Management of pneumatosis cystoides intestinalis with pneumoperitoneum: 5-years systematic review

Introduction: Pneumatosis cystoides intestinalis (PCI) is a rare condition characterized by gas-filled cysts in the intestinal wall. Although rare, it may also involve other regions, such as the mesentery. PCI is classified as primary or secondary and is associated with multiple predisposing factors. It may be associated with either a benign condition or a potentially fatal condition, such as mesenteric ischemia. The objective of this study was to review the medical literature on the rare benign presentations of PCI, excluding cases associated with intestinal ischemia.Methods: We conducted a systematic literature review according to the PRISMA statement. We searched PubMed and LILACS databases for articles published between January 2015 and December 2020 using the following Medical Subject Headings: "pneumatosis cystoides intestinalis" and "pneumoperitoneum," "pneumatosis intestinalis," and "pneumoperitoneum" or "mesenteric pneumatosis."Results: We included 51 articles comprising 58 patients with PCI and pneumoperitoneum. Most patients were men, and mean patient age was 64.9 years. We identified an idiopathic etiology in 29.31% of cases, and the most common predisposing factor was immune dysfunction (29.31%). A total of 24.13% of patients were asymptomatic. The most commons symptoms were abdominal pain (43.10%), nausea and vomiting (41.37%), and abdominal distention (37.93%). Diagnostic surgery was conducted in 26 patients (44.82%). Only 1 patient underwent surgical treatment.Conclusions: PCI is a clinical condition that may have a benign etiology and not require surgery. Treatment of the benign etiology is conservative. Thus, life-threatening conditions should be excluded in all cases.

Pneumatosis cystoides intestinalis - an incidental finding with unpredictable evolution / Pneumatose cistoide intestinal - um achado incidental com evolução imprevisível

Abstract Pneumatosis cystoides intestinalis is an uncommon disease with unknown etiology characterized by the presence of multiple gas-filled cysts within the submucosa or subserosa of the intestinal wall. Pneumoperitoneum and/or intestinal perforation are complications that may be associated with pneumatosis cystoides intestinalis. The patients are often prone to misdiagnosis or mistreatment.We are presenting a case of pneumatosis cystoides intestinalis in a 42 year-old woman affected by peritoneal free air and numerous, diffuse, bubble-like intramural gas collections into the jejunum and ileum, showed in CT-enterography images. The woman had a carcinoid tumor located in jejunum two years ago, treated with enterectomy. Recent complaints of nonspecific symptoms of abdominal discomfort and diarrhea motivated the realization of CT scan, serum chromogranin and urine 5-hidroxindolacetic acid for hypothesis of tumor carcinoid recurrence withdraw. The only change found was the presence of pneumatosis cystoides intestinalis in CT-enterography images without intestinal necrosis, bleeding or evident obstruction. For that reason no surgical procedure was realized and the patient stayed on surveillance. Actually, the patient complaints are sporadic abdominal discomfort, without pneumatosis cystoides intestinalis clinical evidence. Conclusion: The treatment plan of patient with PCI depends on underlying cause and clinical condition severity. When conservative treatment is adopted the clinical evolution of pneumatosis cystoides intestinalis is unpredictable and can even disappear in an indeterminate number of patients.

