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Pneumatosis intestinalis in association with connective tissue disease.

Pneumatosis intestinalis in association with connective tissue diseases is an unusual combination whose pathogenesis is not yet understood. Furthermore, steroid medication, often used to treat these diseases, may itself cause pneumatosis. Three cases of scleroderma, systemic lupus erythematosus, and amyloidosis in association with pneumatosis and without prior steroid therapy are presented. The small vessel occlusive pathologic processes in these diseases may cause focal areas of mucosal ischemia resulting in small, perhaps transient ulcerations that allow gas to enter the gut wall from the lumen.

Adult↗

The "circle sign": a new sonographic sign of pneumatosis intestinalis - clinical, pathologic and experimental findings.

BACKGROUND: Pneumatosis intestinalis (PI) represents gas in the bowel wall. The appearance of PI using high-resolution ultrasound (HRUS) has not been well described. OBJECTIVE: The purpose of this report is to describe a new ultrasound sign of pneumatosis seen in three patients. This sign, called the "circle sign", is indicative of bubbles of gas within the circumference of the bowel, producing an appearance of a continuous echogenic ring on ultrasound. Further studies of the sonographic characteristics of pneumatosis were performed with an in vitro model. MATERIALS AND METHODS: HRUS was performed prospectively in three patients demonstrating extensive PI radiographically. The appearance of the gas was characterized and the behavior of the intramural bubbles was studied when the bowel was compressed with the ultrasound transducer. Either CT scan or pathologic correlation was obtained in all patients. Experimental models of PI using air injected into the wall of sausage casing were developed. RESULTS: The presence of echogenic gas bubbles within the circumference of the wall of the bowel seen with HRUS was shown to represent pneumatosis intestinalis at histologic examination or by CT scanning in the three study patients. In vitro studies confirmed the clinical impression that the use of compression is helpful in distinguishing intramural from intraluminal air. CONCLUSION: The presence of echogenic gas bubbles in the wall of the bowel, often seen as a circle within the circumference of the bowel, may be helpful in diagnosing PI on ultrasound using HRUS.

Adolescent↗

Pneumatosis coli: a case report with ultrastructural study.

A case of pneumatosis intestinalis studied by light and electron microscopy is presented. The cysts are dilated lymphatic vessels. Abundant fine fatty globules are found in the cysts and in the histiocytes and multinucleated giant cells which line the lymphatics and which are present in the interstitium. The giant cell formation and florid histiocytic reaction are most probably due to leakage of fatty material from the dilated lymphatics.

Aged↗

Pneumatosis intestinalis and portomesenteric venous gas in intestinal ischemia: correlation of CT findings with severity of ischemia and clinical outcome.

OBJECTIVE: The purpose of this study was to analyze the correlation between pneumatosis or portomesenteric venous gas, or both, the severity of mural involvement, and the clinical outcome in patients with small- or large-bowel ischemia. MATERIALS AND METHODS: CT scans of 23 consecutive patients presenting with pneumatosis or portomesenteric venous gas caused by bowel ischemia were reviewed. The presence and extent of both CT findings were compared with the clinical outcome in all patients and with the severity and extent of ischemic bowel wall damage as determined by surgery (15 patients), autopsy (three patients), or follow-up (five patients). RESULTS: Seven patients showed isolated pneumatosis, and 16 patients showed portomesenteric venous gas with or without pneumatosis (11 and five patients, respectively). Pneumatosis and portomesenteric venous gas were associated with transmural bowel infarction in 14 (78%) of 18 patients and 13 (81%) of 16 patients, respectively. Nine patients (56%) with portomesenteric venous gas died. Of seven patients with infarction limited to one bowel segment (jejunum, ileum, or colon), only one patient (14%) died, whereas of the 10 patients with infarction of two or three bowel segments, eight patients (80%) died. CONCLUSION: CT findings of pneumatosis intestinalis and portomesenteric venous gas due to bowel ischemia do not generally allow prediction of transmural bowel infarction, because they may be observed in patients with only partial ischemic bowel wall damage. The clinical outcome of patients with bowel ischemia with these CT findings seems to depend mainly on the severity and extent of their underlying disease.

