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1/16. Spontaneous biliary perforation and necrotizing enterocolitis.

    A case of spontaneous bile-duct perforation in a 5-month-old boy with a history of necrotizing enterocolitis in his 1st week of life is reported. To our knowledge, this is the second case reported with such an antecedent, supporting a vascular etiology for some cases of spontaneous biliary perforation.
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2/16. Necrotizing enterocolitis in omphalopagus conjoined twins.

    female omphalopagus twins underwent laparotomy on the second day of life after an antenatally diagnosed high jejunal bowel obstruction. Bowel resection and choldocho-enterostomies were performed. Despite recovery from laparotomy, the development of severe necrotizing enterocolitis (NEC) in one twin led to rapid deterioration and the death of both infants on day 34. The elucidation of the combined biliary tree, the dilemma of NEC in conjoined twins, and the possibilities of emergency separation are discussed. Consideration should be given to emergency separation of conjoined twins in the event of potentially lethal complications.
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3/16. Acquired ileal atresia and spontaneous reconstitution of intestinal continuity in a premature infant with necrotizing enterocolitis.

    An 849-g (26-week gestation) premature infant had pneumoperitoneum on the 20th day of life after having normal stools and accepting partial enteric alimentation. Percutaneous penrose drainage had to be performed on 2 consecutive days at 2 different sites (right lower quadrant, left lower quadrant), at which time she stabilized. Eleven days later, she started to pass stool, and oral feeding was begun (1 to 2 mL every 4 hours). Enteral intake could not be advanced because of repeated bouts of abdominal distension, despite having regular bowel motions. Gastrointestinal contrast radiographic investigation suggested a stricture of the ileum. At laparotomy (at age 2 months) ileal atresia with a "V"-shaped defect in the mesentery was found. Surprisingly, intestinal continuity was established via an ileoileal fistula. After resection and anastomosis, she recovered fully. Mesenteric and enteric vascular ischemia (necrotizing enterocolitis) produced acquired ileal atresia-a rare occurrence. More rare is the reestablishment of intestinal continuity by fistulization.
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4/16. enteritis necroticans (pigbel) in a diabetic child.

    BACKGROUND AND methods: enteritis necroticans (pigbel), an often fatal illness characterized by hemorrhagic, inflammatory, or ischemic necrosis of the jejunum, occurs in developing countries but is rare in developed countries, where its occurrence is confined to adults with chronic illnesses. The causative organism of enteritis necroticans is clostridium perfringens type C, an anaerobic gram-positive bacillus. In December 1998, enteritis necroticans developed in a 12-year-old boy with poorly controlled diabetes mellitus after he consumed pig intestines (chitterlings). He presented with hematemesis, abdominal distention, and severe diabetic ketoacidosis with hypotension. At laparotomy, extensive jejunal necrosis required bowel resection, jejunostomy, and ileostomy. Samples were obtained for histopathological examination. Polymerase-chain-reaction (PCR) assay was performed on paraffin-embedded bowel tissue with primers specific for the cpa and cpb genes, which code for the alpha and beta toxins produced by C. perfringens. RESULTS: Histologic examination of resected bowel tissue showed extensive mucosal necrosis, the formation of pseudomembrane, pneumatosis, and areas of epithelial regeneration that alternated with necrotic segments--findings consistent with a diagnosis of enteritis necroticans. Gram's staining showed large gram-positive bacilli whose features were consistent with those of clostridium species. Through PCR amplification, we detected products of the cpa and cpb genes, which indicated the presence of C. perfringens type C. Assay of ileal tissue obtained during surgery to restore the continuity of the patient's bowel was negative for C. perfringens. CONCLUSIONS: The preparation or consumption of chitterlings by diabetic patients and other chronically ill persons can result in potentially life-threatening infectious complications.
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5/16. Successful use of the "patch, drain, and wait" laparotomy approach to perforated necrotizing enterocolitis: is hypoxia-triggered "good angiogenesis" involved?

