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Churg-Strauss syndrome with perforating ulcers of the colon.

We report a case of a 72-year-old woman with Churg-Strauss syndrome, who presented with intestinal perforation. She has had bronchial asthma with peripheral blood eosinophilia for 30 years. Gross findings of a resected colon showed multiple ulcers with perforation. Histologic findings demonstrated transmural inflammation infiltrated with large numbers of eosionophils, neutrophils and lymphoplasma cells, and characteristic extravascular granuloma in the subserosa. There were multifocally-distributed transmural vasculitis showing all stages of activity in medium and small-sized arteries and veins located in the submucosa, and proper muscle and subserosal layers of the colon, some of which revealed granulomatous inflammation. Histologic finding of liver showed chronic viral hepatitis B with mild inflammatory activity and macronodular cirrhosis. Immunohistochemical findings, acid fuschin orange G staining and electromicroscope found no evidence of hepatitis B virus infection contributing to the pathogenesis of this lesion.

Aged↗

Colonization of intestinal bacteria in ill neonates.

Most previous studies have shown that the digestive tract of the neonate is rapidly and heavily colonized in the first few days of life, but all the studies so far used either feces or rectal swabs to isolate and identify bacterial colonization. The exact timing of intestinal colonization is not yet certain. From a retrospective analysis of 24 neonates with intestinal perforation and a prospective study of 30 ill neonates aged less than 10 days who recieved intestinal-tract operations, we found that the incidence of bacterial growth from small-and large-bowel specimens was significantly lower within 48 h after birth and the intestinal tract was almost completely sterile within 24 h after delivery. Most of the bacteria were aerobic gram-negative bacilli, and the most common species was Escherichia coli. Although our results may not represent conditions in the normal neonate, knowledge of bowel colonization in such patients will be helpful for further management.

Chi-Square Distribution↗

Eosinophilic gastrojejunitis associated with connective tissue disease.

We report a case of eosinophilic enteritis associated with an ill-defined connective tissue disorder. The case was complicated by intestinal perforation and managed successfully without surgical intervention. No peripheral blood eosinophilia was present. Histologic examination of an intestinal biopsy revealed bands of eosinophils and mast cells at the base of the crypts with limited chronic inflammatory cell infiltrate.

Connective Tissue Diseases↗

Peritoneal drainage under local anesthesia for necrotizing enterocolitis (NEC) perforation: a second look.

Between 1974 and 1979, 15 extremely ill neonates with necrotizing enterocolitis (NEC) were initially treated with peritoneal drainage under local anesthesia for intestinal perforation. They weighed 600 to 3040 g with half less than 1000 g. Most had other serious illnesses (RDS, PDA, jaundice, CNS abnormalities). There were no immediate complications such as hemorrhage or bowel evisceration from the local drainage procedures. Seven of the 15 (46%) survived. Three (20%) died because of unrelated problems (CNS, liver failure) with an intact gastrointestinal tract, while another 8 (34%) died from intestinal sepsis. Seven (87%) of the neonates weighing less than 1000 g had an adequately functioning GI tract after this drainage procedure. Half of the neonates requiring additional surgery within 24 hr of initial peritoneal drainage survived and half of the neonates requiring subsequent surgery survived. Five of 15 infants developed strictures one died before excision. This technique is contrary to standard practice and was employed in less than 10% of the neonates with NEC treated at our institution. These results indicate that this method is effective in possibly temporizing the very ill neonate with NEC. An added bonus, however, is that 40% of the neonates treated in this fashion had complete resolution of their disease without residual scarring of the gastrointestinal tract requiring further surgery. It is our continued conclusion that this form of peritoneal drainage under local anesthesia is warranted in certain carefully selected instances.

Anesthesia, Local↗

Gastrointestinal involvement in Ehlers-Danlos syndrome classical-like type 2 associated with a novel AEBP1 splice-site variant.