Adult↗

Neonatal herpes simplex virus infection introduced by fetal-monitor scalp electrodes.

An infant was severely infected with herpes simplex virus as a result of fetal monitoring with scalp electrodes on the buttocks. The first vesicles appeared at the site where the electrodes had been placed and, by day 9 after birth, additional lesions covered the entire perineum and lower extremities. The child developed acute meningoencephalitis and pneumatosis intestinalis. Herpes simplex virus was isolated from cultures of skin vesicle fluid, cornea, saliva, blood, and spinal fluid. The infection resolved after treatment with adenosine arabinoside, administered intravenously for 11 days and applied topically for another 15 days. A follow-up examination when the child was 2 years old disclosed no physical or neurologic abnormalities. This case illustrates the risk of introducing herpes simplex infection by internal fetal monitoring.

Electrodes↗

Pneumatosis intestinalis complicating C. difficile pseudomembranous colitis.

Pneumatosis intestinalis (PI) is characterized by multiple gas-filled cysts or linear gas within the bowel wall. PI may be idiopathic (15%) or secondary (85%) to a variety of disorders. We report here the first otherwise healthy adult with C. difficile infection complicated by PI and review the possible mechanisms of this previously unrecognized complication of pseudomembranous colitis. With treatment of the underlying infection, the PI resolved within 6 days of presentation.

Enterocolitis, Pseudomembranous↗

Micropneumatosis coexistent with Helicobacter pylori and its improvement.

Micropneumatosis intestinalis is the occurrence of gas-filled circular voids with diameter of 20-200 microns, not lined with epithelium. We report a 39-year-old man with superficial gastritis and Helicobacter pylori infection who also had gastric, duodenal and colonic micropneumatosis. Endoscopic biopsy after treatment for H. pylori gastritis showed no micropneumatosis in gastric, duodenal or colonic mucosal sections. We suggest that H. pylori may be one of the causative factors for micropneumatosis.

Adult↗

Midgut volvulus as a complication of intestinal malrotation in adults.

Seven adults with midgut volvulus as a complication of malrotation are presented. All patients were men with an average age of 33 years. Four presented with long histories of intermittent abdominal pain. In three patients, the twisted, corkscrew appearance of the barium column in the duodenum and proximal jejunum in an upper gastrointestinal series made the diagnosis. In the remaining patient, small-bowel follow-through performed three years before surgery showed intestinal malrotation. Three patients presented with the acute onset of severe abdominal pain. Plain films showed small-bowel obstruction and pneumatosis intestinalis in two patients and only nearly complete small-bowel obstruction in the third. Barium studies were not done in this group because of the need to proceed to exploratory laparotomy and the risk of perforation. In one patient, abdominal angiography suggested the diagnosis by showing abnormal courses of the mesenteric vessels to the volvulized segment of small bowel. All three of these patients showed ischemic segments of bowel at laparotomy.

Abdomen, Acute↗

Cystic pneumatosis of the colon after liver transplantation.

Cystic pneumatosis (CP) is an uncommon but significant condition in adults in which gas is found in a linear or cystic form in the submucosa or the subserosa of the bowel wall. The diagnosis was made by conventional X-ray and confirmed by abdominal computed tomography. Benign pneumoperitoneum due to CP should be considered in the differential diagnosis of free intra-abdominal air after chemotherapeutic or immunosuppressive therapy. As such, pneumatosis intestinalis is only a sign and must be interpreted in light of the clinical findings because it may be found in various scenarios: in patients who are otherwise healthy, and associated with pyloric stenosis, jejunoileal bypass, progressive systemic sclerosis, transplantation, chemotherapy, immunosuppression (including AIDS), obstructive pulmonary disease and finally, as in our case, after liver transplantation. Since there were no signs of secondary complications such as peritonitis, ischemia, or perforation, conservative treatment with broad-spectrum antibiotics and parenteral nutrition was initiated.

Adult↗

Gastrointestinal complications of cytosine-arabinoside chemotherapy: findings on plain abdominal radiographs.