    The traditional and most frequently employed surgical approach to perforated necrotizing enterocolitis (NEC), laparotomy and bowel resection with enterostomy creation, has been associated with an unacceptably high mortality and major morbidity (sepsis, short-gut syndrome, strictures, long-term total parenteral nutrition (TPN), prolonged and costly hospitalizations with multiple operations, the inevitable open-and-close procedure for "hopeless" extensive gut ischemia in approximately 10% of laparotomy cases, etc.). The use of the laparotomy "patch, drain, and wait" (PD&W) approach to this serious of NEC complication has provided a simple, direct, and effective means of dealing with this problem. The basic principle is to resect no gut and do no enterostomies. The details are presented here as well as the multiple types of "patching" and the importance of use of extensive direct-vision draining with bilateral small Penrose drains from the undersurfaces of both diaphragms into the pelvis with exit sites in both lower quadrants. Proper and effective patching and draining cannot be done blindly,but requires direct vision (laparotomy or laparoscopy). The critical components and timing of the "waiting" are emphasized, including the vital importance of strict avoidance of early post-drainage laparotomy in the 7- to 14-day post-drainage period (whether the drainage is percutaneous, laparotomy PD&W, or laparoscopy PD&W) due to the early, life-threatening-ending hypervascularity that occurs at this time and if left unmolested will function beneficially as life- and gut-saving "good angiogenesis". The bilateral Penrose drains capture fecal fistulas and function quite well as de-facto enterostomies as the peritoneal cavity is rapidly obliterated by adhesions and massive, florid hypervascularity/gut hypoxia triggered "good angiogenesis" (no peritoneal cavity, no peritonitis). Broad-spectrum triple antibiotics and the routine use of TPN contribute to favorable results. The lessons/experiments of nature encountered in newborns with midgut atresia(s) and remarkable levels of gut survival, in the occasional case with only meconium peritonitis and no obstruction ("auto-anastomosis") are pertinent here as the TPN of PD&W is provided in atresia(s) by the maternal-placental circulation and the sterile peritoneal cavity of atresia(s) is simulated by the combination of antibiotics and peritoneal-cavity obliteration. life- and gut-saving "good angiogenesis" is common to both situations. A 15-year personal experience with the PD&W laparotomy approach to perforated NEC in 23 cases is reported here with no mortality in the initial 60 postoperative days, no major morbidity, and no second operation required in 70% (spontaneous "auto-anastomosis") of cases. All infants with extensive gut ischemia/necrosis (NEC totalis) who would otherwise be classified as "hopeless" and managed by open-and-close only were managed in this experience successfully by PD&W with preservation of both life and an adequate amount of gut, although a second operation was required in these cases to re-establish intestinal continuity. A particularly striking observation was the rapid transition of these infants from profound illness to near-normalcy in a matter of hours after the initiation of PD&W--much like the rapid clinical changes accompanying the lancing of a boil or an abscess. An involvement of hypoxia-induced "good angiogenesis" with marked hypervascularity and involving molecules, genes, and receptors of the vascular endothelial growth factor family of hypoxia-induced angiogenesis molecules is speculated upon, and clinical studies to document these speculations are suggested as well as studies evaluating the potential of laparoscopic PD&W. The usefulness of Argyle chest-tube "venting" and "stenting" by trans-anal passage above colonic "patched" areas as seen in 2 cases is worthy of further study and use.
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6/16. Hirschsprung's disease presenting with diffuse intestinal pneumatosis in a neonate.

    In the neonate, pneumatosis intestinalis is almost always associated with necrotizing enterocolitis. The manifestation of diffuse intestinal pneumatosis in Hirschsprung's disease has been reported rarely. It may occur as a result of Hirschsprung's disease complicated with enterocolitis. We report a two-day-old female baby born at term with the problems of failure to pass meconium, progressive abdominal distension and bile stained vomiting. There was an early roentgenographic presentation of pneumatosis intestinalis which might have led to a diagnosis of necrotizing enterocolitis. However, the intestinal pneumatosis resolved within 48 hours. After anorectal manometry and contrast enema examination, an ileostomy was performed at the age of 23 days, and multiple biopsies of intestine showed aganglionosis up to the ileum at the level of 85 cm above the ileocecal valve. Unfortunately, the patient developed short bowel syndrome after operation and died suddenly after an accidental choking at the age of three months. This case suggests that Hirschsprung's disease may have an unusual early roentgenographic presentation with diffuse intestinal pneumatosis in the first few days of life. Anorectal manometries and suction biopsies are crucial for further diagnosis.
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7/16. Necrotizing enterocolitis presenting in the Emergency Department: case report and review of differential considerations for vomiting in the neonate.

    Historically, most of the acute complications of prematurity have occurred in the neonatal intensive care unit, not in the Emergency Department (ED). It is becoming increasingly common, however, for premature infants to be discharged from the hospital before they have reached a postconceptual age of 40 weeks. Such infants remain at relatively increased risk for a variety of complications of prematurity and may present to the ED in their first month of life. To highlight its symptomatology and review its management, we present the case of an infant presenting back to the ED with coffee ground emesis and fulminant necrotizing enterocolitis.
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8/16. An unusual late complication of necrotizing enterocolitis.

    Necrotizing enterocolitis (NEC) now is managed frequently successfully without surgical intervention. NEC may result in strictures, which present after the acute inflammatory process has resolved. Strictures usually present as obstruction in the first year or two of life. A case report is presented of an 11-year-old child who had symptoms from a previously undiagnosed NEC stricture as a result of pica when coins obstructed the stricture. As treatment of NEC continues to improve, more and later complications of this disease can be expected. J Pediatr Surg 36:1853-1854.
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9/16. Ileoileal intussusception and ileal stricture associated with necrotizing enterocolitis in a premature infant: report of a case.

    A 630-g male infant developed presumed necrotizing enterocolitis (NEC) after fungal enteritis that resulted in subsequent fungal septicemia associated with pneumoperitoneum on the 9th day of life. Urgent percutaneous Penrose drainage was required. During the operation on the 14th day, an ileoileal intussusception and an ileal stricture were found with perforations on each oral side of the lesions. The distinction between NEC and intussusception in premature infants when they coexist can be difficult, as these conditions share common symptoms. The relation between ischemic injury and dysmotility of the intestine is also discussed. The similarity of intrauterine fetal distress and NEC in premature infants, as seen in the present case of the intussusception associated with focal NEC, suggests that mesenteric and enteric vascular ischemia could be one trigger of intussusception in neonates.
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10/16. Bladder wall necrosis in an extremely low-birth-weight infant: a thought-provoking complication of necrotizing enterocolitis.

    An 879-g baby boy had catastrophic necrotizing enterocolitis (NEC) at 29 days of life and underwent surgical laparotomy with a subsequent ileostomy and peritoneal drain placement. The infant was subsequently stable until 42 days of life when a spontaneous perforation of the bladder apex was diagnosed by a suprapubic cystogram. laparotomy on day of life 46 found a loop of dead bowel herniating into a necrotic hole of the bladder dome. This case shows a previously unreported complication of NEC and discusses the possibility that prolonged use of a peritoneal drain may have permitted its genesis.
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