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Computed tomography in pneumatosis intestinalis: differential diagnosis and therapeutic consequences.

Case reports of five patients with pneumatosis intestinalis diagnosed by computed tomography (CT) are presented. Etiology, differential diagnoses, and clinical consequences arising from CT imaging are discussed. In four of the patients, pneumatosis was found to be secondary to gastric ulcer, colon carcinoma, metastasis in the mesentery, and trauma-induced mesenteric ischemia. In one patient, the etiology remained elusive. Using CT, both the extent and the distribution pattern of pneumatosis could be depicted, allowing for differentiation of primary and secondary forms and assessment of prognosis. Evaluation with a lung window is a pre-requisite for reliable diagnosis of pneumatosis with CT. The presence of gas in the mesenteric or portal venous system in mesenteric ischemia is indicative of an unfavorable prognosis.

Aged↗

Additive contribution of multiple factors in the development of pneumatosis intestinalis: a case report and review of the literature.

We describe a 53-year-old patient with dermatomyositis, who developed pneumatosis intestinalis (PI) accompanied by pneumoperitoneum, pneumoretroperitoneum, pneumomediastinum, and subcutaneous emphysema of the neck. The development of PI in our patient was possibly attributed to the effect of factors such as dermatomyositis, corticosteroids, methotrexate, and alpha-glucosidase inhibitor (AGI). The coexistence of multiple factors associated with PI might enhance the risk of developing PI, even though each of them alone is not sufficient to induce it. In particular, the use of AGIs for patients treated with immunosuppressive agents such as corticosteroids requires evaluation.

Adrenal Cortex Hormones↗

Retropneumoperitoneum and pneumatosis intestinalis in 2 patients with mixed connective tissue disease and the overlap syndrome.

Two patients with pneumatosis intestinalis (PI) and mixed connective tissue disease/overlap syndrome are discussed. One patient also presented a retropneumoperitoneum, a feature as yet undescribed in PI. A review of previously reported cases revealed that PI should be suspected in those patients known to have systemic sclerosis-type involvement of the oesophagus and the small bowel, and who present with abdominal distension. PI may occur early in the course of the disease, may resolve rapidly through medical intervention only, and is compatible with a 2-year survival rate.

Adult↗

Pneumatosis intestinalis and AIDS: a case report and review of the literature.

We report five AIDS patients who developed pneumatosis intestinalis and review the pertinent literature to clarify the contributory importance of underlying infections, to suggest a management plan, and to determine whether pneumatosis intestinalis alters prognosis. Of the five patients reported, three had concurrent infections including cryptosporidiosis (one patient), presumptive CMV (one patient), and toxoplasmosis of the central nervous system (one patient). One patient also had neutropenia. Another patient was immunosuppressed during treatment for lymphoma, and the fifth patient had been taking corticosteroids before the diagnosis of CNS lymphoma. In four of five patients pneumatosis involved the right colon. Pneumatosis was linear in all five patients and also was cystic in two of the five. All patients were followed conservatively without short term adverse events, despite the known association of linear pneumatosis with bowel necrosis. We advocate conservative management and an attempt to avoid surgery whenever possible.

AIDS-Related Opportunistic Infections↗

Pneumatosis intestinalis in cystic fibrosis.

We retrospectively reviewed the clinical and radiographic findings in patients with pneumatosis intestinalis (PI), who were identified among 441 patients with cystic fibrosis. Since 1944, the age at onset and the incidence of PI have increased. Pneumomediastinum, pneumothorax, or pulmonary interstitial emphysema was found in 95% of patients with PI compared with 62% of patients without PI. The type, distribution, and severity of PI often changed with time. PI is correlated with the development of obstructive pulmonary disease, which facilitates air dissection into interstitial spaces. Dissection of air is often clinically silent and tends to be self-perpetuating.

Adolescent↗

Pneumatosis intestinalis in children.