We analyzed the findings on plain abdominal radiographs in 24 patients who had adverse gastrointestinal reactions after chemotherapy with cytosine-arabinoside (Ara-C) for treatment of acute leukemia or non-Hodgkin lymphoma. Ara-C was given with vincristine, VP 16-213, daunorubicin, amsacrine, adriamycin, or corticosteroids in various combinations and dosages. The abnormalities noted on plain abdominal radiographs included paralytic ileus (73%), cecal distension (38%), pneumatosis intestinalis (27%), thickened loops of small bowel (19%), and pneumoperitoneum (8%). One patient had small-bowel ileus simulating an obstruction. In 23%, death was directly related to gastrointestinal complications. Bowel wall erosions, necrosis, and transmural or submucosal hemorrhage were the main findings at autopsy. This experience suggests that plain abdominal radiographs are useful in the diagnosis of gastrointestinal complications associated with chemotherapy with Ara-C.

Adolescent↗

Clinically significant pneumatosis intestinalis with postoperative enteral feedings by needle catheter jejunostomy: an unusual complication.

We evaluated the incidence of clinically significant pneumatosis intestinalis and intestinal necrosis with the use of needle catheter jejunostomy in 217 consecutive patients who had undergone complicated abdominal operations or selected bariatric procedures. The needle catheter jejunostomy was used to deliver immediate postoperative nutrition, maintenance, and replacement fluids, and selected medications. In this group, no serious complications requiring surgical intervention were related to the use of needle catheter jejunostomies. Clinically significant pneumatosis intestinalis was encountered in two of 217 patients (1%). With the needle catheter jejunostomy in place, both patients improved rapidly when enteral feedings were discontinued and parenteral antibiotics were administered. None of the 217 patients developed ischemic intestinal necrosis. We conclude that 1) clinically significant pneumatosis is a rare complication of enteric feeding via needle catheter jejunostomy when the intrajejunal feeding is begun with a diluted, hypoosmolar solution with stepwise increases in osmolality, and 2) patients who do develop clinically significant pneumatosis (n = 2) seem to respond rapidly to a temporary stoppage of enteral feedings and administration of parenteral antibiotics.

Catheterization↗

Portal venous gas following trauma in a preterm infant.

During the neonatal period, pneumatosis intestinalis (PI) and portal venous gas (PVG) are pathognomonic of necrotizing enterocolitis (NEC). Rare cases of such findings evolving after abdominal trauma have been reported in adults and children, but none in premature infants. We report a case of a 3-week-old premature infant where both PI and PVG were found following a fall from a scale. The presence of these findings prior to the evolvement of clinical symptoms suggestive of NEC strongly supports a traumatic etiology.

Accidental Falls↗

Severe acute asthma with bowel infarction and pneumatosis intestinalis in a young adult.

A young patient presented with a small bowel infarction with pneumatosis intestinalis in the early course of life-threatening severe acute asthma. Low cardiac output with severe congestive right heart failure combined with the use of high doses of epinephrine to reverse the near-fatal bronchospasm probably contributed to this previously unreported complication. The presence of gas collections in the submucosal space was possibly the consequence of diffuse small bowel mucosal disruption. Early recognition of this unusual complication is of major importance to ensure appropriate therapeutic management.

Acute Disease↗

The role of lymphatics in the pathogenesis of pneumatosis in experimental bowel ischemia.

Twelve spontaneously delivered, normally suckled, small-for-gestational-age (weighing 756 to 1,213 g) neonatal piglets were used to assess the role of the mesenteric vasculature in the pathogenesis of neonatal necrotizing enterocolitis (NEC) by producing intestinal ischemia. Component vessels (arteries, veins, lymphatics) of the mesenteric vascular arcades were variously occluded by ligation for 48 hours. Nine adjacent vessels of the same type or nine adjacent combinations of vessels were occluded in piglets 12 to 18 hours postpartum. Arterial plus lymphatic ligation induced lesions showing the complete histopathological spectrum of NEC (mucosal stripping, hemorrhage, submucosal disruption and destruction, full-thickness necrosis, inflammatory infiltration) including pneumatosis intestinalis. Two of the lowest birth weight animals produced complete NEC in response to lymphatic ligation alone. A condition consistent with "prepneumatosis" was found when lymphatics only were ligated. The distended lymphatic vessels in the submucosa resembled pneumatosis with reference to shape size and distribution, but contained milk-derived lipids, some proteins and lymph but no gas. Arterial ligation alone induced NEC-like lesions without pneumatosis. Venous ligation alone induced minor congestive/hemorrhagic lesions. Pneumatosis appears to originate in the lymphatic vessels of the submucosa in this experimental model of NEC. Lymphatic occlusion alone can cause complete NEC in very SGA neonatal piglets. Arterial plus lymphatic occlusion produces a unique combination of specific pathology resembling human NEC.