Resumo A pneumatose cistoide intestinal é uma doença incomum, de etiologia desconhecida, caracterizada pela presença de múltiplos cistos preenchidos com gás na submucosa ou subserosa da parede intestinal. O pneumoperitoneu e/ou a perfuração intestinal são complicações que podem estar associadas à pneumatose cistoide intestinal. Os pacientes geralmente estão sujeitos a erros de diagnóstico ou de tratamento.Apresentamos um caso de pneumatose cistoide intestinal em paciente do sexo feminino, 42 anos de idade, com ar livre peritoneal e numerosas coleções gasosas intramurais, difusas e semelhantes a bolhas no jejuno e íleo, visualizados em imagens de enterografia por tomografia computadorizada (TC). Há dois anos, a paciente teve um tumor carcinoide localizado no jejuno que foi tratado com enterectomia. As queixas recentes de sintomas inespecíficos, desconforto abdominal e diarreia motivaram a realização da TC e exame de cromogranina sérica e ácido 5-hidroxindolacético na urina para excluir a hipótese de recorrência do tumor carcinoide. A única alteração encontrada foi a presença de pneumatose cistoide intestinal em imagens de enterografia por TC sem necrose intestinal, sangramento ou obstrução evidente. Por esse motivo, nenhum procedimento cirúrgico foi realizado, e a paciente permaneceu em observação. Atualmente, a queixa da paciente é de desconforto abdominal esporádico, sem evidência clínica de pneumatose cistoide intestinal. Conclusão: O plano de tratamento de pacientes com PCI depende da causa subjacente e da gravidade da condição clínica. Quando o tratamento conservador é adotado, a evolução clínica da pneumatose cistoide intestinal é imprevisível e pode até desaparecer em alguns pacientes.

Neumatosis quística intestinal complicada con vólvulo intestinal / Pneumatosis cystoides intestinalis complicated with intestinal volvulus

La neumatosis quística intestinal es una condición infrecuente caracterizada por la presencia de quistes con gas en la subserosa o submucosa del tracto gastrointestinal. Su patogénesis es incierta y se han propuesto varios mecanismos patogénicos para explicar su origen. Se presenta el caso de un paciente de sexo masculino de 46 años de edad con diagnóstico previo de neumatosis quística intestinal, que consultó por dolor abdominal, vómitos y fiebre (39 °C). Ingreso con signos de irritación peritoneal. La radiografía simple de abdomen reportó distensión intestinal y niveles hidroaéreos. Se realizó laparotomía exploratoria que reveló vólvulo de intestino delgado con estrangulamiento de algunas asas intestinales. El diagnóstico anatomopatológico fue neumatosis quística intestinal complicada con Infarto transmural por vólvulo intestinal. El paciente evoluciono satisfactoriamente

Pneumatosis cystoides intestinalis is a rare condition in which multiple gas-filled cysts are found within the wall of the gastrointestinal tract either in the subserosa or submucosa. Its pathogenesis is uncertain and several pathogenic mechanisms have been proposed to explain its origin. The case of a male patient of 46 years with previous diagnosis of pneumatosis cystic intestinalis, who consulted for abdominal pain, vomiting and fever (39 °C) is presented. By the time of admission ther were signs of peritoneal irritation. The X-ray abdominal reported distension and intestinal hydro-air levels. Exploratory laparotomy was performed and revealed small bowel volvulus with strangulation of some intestinal segment. Histological diagnosis was pneumatosis cystic intestinalis complicated with Infarction trans-mural by intestinal volvulus. The patient evolved satisfactorily.

Pneumatosis intestinalis and volvulus: a rare association / Pneumatose intestinal e volvo: uma associação rara

Pneumatosis intestinalis (PI) is a rare condition, especially when associated with volvulus; it is often misdiagnosed and inappropriately treated. We present the case of a 27 year-old woman suffering from an acute abdomen. An abdominal tomography was performed revealing Pneumatosis intestinalis. Once in the operating theatre sigmoid volvulus was diagnosed and Hartmann surgery performed. Histology showed intestinal ischemia. During the hospital stay, evolution was favourable. The authors present this case and a brief theoretical review, due to its rarity and clinical interest.

A pneumatose intestinal (PI) é uma condição pouco frequente, sendo ainda mais rara em associação com volvo; sendo muitas vezes mal diagnosticada e tratada inapropriadamente. Apresentamos o caso de uma mulher de 27 anos com um quadro de abdómen agudo. Realizou TAC abdominal que demonstrou pneumatose intestinal. Intra-operatoriamente foi diagnosticado volvo da sigmoideia e optado por cirurgia de Hartmann. O resultado anatomo-patológico da peça foi compatível com isquémia intestinal. Durante o internamento hospital, a doente evoluiu favoravelmente. Os autores apresentam este caso e uma breve revisão teórica, pela sua raridade e interesse clínico.

