxicity-associated molecule of TIA-1, and 11 of 14 TIA-1 positive cases expressed activated cytotoxic molecules of perforin, granzyme B, and/or Fas ligand. Despite the morphological, genetic and phenotypic heterogeneity, prognosis was poor, and 11 of 13 patients with small intestinal lesions died albeit appropriate treatment, but 3 of 4 patients with gastric or colonic lesions were still alive. The main cause of death was intestinal perforation. The latter might be due to the site specificity of small intestine and tumour cytotoxicity.

Adult↗

Detection and prevention of NSAID-induced enteropathy.

Non-steroidal anti-inflammatory drugs (NSAIDs) may cause damage distal to the duodenum. We reviewed the prevalence, clinical spectrum, assessment, pathogenesis, and treatment of adverse effects of NSAIDs on the small intestine. NSAIDs can cause small intestinal perforation, ulcers, and strictures requiring surgery. NSAIDs produce inflammation of the small intestine in 40 to 70% in long-term users, and the associated complications of blood loss and protein loss are difficult management problems. The pathogenesis of NSAID enteropathy is a multi-stage process involving specific biochemical and subcellular organelle damage followed by inflammatory tissue reaction. Various suggested treatments of NSAID-induced enteropathy (e.g., sulphasalazine, misoprostol, and metronidazole) have yet to undergo rigorous clinical trials. Cyclo-oxygenase-2 inhibitors appear to be safer to the small intestine than traditional NSAIDs. Pre-clinical and clinical data suggests meloxicam, celecoxib, nimesulide and rofecoxib may have less small intestine toxicity than traditional non-selective NSAIDs.

Animals↗

Management of multiple intestinal atresias and perforation with intraluminal stenting.

Intraluminal stenting of the gastrointestinal (GI) tract in both multiple intestinal atresias and perforations was used in three patients. In the atresia patients (2), a piercing trocar was used to create continuity of the GI tract and as a guide to thread the intestine over the stent. All patients currently demonstrate normal growth and development on routine enteral feeds. None developed anastomotic leaks or strictures.

Humans↗

Benign cystic teratoma of ovary perforating into small intestine with co-existent typhoid fever.

Intra-peritoneal rupture or perforation into adjacent abdominal viscera is a rare complication of benign cystic teratoma (dermoid cyst) of the ovary. We report a 48-year-old woman in whom an ovarian dermoid cyst perforated into a loop of the small bowel. The patient had co-existing typhoid fever, which probably predisposed to this complication. She recovered on antibiotic therapy and after surgical excision.

Female↗

Meconium obstruction in the very low birth weight premature infant.

Meconium obstruction of prematurity is a distinct clinical condition that occurs in very low birth weight infants, predisposing them to intestinal perforation and a prolonged hospitalization if not diagnosed and treated promptly. We report a series of 21 infants, including 2 detailed case reports, whose clinical course is indicative of meconium obstruction of prematurity. Specific risk factors are identified along with descriptions of clinical and radiologic findings, disease course, treatment, and outcome. Meconium obstruction of prematurity was more common in infants with a maternal history of pregnancy-induced or chronic hypertension, suggesting the possibility of decreased intestinal perfusion prenatally. Inspissated meconium was located most frequently in the distal ileum, making this disease process difficult to treat. Gastrografin enemas were safe, diagnostic, and therapeutic. Delay in diagnosis and treatment was associated with perforation and delay in institution of enteral feeds.

Diagnostic Errors↗

Ileal perforation in diffuse intestinal Behçet disease: report of a case.

Intestinal ulcers in Behçet disease tend to cause perforation, and postoperative recurrence is common with a high mortality rate. The optimal therapeutic strategy has yet to be elucidated, particularly in cases of diffuse intestinal involvement. We herein present a case of diffuse intestinal Behçet disease with ileal perforation. A 57-year-old Japanese woman was referred to our institution with complaints of intractable oral ulcers and abdominal pain. The patient underwent an emergency laparotomy for perforated peritonitis in spite of the intravenous administration of prednisolone (1.5 mg/kg) under total parenteral nutrition. Macroscopically, an inflamed ileum measuring 1.6 m in length was resected, including a 1-cm perforated ulceration. Innumerable small and deep ulcers were also observed, consisting of nonspecific inflammation. The patient has been free from any recurrence of intestinal ulcers while being treated with prednisolone, colchicine, and a low-residue diet for 1.5 years.

Behcet Syndrome↗

[Surgical treatment of Crohn disease of the small intestine].