Animals↗

Pre-operative radiological diagnosis of acute necrotizing enteritis in systemic lupus erythematodes.

Acute abdominal complications of systemic lupus erythematodes requiring laparatomy are not frequent. A 30-year-old man with systemic lupus erythematodes developed a necrotizing enteritis of the small bowel with pneumatosis intestinalis. Only one case of intestinal vasculitis associated with systemic lupus erythematodes has previously been reported. Presence of intramural gas in the small bowel and of gas in the portal vein was detected on plain abdominal film and confirmed by subsequent CT examination. Since corticosteroids often mask the severity of the disease, radiological evaluation is essential to the overall management of the patient with intestinal vasculitis. Due to the observed radiological signs, emergency laparotomy was performed. Histologic examination revealed necrotizing enteritis due to vasculitis.

Adult↗

Pneumatosis intestinalis. Two case reports and a retrospective review of the literature from 1985 to 1995.

Pneumatosis intestinalis (PI) is characterized by subserosal or submucosal gas-filled cysts of the gastrointestinal tract. The course may be benign or may lead to the need for urgent surgery. Knowledge of the differential diagnosis, course, and treatment modalities are key in providing optimal care to patients who present with this entity. In this article, two cases of "benign" pneumatosis seen at our institution over a one-month period are presented, along with a retrospective review of the English literature from January 1985 to March 1995. Incidence, symptoms, gross and microscopic appearance, radiographic appearance, etiology, differential diagnosis and therapy are reviewed.

Aged↗

Innocuous pneumatosis intestinalis of the right colon in renal transplant recipients. Report of three cases.

Three cases of innocuous pneumatosis intestinalis (IPI) of the right colon occurred in a series of 218 renal transplant recipients over a five-year period. Each of the three transplants was in the right iliac fossa. Clinically, these patients had little or no gastrointestinal symptoms and had normal physical examinations of the abdomen. One of the patients had a generalized herpes simplex type 2 (HSV II) infection. Cystic or linear lucencies were present within the bowel wall, associated with varying degrees of localized colonic distention. The condition (IPI) did not warrant surgical intervention or reduction of immunosuppressive therapy. The pneumatosis resolved over a period of several weeks without sequelae or recurrence.

Adult↗

Benign pneumatosis in children.

BACKGROUND: In pediatrics, pneumatosis intestinalis (PI) is usually due to necrotizing enterocolitis in premature newborns. Beyond infancy, PI is uncommon. "Benign pneumatosis" is PI in patients with few or no symptoms that resolves with conservative management. OBJECTIVE: Our goal was to better characterize benign PI in children. Our investigation focused on identifying underlying risk factors, symptoms at time of diagnosis, management and outcome. MATERIALS AND METHODS: Available medical records and radiographs of children with pneumatosis intestinalis from 1990 to 1998 were reviewed for underlying conditions, symptoms at time of radiographs, management and outcome. RESULTS: Thirty-seven children (mean age 4 years) were included. Thirty-two children had identifiable risk factors. Twenty-five children were immunocompromised by their underlying conditions or therapeutic regimen. Thirty-five children were managed conservatively with resolution of PI. Two patients, however, required surgery and one patient died. CONCLUSION: Benign pneumatosis does occur in children. The majority have underlying risk factors, most commonly related to immunosuppression. Clinical deterioration is the most useful indicator for surgical intervention. In most patients PI resolves with conservative management.

Adolescent↗