Pneumatosis quística intestinalis: presentación de un caso y revisión de la literatura / [Pneumatosis cystoides intestinalis: a case report and review of the literature].

Pneumatosis cystoides intestinalis features the presence of subserum or submucous cysts with gas in the intestinal wall. Its prevalence is 0.03

, although it has increased in the past decades due to a greater use of colonoscopy in everyday medical practice. Though there are several theories about its origin and many associated diseases were reported, its pathogenesis still remains uncertain. It is generally diagnosed as a finding in an imaging test. The treatment depends on the severity of the associated disease and symptoms. We report the case of a 59-year-old woman, heavy smoker, with no other clinical conditions who took a medical consultation due to abdominal bloating. She underwent a screening colonoscopy which detected the existence of cysts on the colonic wall. Afterwards, a computed tomography was performed and showed apical lung bullae, gas in a colonic wall segment, and ascitis associated to a big anexial tumor. She underwent a cytoreductive surgery, confirming the presence of advanced ovary neoplasm. The endoscopic biopsy confirmed the diagnosis of penumatosis cystoides intestinalis. We report these case because it is a rare entity which requires uncommon management and treatment guidelines.

A Case with Primary Pneumatosis Cystoides Treated after Colonoscopic Endoscopic Mucosal Resection / 대한소화기학회지

No abstract available.

A Case of Pneumatosis Intestinalis Associated with Sunitinib Treatment for Renal Cell Carcinoma / 대한소화기학회지

Sunitinib as a multitarget tyrosine kinase inhibitor is one of the anti-tumor agents, approved by the United States Food and Drug Administration to use treat gastrointestinal stromal tumor and metastatic renal cell carcinoma. The agent is known to commonly induce adverse reactions such as fatigue, nausea, diarrhea, stomatitis, esophagitis, hypertension, skin toxicity, reduciton in cardiac output of left ventricle, and hypothyroidism. However, it has been reported to rarely induce adverse reactions such as nephrotic syndrome and irreversible reduction in renal functions, and cases of intestinal perforation or pneumatosis interstinalis as such reactions have been consistently reported. In this report, a 66-year old man showing abdominal pain had renal cell carcinoma and history of sunitinib at a dosage of 50 mg/day on a 4-weeks-on, 2-weeks-off schedule. Seven days after the third cycle he was referred to the hospital because of abdominal pain. Computed tomography showed pneumoperitoneum with linear pneumatosis intestinalis in his small bowel. The patient underwent surgical exploration that confirmed the pneumatosis intestinalis at 100 cm distal to Treitz's ligament. We report a rare case of intestinal perforation with pneumatosis intestinalis after administration of sunitinib to a patient with metastatic renal cell carcinoma.

Asymptomatic Pneumatosis Intestinalis in Immune-competent Adult / 대한소화기학회지

No abstract available.

What is your diagnosis?

A male preterm infant, first-born of twins delivered by cesarean section at 31 weeks' gestation. Birth weight was 1720 g and APGAR scores were 5 and 8 at 1 and 5 min, respectively. The initial clinical course of the infant was unremarkable except mild respiratory distress which required supplemental oxygen. Enteral feeding with breast milk was started at 16 hours of age and increased with amounts of 20 ml/kg/day over the next days. On day 5 of life, clinical deterioration occurred with fecaloid vomiting, abdominal distention and bloody stools. Serum C-reactive protein and interleukin-6 levels were 37,8 mg/L and 482,1 pg/mL, respectively. Throm-bocytopenia [118x10[6]L] was detected. White blood cell count and immature-to-total ratio of the blood count remained normal. What is your diagnosis of the infant whose abdominal graph is shown below?