Crohn's disease is potentially panintestinal and early, wide resections do not always prevent recurrences. The current approach is to intervene when indicated by the clinical situation and to perform small intestine-saving procedures. Resections remove involved zones and a limited margin of healthy tissue. Laparoscopic procedures have been developed, particularly for ileocaecal resection. These resections are followed by a high recurrence rate, mainly anastomotic, requiring reoperations. No technical factor appears to be involved in the appearance of these recurrences and their prevention is based on medical treatment of limited efficacy. At best augmentation plasties of stenoses save the length of the small intestine. They are particularly indicated in the case of stenoses at various levels of the small intestine. They are performed safely and effectively. However, the recurrence and reoperation rates are at least equal to those of resections. The indications for surgery are primarily emergencies: intestinal perforation, obstruction, haemorrhage. Sepsis of the peritoneal cavity may justify deferred restoration of intestinal continuity, with creation of a stoma. Forms complicated by an abscess may benefit from elective drainage followed by secondary resection. In the case of fistulas, it is important to spare the "target" structure, intestine or bladder, as far as possible in order to limit the extent of resection. In poorly tolerated chronic forms, refractory to medical treatment, the decision to operate must not be delayed. Surgery generally provides improvement of the patient's condition and the consequences of intestinal resection are less serious than those of the spontaneous course of lesions which are left untreated for too long.

Crohn Disease↗

Clinical observation of meconium peritonitis.

Twenty cases of meconium peritonitis were found between September 1980 and March 1988 at Mackay Memorial Hospital. The male to female ratio was 1.5 to 1. Six of the 20 cases involved premature babies. The great majority presented symptoms and signs before 5 days old, but one case was not diagnosed until 13 months of age. Polyhydramnios of the mother was found in six cases (30%). Abdominal distension was the universal symptom in all 20 cases. Hydrocele was noticed in 8 of the 12 male babies. In seven cases (35%) radiological examination showed abdominal calcifications. All patients underwent surgical intervention. Peritonitis was of cystic type in seven cases (35%); fibroadhesive in nine cases (45%); generalized in four cases (20%). Evident intestinal perforation was noted in 13 patients, and a mechanical obstructive lesion was found in 9 cases (45%) including intestinal diaphragm, atresia, volvulus, and Hirschsprung's disease. In only two patients (10%) was neither evident perforation nor obstructive lesion. The overall mortality rate was 30% (6/20), with no mortality after 1986 (0/6). The mortality seemed increased in those associated with perforation (4/13) and midgut volvulus (2/2) in particular, compared with non-perforation cases (1/7). It appears that early aggressive operation, and meticulous postoperative care, have contributed to the higher survival rate in recent years.

Cysts↗

Severe intestinal involvement in Wegener's granulomatosis: report of two cases and review of the literature.

The clinical and pathological manifestations of severe intestinal involvement in Wegener's granulomatosis were studied by a review of the literature and reports of two patients. Altogether, six cases, two females and four males, were studied. One patient developed two episodes of bowel manifestations necessitating immediate surgical interventions. The average age at onset of intestinal symptoms was 43.3 yr (26-55 yr) and, in all cases, the first signs of such manifestations developed within the first 2 yr of disease. Prior to the onset of intestinal symptoms, immunosuppressive therapy was administered in six of seven instances. Acute abdominal pain with signs of peritonitis or distention only constituted the main clinical picture in six of the seven events. The last episode was manifested clinically with profuse diarrhoea with blood and mucus. Of the seven instances of severe intestinal manifestations, the small bowel was involved in two, the large bowel in three, and both the small and large bowel were affected in two episodes. Histological evidence of vasculitis in the bowel was demonstrated in three of the seven biopsy specimens, while in four, ischaemia, inflammation and ulceration were the pathological findings. Intestinal perforation was seen four times and surgery was performed in six of seven episodes. Severe intestinal involvement is rare in Wegener's granulomatosis. The initial bowel manifestations occur within the first 2 yr of disease, and affect both the large and small bowel. Histologically, vasculitis, ischaemia, inflammation and ulceration are the prevailing findings. Death due to intestinal catastrophy occurred in one of the six patients reported. Most likely, the manifestations are associated with the disease process rather than related to the use of immunosuppressive agents.

Adult↗

Controversies in the management of necrotizing enterocolitis.

BACKGROUND: Necrotizing enterocolitis (NEC) is the most frequent and lethal disease that affects the gastrointestinal tract of the premature infant. Controversy persists as to the most appropriate management once the diagnosis is confirmed. METHODS: Review of the pertinent medical literature. RESULTS: The incidence of NEC is increasing, but the survival rate is not. Initial management of NEC consists of bowel rest, orogastric decompression, intravenous hydration, and broad-spectrum antibiotics; surgical intervention is typically reserved for infants with advanced disease or evidence of intestinal perforation. There is no consensus in the literature regarding the optimal treatment strategy for patients who require surgical intervention. There exists a lack of randomized trials comparing definitive intestinal resection with or without primary anastomosis, intestinal diversion with limited resection, or peritoneal drainage without resection. CONCLUSION: An individualized approach must be taken to achieve optimum survival for patients with NEC. Isolated perforation, in our opinion, is best managed with resection and enterostomy, whereas pan-intestinal involvement is best managed with proximal diversion alone.

Attitude of Health Personnel↗