Pneumatosis intestinalis in children is well documented condition, and is accepted as a manifestation of differing gastrointestinal tract disorders such as mechanical obstruction, vascular disturbances of the bowel with or without bacterial contamination. However, the total spectrum of the mechanisms of production of pneumatosis is not yet fully understood. The presence of pneumatosis in necrotizing enterocolitis is a very serious condition and the mortality rate is very high. Transient ischaemia in infants due to perinatal stress is considered as an important aetiological factor. Gas in the portal venous system and pneumoperitoneum are also well-known associations. Gross peritonitis and perforation of the bowel are regarded as an indication for urgent surgery. Pneumatosis due to other causes follows a benign course, and does not influence the management.

Enterocolitis, Pseudomembranous↗

Pneumatosis intestinalis in the small bowel of an adult: report of a case.

We present the case of a 70-year-old man found to have pneumatosis of the small intestine. An abdominal computed tomography (CT) scan detected massive intramural air in the entire circumference of the small intestine without any venous gas. During emergency surgery, 80 cm of the small bowel was removed. The pathological findings of thrombi in the resected specimen thus indicated that surgical intervention should be immediately performed in patients with pneumatosis throughout the small intestine. CT was useful for confirming the presence of intramural gas in pneumatosis of the small intestine. The presence of a massive amount of intramural gas in the entire small intestine circumference on the CT findings is thus considered to be an early indication of bowel infarction.

Aged↗

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.

Diagnosis, Differential↗

[Pneumatosis intestinalis in a patient of myasthenia gravis treated with high-dose corticosteroid].

We report a patient who developed persistent and severe diarrhea in the course of high-dose corticosteroid therapy for myasthenia gravis (MG). The patient, a 37-year-old woman, developed diplopia, ptosis, and muscle weakness with fatigability. She was admitted to our hospital and was diagnosed as having MG. Five months after thymectomy and oral corticosteroid administration, she developed abdominal pain and severe diarrhea. Abdominal X-ray, showed extensive pericolic gas accumulation surrounding the ascending and transverse colon. She was diagnosed as having pneumatosis intestinalis (PI). Hyperbaric therapy was not effective. Symptoms began to improve gradually when the dose of prednisolone was reduced. PI is a rare condition characterized by gas-filled cysts or linear gas in the bowel wall caused by a variety of disorders and drugs such as necrotizing colitis, obstructive pulmonary diseases, and immunosuppressants. In the present case, PI appeared to be induced by corticosteroid therapy. To our knowledge, MG complicated by PI is unusual, but this rare case highlights the importance of taking it into consideration during steroid therapy for MG.

Adult↗

Pneumatosis intestinalis associated with immune-suppressive agents in a case of minimal change disease.

We report treatment of a 38-year-old man with minimal change disease (MCD) who developed pneumatosis intestinalis (PI) during administration of immune-suppressive agents. His immunosuppressive medication had been tapered to 15 mg/day of prednisolone. MCD was steroid-resistant type. Abdominal examination and laboratory studies were not clinically remarkable. Radiologic findings were consistent with PI. Abnormal air accumulation was noted in the bowel, peritoneum, mediastinum and retroperitoneum. Conservative therapy with oxygen and metronidazole improved the PI symptoms. In 1993, a case of PI with nephrotic syndrome following steroid treatment was reported in Japan. However this is only the second case reported in the literature, and the first in English.

Adult↗

Detection of hepatic portal venous gas: its clinical impact and outcome.

