[Pneumatosis cystoides intestinalis: a case report and literature review]. / Kistoznyi pnevmatoz tonkoi kishki: klinicheskii sluchai i obzor literatury.

Pneumatosis cystoides was first described by Du Vernay in 1783. This is a fairly rare disease with nonspecific symptoms and CT data on pneumoperitoneum. The authors present pneumatosis intestinalis in a patient with systemic connective tissue disorder. Free gas in abdominal cavity and dilated intestinal loops were an indication for emergency surgery with subsequent resection of intestine due to signs of ischemic damage. A review of clinical cases allows us to conclude that pneumoperitoneum requires careful differential diagnosis. Free gas in abdominal cavity in patients with cystic pneumatosis is an indication for emergency surgery only in case of complicated course of disease.

Pneumatosis cystoides intestinalis as a rare cause of non-surgical pneumoperitoneum.

Pneumoperitoneum as a finding on imaging examinations is not always a sign of acute abdomen due to gastrointestinal perforation. These findings must be viewed in connection with the clinical condition and personal history of each patient because they may also indicate a non-surgical or spontaneous pneumoperitoneum. This condition is repeatedly described but very often neglected. This paper presents the case report of a patient with non-surgical pneumoperitoneum where, despite proceeding according to the guidelines, no expected intra-abdominal pathology explaining the patient's problems was found.

Pneumatosis cystoides intestinalis associated with etoposide in hematological malignancies: a case report and a literature review.

Pneumatosis cystoides intestinalis (PCI) is a rare condition characterized by the presence of air collection within the subserosa and/or submucosa of the gastrointestinal wall. Due to the lack of specific symptoms, PCI is likely to be misdiagnosed or missed without the use of imaging techniques or gastrointestinal endoscopy. Here, we report a patient who complained of abdominal distention and constipation after chemotherapy for hematological malignancies, and was diagnosed with secondary PCI via computed tomography (CT) and exploratory laparotomy. Pneumoperitoneum was no longer observed after two weeks of conservative treatments. Notably, the possibility of intra-abdominal pressure (IAP) as a predictor for surgical intervention was proposed. Furthermore, we conducted a literature review on PCI after chemotherapy in hematological malignancies to raise awareness of etoposide-related PCI, while whether PCI could be identified as an adverse event of etoposide requires more evidence.

Acute abdomen with gastric volvulus revealing an underlying pneumatosis cystoides intestinalis: a case report.

BACKGROUND: Pneumatosis intestinalis is an abnormal presence of free air outside the lumen of the intestines in many shapes. It is classified based on its etiology to primary or secondary, it affects adults as well as infants and can involve any part of the GI tract. CASE PRESENTATION: We report a case of a 55-year-old man with a past medical history of a surgically repaired perforated duodenal ulcer who presented with an acute abdominal pain, Flatulence and constipation. On examination of the abdomen; severe distension, tenderness and tympanicity on percussion were noted. An erect CXR was performed and showed bilateral sub-diaphragmatic air levels. We performed an abdominal Paracentesis under the right subcostal margin which led to evacuation of large amounts of air. Next, an investigational laparotomy showed that the reason was a gastric volvulus associated with an anterior and posterior gastric wall lacerations. The suitable surgical repair approach was taken, but another lesion was detected incidentally. A pneumatosis cystoides intestinalis (PCI) was extended along large length of the intestines in many shapes and without any symptoms or signs. CONCLUSIONS: Pneumatosis cystoides intestinalis has been reported continuously in relation to peptic ulcer disease (PUD). We aim to report a new association of a gastric volvulus and PCI secondary to pyloric stenosis caused by a duodenal ulcer; which we believe can aid in the diagnosing of dangerous complications, of a rare disease.

Pneumatosis cystoides intestinalis: A rare complication after hematopoietic stem cell transplantation.

BACKGROUND: Pneumatosis cystoides intestinalis (PCI) is a disorder in which widespread air sacs are present in mucosa, submucosa, subserosa, and intraabdominal area of the intestinal wall. It has a heterogeneous clinical presentation as a rare complication of intestinal graft-versus-host disease (GVHD). Computed tomography is the preferred imaging method for the diagnosis. Since the air sacs could be ruptured spontaneously, the presence of free air in the peritoneal cavity does not confirm intestinal perforation. The conservative treatment approach is sufficient in cases that do not require urgent surgical intervention, such as perforation or obstruction. CASE: Here, we present a 2.5-year-old patient diagnosed with primary hemophagocytic lymphohistiocytosis (pHLH), who underwent allogeneic hematopoietic stem cell transplantation from a matched unrelated donor (MUD) and developed PCI secondary to intestinal GVHD 14th months after HSCT. CONCLUSIONS: Pneumatosis cystoides intestinalis, which is a rare complication, should be kept in mind, especially in patients with intestinal GVHD and receiving intensive immunosuppressive, octreotide, and steroid treatment after HSCT.