Pneumatosis Intestinalis with Pneumoperitoneum Mimicking Intestinal Perforation in a Patient with Myelodysplastic Syndrome after Hematopoietic Stem Cell Transplantation

Pneumatosis intestinalis (PI) is an uncommon disorder characterized by an accumulation of gas in the bowel wall, and has been associated with a variety of disorders and procedures. We describe a 35-year-old man who undertook hematopoietic stem cell transplantation due to myelodysplastic syndrome. An abdominal X-ray demonstrated extensive PI with pneumoperitoneum mimicking hollow organ perforation. However, the patient had no abdominal symptoms and there was no evidence of peritoneal inflammation. After two weeks of conservative management, including bowel rest and antibiotics, his pneumoperitoneum resolved spontaneously without any complications. Of the many factors that affect the gastrointestinal tract mucosal integrity, intramural pressure, and bacterial flora-produced intraluminal gas interact to produce PI. If the condition is accompanied by bowel ischemia, portomesenteric venous gas, metabolic acidosis, and abdominal sepsis, or if PI is severe in extent immediate surgical intervention is indicated. The described case supports that a mechanical rather than a bacterial etiology underlies the pathogenesis of PI.

A Case of Recurrent Pneumatosis Cystoides Intestinalis Associated with Recurrent Pneumoperitoneum / 대한소화기학회지

Pneumatosis cystoides intestinalis is an uncommon condition of unknown etiology, characterized by the presence of multiple gas filled cysts in the gastrointestinal tract. Many different causes of pneumatosis cystoides intestinalis have been proposed, including mechanical, pulmonary, and bacterial causes. Approximately 85% of cases are thought to be secondary to coexisting disorders of the gastrointestinal tract or the respiratory system. The condition has been associated with the therapeutic uses of lactulose, steroids, and various cancer chemotherapeutic regimens. Lactitol is a disaccharide analogue of lactulose which is available as a pure crystalline powder. There are three previous case reports suggestive of lactulose causing pnumatosis intestinalis. We report a case of recurrent pneumatosis cystoides intestinalis associated with benign recurrent pneumoperitoneum developed probably secondary to lactitol therapy.

Imágenes en abdomen: aire donde no tiene que haber aire / Images abdomen: air which does not have to be air

La presencia de aire en lugares del abdomen donde normalmente no tiene que haber aire representa, en general, un riesgo potencial de muerte para el paciente si no se hace un diagnóstico temprano y un manejo agresivo, ya sea médico o quirúrgico. El diagnóstico por imágenes juega un rol fundamental en estos casos. Las principales causas son la perforación de vísceras huecas y la producción de gas en procesos infecciosos. En general, el estudio de estos pacientes comienza con radiología o ecografía pero el método más sensible y específico es la TC, la que detecta muy bien la localización y la extensión del gas anormal.

Pneumatosis cystoides intestinalis: disease or sequel? A case report and review of theories regarding pathogenesis.

Pneumatosis cystoides intestinalis is an uncommon condition characterized by gas filled cysts within the bowel wall. We present a case of a 40-year old male who complained of abdominal pain and distension. The clinical diagnosis was intestinal obstruction and intraoperative impression was intestinal lymphangioma. The resected segment of the bowel showed multiple thin walled, tense, air-filled cysts on the serosal surface, which collapsed with a popping sound when pricked. The histologic diagnosis was pneumatosis cystoides intestinalis. Pneumatosis cystoides intestinalis has varied clinical manifestations, course and outcome depending on its benign or fulminant form. There are varying theories regarding its pathogenesis and it is seen in association with a variety of diseases. Hence it should be interpreted with relevance to its entire clinical context, as it may not be a disease in itself but a sequel to other conditions.

Pneumatosis coli, a benign form of necrotising enterocolitis.

Necrotising enterocolitis (NEC) is the most common acquired gastrointestinal emergency in neonates. Presence of pneumatosis intestinalis is taken as evidence of definite NEC. A distinctive but rare form of NEC called "pneumatosis coli" has been described, presenting with gross blood in stools and minimal or absent local and systemic signs. Radio-graph characteristically reveal isolated colonic pneumatosis without small bowel involvement. Pneumatosis coli has a more benign course compared with definite NEC. Total parenteral nutrition, antibiotics, an appropriate duration off feeds and close observation remain the corner stones of therapy assuring a benign course.