The clinical impact and outcome of a rare radiographic finding of hepatic portal venous gas (HPVG) as well as the effectiveness of computed tomography (CT), CT scanogram, and conventional radiography in the detection of HPVG were retrospectively analyzed. CT scans, CT scanogram, and plain film radiographs of 11 patients with HPVG were reviewed and compared with their medical records and surgical and pathology reports. Eight of the 11 patients underwent plain film radiographs 1 day before or after the CT scan. HPVG was detected at CT in all 11 patients, on CT scanogram in three (3 of 11, 27.3%), and on plain films in one (one of eight, 12.5%). In nine of 11 patients (81.8%), CT revealed an associated pneumatosis intestinalis. In six of the 11 patients (54.6%), acute mesenteric ischemia was the underlying disease for HPVG. Seven patients (63.6%) underwent emergency exploratory laparotomy. The mortality rate for HPVG alone was 27.3% (3 of 11) and for HPVG related to mesenteric bowel disease 50% (three of six). Acute mesenteric ischemia is the most common cause of HPVG, which continues to have a predictably higher mortality. CT is superior to CT scanograms and radiographs in the detection of HPVG and its underlying diseases and, therefore, should be used as the primary diagnostic tool.

Aged↗

Pneumatosis intestinalis: role of computed tomography in diagnosis and management.

Four patients with pneumatosis intestinalis (PI) demonstrated by computed tomography are presented. Computed tomography delineated the location and extent of PI more accurately than plain abdominal radiographs and demonstrated any coexisting abdominal pathology. Lung window settings were important in the detection of intramural air and obviated the need for intraluminal contrast to outline the circumferential pattern of PI. In one patient PI would have been overlooked entirely using only conventional abdominal window settings. This technical observation could have potential clinical significance in the investigation of patients with suspected intestinal ischemia or gangrene where the appearance of intramural air is an ominous prognostic sign.

Adult↗

Pneumatosis intestinalis in patients with Crohn's disease.

The prevalence, predictors, and significance of pneumatosis were determined in 50 patients with Crohn's disease who had abdominal CT scans to rule out abscess. The presence or absence of six CT descriptors and 17 clinical descriptors was documented. CT scans of a control group of 50 subjects without inflammatory bowel disease were also examined. Data was analyzed by two-sample t tests and Fisher's exact test. Pneumatosis was found in six of 50 patients with Crohn's disease and in none of the controls. Corticosteroid treatment was the single clinical variable relating significantly (P = 0.025) to pneumatosis, although trends toward absence of resection, short duration of illness, and more severe anemia were also evident in this group. This study suggests that the presence of pneumatosis alone does not dictate a specific course of treatment, but when pneumatosis is present, careful monitoring is required and therapy is based on the overall clinical picture.

Adult↗

Predictors of gangrenous necrotizing enterocolitis and extent of disease. Early laparotomy versus peritoneal drainage.

OBJECTIVE: Evaluation of known predictors of gangrene in neonates with necrotizing enterocolitis (NEC) and identification of those suggestive of severe disease, requiring expeditious laparotomy rather than primary peritoneal drainage as a definitive treatment. METHODS: This is a retrospective review of data collected from the medical records of newborns with confirmed NEC, treated at the Maternity and Children's Hospital, Dammam, Kingdom of Saudi Arabia, from May 1993 to May 2004. Fifty-five cases were selected for the study, 23 had successful medical management and 32 underwent laparotomy. Of this group, 15 had peritoneal drainage prior to laparotomy. Nine known clinical, radiological and laboratory features suspicious of bowel perforation or gangrene were evaluated. The operated group was classified according to the extent of disease into isolated, multifocal or pan intestinal and the distribution of these 9 criteria was calculated for each of the 3 groups. Comparison was then carried out between the group with isolated NEC and those with extensive disease. RESULTS: Isolated NEC was present in 8 (25%), multifocal NEC in 19 (59%) and pan intestinal NEC in 5 (16%) of the operated cases. Pneumoperitoneum and palpable abdominal mass were the most specific and predictive signs of perforated or gangrenous bowel in NEC. Severe pneumatosis intestinalis and gasless abdomen were also highly specific and predictive of the same but had a low prevalence. Abdominal wall erythema, persistent metabolic acidosis, portal vein air, gasless abdomen and severe pneumatosis intestinalis were found to be associated with severe or extensive gangrene. Palpable abdominal mass and fixed dilated loops were increased in cases of isolated NEC. Portal vein air was associated with the highest mortality. CONCLUSION: Pneumoperitoneum, though the only absolute evidence of bowel perforation, cannot predict the extent of disease. Peritoneal drainage is a useful stabilizing procedure but the presence of any of the above mentioned criteria which are associated with severe disease necessitate a quick decision in favor of laparotomy. The absence of these signs, however, cannot rule out extensive or progressive NEC and failure to improve after peritoneal drainage also requires an emergency laparotomy, regardless of birth weight or gestational age.