Pneumatosis cystoides intestinalis: a case report and literature review.

BACKGROUND: Pneumatosis cystoides intestinalis (PCI) is a low-incidence disease that confuses many doctors. A vast number of factors are suspected to contribute to its pathogenesis, such as Crohn's disease, intestinal stenosis, ulcerative colitis, drug use, extra-gastrointestinal diseases, and chronic obstructive pulmonary disease. Most consider its pathogenesis interrelated to an increase in intra-intestinal pressure and the accumulation of gas produced by aerogenic bacteria, and patients with atypical symptoms and imaging manifestations tend to be misdiagnosed. CASE PRESENTATION: A 64-year-old man complained of a 3-month history of bloody stool without mucopurulent discharge, abdominal pain, or diarrhea. Colonoscopy revealed multiple nodular projections into the segmental mucosa of the sigmoid colon. Crohn's disease and malignant disease ware suspected first according to the patient's history, but laboratory examinations did not confirm either. Endoscopic ultrasound (EUS) revealed multiple cystic lesions in the submucosa. Moreover, computer tomography scan showed multiple bubble-like cysts. Combined with ultrasonography, computed tomography, and pathology findings, we ultimately made a diagnosis of PCI. Instead of surgery, we recommended conservative treatment consisting of endoscopy and oral drug administration. His symptoms improved with drug therapy after discharge, and no recurrence was noted on follow-up. CONCLUSIONS: The incidence of PCI is low. Due to a lack of specificity in clinical manifestations and endoscopic findings, it often misdiagnosed as intestinal polyps, tumors, inflammatory bowel disease, or other conditions. Colonoscopy, computed tomography, and ultrasonography have demonstrated benefit in patients with multiple nodular projections in colon. Compared to the treatment of the above diseases, PCI treatment is effective and convenient, and the prognosis is optimistic. Therefore, clinicians should increase their awareness of PCI to avoid unnecessary misdiagnosis.

Pneumatosis cystoides intestinalis in a patient with aseptic meningitis: a case report.

BACKGROUND: Although pneumatosis cystoides intestinalis (PCI) is observed in patients who are on corticosteroid treatment, most patients have underlying diseases requiring long-term corticosteroid treatment. Herein, we present a rare case of a patient with aseptic meningitis who had PCI of the ascending colon while receiving betamethasone treatment. CASE PRESENTATION: A 46-year-old man was sent to our institution due to disturbance in consciousness and general weakness. Brain computed tomography (CT) scan showed multiple hyperdense lesions over the bilateral hemisphere at the white-gray matter junction. Empiric antibiotic treatment with vancomycin and ceftriaxone was prescribed. Due to acute generalized exanthematous pustulosis (AGEP), we ordered betamethasone and diphenhydramine. Two days later, the patient had bloating and abdominal tenderness. Moreover, contrast-enhanced abdominal CT scan revealed PCI of the ascending colon. Since ischemic bowel disease was suspected, laparoscopy and colonoscopy were carried out. However, no abnormal mucosa or mass lesion was noted. Then, tachycardia, hypotension, and change in consciousness along with loss of brainstem reflex and increased intracranial pressure were noted. After further treatment, the patient's condition worsened, and he eventually died. CONCLUSION: As the outcomes of PCI range from benign to life-threatening, an accurate diagnosis must be made to prevent unnecessary abdominal surgeries. Benign PCI in a patient without PCI correlated to underlying diseases, but received short-term corticosteroid treatment should be considered.

"Cystamatic" Review: Is Surgery Mandatory for Pneumatosis Cystoides Intestinalis?

INTRODUCTION: Pneumatosis cystoides intestinalis (PCI) is a rare condition characterized by the presence of intramural gas cysts within the small and large intestines. We describe a case of a 70-year-old man admitted to the Emergency Surgery Department for PCI who was treated conservatively. AREAS COVERED: We reviewed 60 cases of PCI described in the international literature over the last 5 years. From our analysis, it appears that the etiology of the gas production within the submucosa or the subserosa of the gastrointestinal tract is still unknown. The rupture of the cysts can lead to pneumoperitoneum that can simulate a surgical emergency. EXPERT COMMENTARY: For patients with PCI, a conservative approach is the treatment of choice, with surgery mandatory only for complicated disease.

Pneumatosis cystoides intestinalis: six case reports and a review of the literature.