Ehlers-Danlos syndrome classical-like type 2 (clEDS2) is a rare autosomal recessive connective tissue disorder caused by biallelic loss-of-function variants in the gene encoding adipocyte enhancer-binding protein 1 (AEBP1). While cutaneous and skeletal manifestations are commonly observed, gastrointestinal complications, including bowel rupture, have been reported only rarely, and their histopathological basis remains poorly characterized. Here, we report findings of molecular investigations, gastrointestinal histopathological evaluation, and long-term clinical follow-up in the 16th reported patient with AEBP1-related clEDS2, complicated by spontaneous bowel perforation. A homozygous AEBP1 splice-site variant (NM_001129.5:c.1401-2 A > G) was identified by a custom next-generation sequencing-based panel analysis for hereditary connective tissue disorders. Transcript-level analysis by reverse transcription-polymerase chain reaction and Sanger sequencing demonstrated complete skipping of exon 12, resulting in a frameshift and predicted loss of function. Clinically, the patient experienced postoperative perforation in the sigmoid colon shortly after rectal cancer surgery, followed nearly 20 years later by spontaneous small intestinal perforation. Histopathological examination of the affected colonic tissue demonstrated mildly widened intermuscular spaces without other overt structural abnormalities. Notably, repeated upper and lower gastrointestinal endoscopic procedures were performed during long-term oncological surveillance without procedure-related bowel perforation. This observation could offer an opportunity to learn about gastrointestinal involvement in AEBP1-related clEDS2 and suggests that its gastrointestinal manifestations may differ in clinical context from those typically associated with vascular Ehlers-Danlos syndrome, warranting further investigation as additional cases are accumulated.

Humans↗

Neonatal gastrointestinal perforation.

During the 7-year period from 1980 to 1986, 56 neonates with gastrointestinal (GI) perforation were treated in the Hospital for Sick Children, London. The overall mortality rate was 30%. The highest mortality rate (60%) was associated with gastric perforation, which in this series occurred only in premature neonates. Colorectal perforation carried the lowest risk of mortality (17%). The mortality rate from small bowel perforation was 35%. Prematurity and low birth weight were significant factors increasing the mortality rate from neonatal intestinal perforation. The predominant cause of perforation in the small and large intestine was necrotizing enterocolitis and the most common site of perforation was the terminal ileum. Mechanical ventilation was thought to be the cause of the perforation in four of the five neonates with gastric perforation. Primary anastomosis is a definite option in neonates who are stable intraoperatively. The mortality rate and in-patient stay for neonates who had primary anastomosis were less than for those who had stomas.

Enterocolitis, Pseudomembranous↗

Typhoid perforation. A review of the literature since 1960.

Typhoid fever is still a major health problem in the developing parts of the world, with an estimated annual incidence of 540 per 100,000. Probably one of the most lethal complications of typhoid fever is ileal perforation, which affects especially young men. We reviewed the literature published after 1960 on typhoid perforation in different developing countries, with special attention to the incidence and outcome of typhoid perforation. Information was obtained on a total number of 1,990 cases of typhoid perforation in 66,157 patients with typhoid fever, published in 52 reports all over the world. The overall frequency of intestinal perforation in typhoid fever was 3% with an overall mortality rate of 39.6%. In an endemic area of typhoid fever, the diagnosis of typhoid perforation should be made on physical examination. Surgery is perferable to medical treatment.

Adolescent↗

Subcutaneous emphysema of the neck and colonic perforation.

Subcutaneous emphysema may result from pathological changes in the thorax or lung, as a result of localised infection with gas producing organisms, after abdominal procedures, or herald an intestinal perforation. The location and spread of extraperitoneal gas is determined by the anatomical barriers and fixed fascial layers surrounding the region of pathological change. The case highlights the extent of extraperitoneal gas after colonic perforation despite minimal clinical features and the importance of intra-abdominal causes of cervical subcutaneous emphysema.

Colonic Diseases↗

Cecal perforation and adrenocortical adenoma in a dog.