Enterocolitis necrotizante: experiencia de 15 años / Necrotizing enterocolitis: 15 years experience

El objetivo fue determinar las características clínicas, exámenes de laboratorio, radiografía de abdomen, evolución, tratamiento y sobrevida de los RN con ECN. Se analizaron las historias clínicas de 157 RN que desarrollaron ECN en un período de 15 años. En cada RN se evaluaron parámetros biomédicos, manifestaciones clínicas, exámenes complementarios, tratamiento efectuado y sobrevida. La incidencia de ECN fue de 1,8 por 1 000 RN de pretérmino fue de 12,1 días y de 4,4 días en los RN de término. Las manifestaciones clínicas más frecuentes fueron: distensión abdominal en el 94,3 por ciento, residuo gástrico en el 77,7 por ciento y sangre en deposiciones en el 36,9 por ciento. La radiografía de abdomen reveló dilatación asimétrica de asas intestinales en el 63 por ciento, neumatosis en el 45,9 por ciento y neumoperitoneo en el 14,6 por ciento. El tratamiento fue médico en el 62,4 por ciento y además quirúrgico en el 37,6 por ciento. La sobrevida fue de 60,5 por ciento. Se concluye que la ECN afecta principalmente a RN prematuros, cuyo inicio se presenta habitualmente a fines de la segunda semana de vida, en relación posiblemente a infección de predominio bacteriano

Pneumatose intestinal: revisão da literatura / Pneumatosis intestinales: literature review

A Pneumatose Intestinal (PI) consiste na presença de gás na parede do tubo gastrointestinal (TGI). Esse sinal clínico-radiológico associa-se a diversas doenças e apresenta significado clínico igualmente variável. Sua prevalência parece estar aumentando e a natureza das doenças associadas vêm se modificando nos últimos decênios. As ulceras pépticas já foram sua principal causa. Na atualidade, estados de imunossupressão e condições clínicas que aumentam a permeabilidade da mucosa do TGI (AIDS, pacientes transplantados, quimioterapia e outros) são as causas mais prováveis de PI. A PI pode ser demonstrada pela radiografia simples e pela tomografia computadorizada do abdome. Seu diagnóstico inclui, além da demonstração de sua presença, a definição de sua causa. O tratamento deve ser dirigido à causa da PI, podendo ser desde expectante até uma laparotomia de urgência. Terapêutica especifica da PI deve ficar restrita a protocolos de pesquisas. A revisão apresentada contribui para a pequena experiência da literatura neste assunto, especialmente considerando as mudanças que vêm ocorrendo na prevalência das principais causas da Pl. É ressaltada a importância da interpretação correta do significado clínico, que é amplamente variável, para a condução adequada dos casos de PI.

Pneumatosis Intestinalis (PI) is the presence of gas-filled cysts within the wall of the gastrointestinal tract (GIT). It is a clinical and/or radiological sign associated with a wide spectrum of diseases, so that it has a variable clinical significance. Probably, its prevalence is increasing. The nature of the diseases causing PI has modified in last decades. Peptic ulcers were its main cause in the past. Nowadays, probably, immunosuppressive conditions and states of increased permeability of the GIT mucosa (AIDS, transplanted patients or in chemotherapy etc) are the most usual causes. PI can be shown on simple abdominal roentgenograms and computed tomographic scans obtained with lung windows. Its diagnosis includes to define the cause in addition to its presence. The treatment should be directed to the cause of the PI, fluctuating from expectant to emergency laparotomy. This review contributes to limited literature experience in this field, especially because the most usual causes of PI have modified their prevalence. The importance of using the clinical significance, which is variable, in managing PI is emphasized.

Neumatosis quística intestinal: presentación de un caso

Presentación de un caso de neumatosis quística intestinal, se revisa su cuadro clínico y fisiopatológico así como sus manifestaciones radiológicas y los problemas que plantea.