Abdomen↗

Pneumatosis coli: an uncommon but treatable cause of faecal incontinence.

Pneumatosis intestinalis is defined as the presence of gas within the bowel wall. Small bowel pneumatosis is less commonly reported and more severe than colonic disease in adults. Pneumatosis coli is characterised by multiple collections of encysted gas occurring within the sub-mucosa and subserosa of the colon and rectum. It is an uncommon condition which typically presents in late middle age and has been associated with a number of gastrointestinal (e.g. pyloric stenosis, sigmoid volvulus and ischaemic bowel) and non-gastrointestinal (e.g. chronic obstructive pulmonary disease, depression and multiple sclerosis) diseases. Some cases, however, are idiopathic or primary. Symptoms can include diarrhoea, constipation, mucus per rectum, bleeding, flatus, abdominal pain and, rarely, faecal incontinence. We report on two patients, one of whom presented with faecal incontinence, the other who had troublesome lower gastrointestinal symptoms including faecal incontinence. Both responded well to continuous oxygen therapy.

Aged↗

Pneumatosis intestinalis in an infant undergoing bone marrow transplantation for Wiskott-Aldrich syndrome.

A 7-month-old patient with Wiskott-Aldrich syndrome (WAS) developed pneumatosis intestinalis (PI) in the immediate post-transplant period after receiving paternal human leucocyte antigen (HLA) phenotypically matched bone marrow (BM). PI has been described in patients with congenital or acquired immunodeficiency states and after bone marrow transplantation (BMT). To our knowledge, the condition has not been described in WAS. The underlying bowel mucosa damage as a result of the history of massive rectal bleeding, the effects of the conditioning regimen, immunosuppression, neutropenia, and infection, may all have contributed to the development of PI. Although the condition resolved by conservative management alone, the patient developed Klebsiella pneumonia sepsis, interstitial pneumonitis, failed to engraft, and died on day +66 following a second infusion of stem cells mobilized from his father's peripheral blood.

Bone Marrow Transplantation↗

Pneumatosis intestinalis in a burn patient: case report and literature review.

Pneumatosis Intestinalis (PI) is a rare condition characterized by gas in the intestinal wall. PI is not a disease in itself but a sign of an underlying problem. It is associated with a wide variety of underlying disorders, ranging from benign to life-threatening. When found, it should prompt a search for the underlying cause. Diagnostic efforts should be aimed at ruling out catastrophic problems such as intestinal ischemia and necrosis. The presence of peritonitis, rectal bleeding, portomesenteric venous gas, and lactic acidosis are important factors to determine the best course of action. Burn patients have multiple risk factors for developing this condition. Despite this, PI is not well-described in the burn literature. We present here a case report, a brief review of the literature, and clinical considerations.

Burns↗

[Air cysts in the small intestine].

Pneumacystoides intestinalis (PCI) is a rare disease consisting of gaseous cysts localized in the wall of the gut. PCI is described from stomach to rectum, but is most frequently associated with chronic obstructive lung disease (COLD), peptic ulcers or other lesions in the gut. Idiopathic PCI is also reported. In this report, the radiological appearance of PCI in the small intestine of a 15 year old male patient with low mechanical obstruction of the small intestine is shown to be difficult to distinguish from the radiological findings under gangrenous conditions in the intestine, where gas assembles in the gut wall. It is suggested that PCI might be considered, when gas is demonstrated in the intestinal wall on routine x-ray of the abdomen, in the absence of accompanying clinical signs of intestinal necrosis.

Adolescent↗