Cecal perforation was diagnosed in a dog with a history of acute vomiting. The dog also had an adrenocortical adenoma. Intestinal perforation can be a serious complication of cortico-steroid treatment in the dog, but has not been attributable to hyperadrenocorticism. Fever and an inflammatory CBC were not observed, which could have been secondary to adrenal-dependent hyperadrenocorticism. The acute abdominal crisis associated with peritonitis required quick resolution in an attempt to save the dog, but also precluded any further diagnostic procedures for possible hyperadrenocorticism. The signs that suggested hyper-adrenocorticism in this dog included alopecia, lymphopenia, eosinopenia, high liver enzyme activities, hypercholesterolemia, and one large and one small adrenal gland. This latter finding presumably indicated negative feedback suppression and atrophy attributable to a functional adrenocortical adenoma.

Adenoma↗

Acute gastrointestinal manifestations associated with use of crack.

Crack, the free-base form of cocaine, causes pulmonary, cardiac, obstetric, neurologic, musculoskeletal, and gastrointestinal complications. As the popularity for crack use increases, it follows that the number of cocaine-related emergency department (ED) visits, hospitalizations, and deaths should increase. We report 3 cases of patients arriving to the ED with acute onset of abdominal pain after smoking crack. These patients required surgical correction of their intestinal perforations. Although the exact pathophysiology of intestinal ischemia is not known, cocaine blocks the reuptake of norepinephrine, which leads to mesenteric vasoconstriction and focal tissue ischemia that may lead to perforation. The chronologic relationship of crack consumption to gastrointestinal perforation leads us to surmise that a possible crack-related ischemic event is the cause of perforation in these patients. Physicians examining patients with abdominal pain should be aware of the potential gastrointestinal complications of crack and consider bowel ischemia whenever a cocaine abuser presents with abdominal pain.

Acute Disease↗

[Perforation of the ileum due to tuberculosis].

The paper presents a 26-year old female patient who was operated two times due to complications of ileal tuberculosis. The first operation was caused by intestinal perforation and the second by external fistula. Diagnosis of tuberculosis was established owing to microscopical examination of specimens.

Adult↗

[Anti-inflammatory activity of SL-573 (author's transl)].

In the carrageenin-induced edema test in rats, the anti-inflammatory activity of SL-573 was 1.6 times as potent as those of phenylbutazone (PB) and ibuprofen (IP), 3.3 times as potent as that of mefenamic acid (MF) and 6.7 times as potent as that of mepirizole (MP). In the yeast-induced edema test in rats, SL-573 showed equipotent activity with IP, the activity of which was 4 times as potent as that of MP. In the dextran-induced edema test in rats, the anti-inflammatory activity of SL-573 was significantly higher than those of IP and MP. SL-573 showed no anti-inflammatory activity in the formalin-induced edema test in rats in the same way as seen with IP and MP. SL-573 markedly inhibited the increase in capillary permeability in mice induced by intraperitoneal administration of acetic acid, and its activity was 12 times as potent as that of PB and 17 times as potent as that of MF. SL-573 showed anti-granuloma activity neither systemically nor locally. SL-573 showed equi-potent activity with PB in the adjuvant arthritis test in rats and had little effect on the healing process of the skin wound in rats. The effect of SL-573 on the carrageenin-induced edema was not diminished in the adrenalectomized rats. The gastric bleeding effect of SL-573 was significantly weaker than that usually seen in nonsteroidal anti-inflammatory drugs. SL-573 did not induce intestinal perforation even at the high dose of 800 mg/kg. Additionally, SL-573 showed a protective effect on the indomethacin-induced intestinal lesions. These pharmacological profiles of SL-573 were considered to be quite characteristic as compared with those of known nonsteroidal anti-inflammatory agents.

Adrenalectomy↗

Perforated granulomatous colitis caused by Histoplasma capsulatum.

A 57-year-old man who presented with an acute abdomen and clinically was thought to have perforated colonic diverticulitis, was found to have transmural granulomatous inflammation and perforation of colon that was caused by Histoplasma capsulatum. Although involvement of any part of the gastrointestinal tract may occur with disseminated histoplasmosis, the complication of intestinal perforation requiring emergency surgery (particularly in the colon) is extremely rare and warrants this case report with discussion of the various clinicopathologic features of gastrointestinal histoplasmosis and the occurrence of primary intestinal histoplasmosis.

Colon↗

Ascaris lumbricoides and perforation of the ileum: a critical review.

Ileal perforation due to typhoid bacilli is the commonest cause of peritonitis in the Tropics. Perforation due to Ascaris lumbricoides is rare. Three cases of ileal perforation believed to be due to A. lumbricoides are presented and the evidence critically reviewed. It is suggested that signs of heavy infestation with A. lumbricoides make a diagnosis of intestinal perforation more likely in a patient with an acute abdomen.

Ascariasis↗

Acute generalized peritonitis in African children: assessment of severity of illness using modified APACHE II score.

BACKGROUND: Generalized surgical acute abdomen is a significant cause of morbidity and mortality among children. Severity assessment is useful in order to prioritize treatment and reduce complications. Patients with a high severity score are often faced with high morbidity and mortality, thus, requiring more intensive treatment than those with low severity scores. The purpose of the present study was to assess the severity of the acute abdomen in paediatric patients using a modification of the acute physiological and chronic ill-health evaluation II score (APACHE II). METHODS: Children admitted and operated on for generalized acute abdomen over a period of 7 years from January 1993 to December 1999 were prospectively studied. A study proforma was drafted and demographic, clinical, preoperative, operative and postoperative data on each patient were entered. Each patient had severity of illness assessed using APACHE II parameters with minor modification to make it applicable to children. Postoperative outcome and severity of illness were compared to determine any correlation. RESULTS: There were 69 patients operated on within the period of the study. Age ranged from 3 months to 15 years, with a mean of 9.1 SD 4.3 years. Forty-two patients (61.2%) were male and 27 (39.8%) were female. Typhoid intestinal perforation accounted for 35 (50.7%) and intestinal obstruction with or without intestinal gangrene accounted for nine (13%). Modified APACHE II score ranged from 0 to 18, mean 8.5 SD 5. For survivors, the mean score was 8; for non-survivors, 13. Eight patients died (11.6%): four of 63 (6.4%) patients who scored 0-15 died; four of six (66.7%) patients who scored 16-18 (P < 00.05) died. A modified APACHE II score greater than 15 was associated with a significantly greater mortality. The data for postoperative morbidity and hospital stay were not conclusive. CONCLUSION: Although the APACHE II score was designed for adults, a modification can be suitably applied to predict mortality in children with generalized peritonitis. There will be a need to apply this to large number of patients in order to validate our finding.

APACHE↗

Biliary tract anomalies in thoraco-omphalopagus conjoined twins.

Thoraco-omphalopagus conjoined twins were separated urgently on the ninth day of life because of bowel obstruction and impending intestinal perforation. Preoperative studies clarified the cardiac, intestinal, and hepatic parenchymal anomalies, but the precise biliary anatomy remained obscure. Operative cholangiogram through the single gallbladder visualized only the extrahepatic biliary anatomy in twin A. At reoperation for abdominal patch removal, exploration in twin A found biliary atresia. Because of the unusual duodenal anatomy and extreme short bowel, the authors elected to treat the biliary atresia in twin A with an appendix portoenterostomy. Twin B had no gallbladder and a profoundly hypoplastic biliary tree. Both twins have normal bilirubin and liver function at 18 months of age. The anatomic details of 8 additional cases of complex biliary tract anomalies in thoraco-omphalopagus conjoined twins are reviewed. In all cases, the complex biliary anatomy has been associated with conjoined duodenum. In most cases the preoperative technetium 99m HIDA scan has been inconclusive or misleading.

Abnormalities, Multiple↗

The association of syndromic paucity of the interlobular bile ducts and congenital mechanical obstruction of the small intestine.

Three patients with congenital mechanical intestinal obstruction and features of syndromic paucity of the interlobular bile ducts (SPILBD) are presented. Two of them have siblings with syndromic paucity of the interlobular bile ducts. Two patients had jejunoileal atresia, while the other had a meconium plug with intestinal perforation. All subjects underwent surgery for intestinal obstruction within the first 24 h of life. Progressive disappearance of the bile ductules was observed in one case. We believe that an insult such as vascular insufficiency to both bile ductal epithelium and the small intestine may be the cause of this congenital disorder.

Abnormalities, Multiple↗