BACKGROUND: Pneumatosis cystoides intestinalis (PCI) is characterized by gas-filled cysts in the intestinal submucosa and subserosa. There are few reports of PCI occurring in duodenum and rectum. Here we demonstrated four different endoscopic manifestations of PCI and three cases with intestinal stricture all were successfully managed by medical conservative treatment. CASE PRESENTATION: There are 6 cases of PCI with varied causes encountered, in which the etiology, endoscopic features, treatment methods and prognosis of patients were studied. One case was idiopathic, while the other one case was caused by exposing to trichloroethylene (TCE), and the remaining four cases were secondary to diabetes, emphysema, therioma and diseases of immune system. Of the six patients, all complained of abdominal distention or diarrhea, three (50%) reported muco-bloody stools, two (33.3%) complained of abdominal pain. In four other patients, PCI occurred in the colon, especially the sigmoid colon, while in the other two patients, it occurred in duodenum and rectum. Endoscopic findings were divided into bubble-like pattern, grape or beaded circular forms, linear or cobblestone gas formation and irregular forms. After combination of medicine and endoscopic treatment, the symptoms of five patients were relieved, while one patient died of malignant tumors. CONCLUSION: PCI endoscopic manifestations were varied, and radiology combined with endoscopy can avoid misdiagnosis. The primary bubble-like pattern can be cured by endoscopic resection, while removal of etiology combined with drug therapy can resolve majority of secondary cases, thereby avoiding the adverse risks of surgery.

Pneumatosis cystoides intestinalis associated with sunitinib and a literature review.

BACKGROUND: Pneumatosis cystoides intestinalis (PCI) is a rare self-limiting condition characterized by air-filled cysts within intestinal walls. Diagnosis should be prudent because it can mimic pneumoperitoneum leading to unnecessary treatment such as surgical exploration. Although various drugs including anti-neoplastic agents have been suggested as etiologies, cases related to sunitinib are sparse. Because of the rarity of this unusual side effect by sunitinib, we report the case report. CASE PRESENTATION: A 68-year-old female with pancreatic neuroendocrine tumor who was treated with sunitinb for 4 months visited to our hospital complaining of severe diarrhea and mild abdominal discomfort. The abdominal X-ray showed subdiaphragmatic air mimicking intestinal perforation. After the meticulous evaluation including abdomino-pelvic computed tomography, the patient was diagnosed of PCI induced by sunitinib and fully recovered with conservative management. CONCLUSIONS: It is important to note that PCI can develop after treatment with sunitinib because PCI has not been widely known as an adverse event caused by the agent. Furthemore, emergent surgery while sunitinib was administrated without adequate washout period can result in substantial surgical complications which could be avoided with the precise diagnosis.

Pneumatosis intestinalis in an SIV-infected rhesus macaque (Macaca mulatta).

Pneumatosis intestinalis (PI) has been described as an incidental finding in domestic animals and humans where it is associated with human immunodeficiency virus infection among other comorbidities. This report describes emphysematous changes consistent with PI in a simian immunodeficiency virus (SIV)-infected rhesus macaque (Macaca mulatta).

A rare case of extensive pneumatosis cystoides intestinalis with intestinal malrotation: Case report.

Intramural bowel gas (Pneumatosis intestinalis) refers to the radiological or clinical evidence of gas within the wall of the bowel lumen. While intramural gas could be secondary to life-threatening pathologies such as mesenteric ischemia in adults and necrotizing enterocolitis in neonates, it could also occur as a rare benign sub-type called Pneumatosis cystoides intestinalis, which is characterized by multiple gas-filled cysts in the submucosa and/or subserosal of the gastrointestinal tract. Distinguishing between life-threatening Pneumatosis intestinalis and its benign subtypes requires careful clinical and imaging evaluation. This involves identifying additional findings that could indicate potentially concerning causes of Pneumatosis intestinalis. Recognizing these signs is essential for effectively managing the patient because conservative management is preferred for Pneumatosis cystoides intestinalis. In this case study, we describe a patient presenting to our hospital with chronic intermittent abdominal pain persisting for about 2 years, accompanied by episodic vomiting. An abdominal CT scan revealed the presence of multiple air-filled cysts within the wall of the mal-rotated cecal bowel loop, which is abnormally located in the right upper quadrant. Associated with this pneumoperitoneum is seen in the peritoneal cavity. No other significant findings were observed on the scan. To our knowledge, this is the first case of pneumatosis cystoid interstinalis occurring in a mal-rotated gut. We also delve into the potential etiologies and management strategies for Pneumatosis cystoides intestinalis, as well as differentiating signs from the life-threatening intramural gas variant.

An unusual case of small bowel volvulus due to pneumatosis cystoides intestinalis: Case report and literature review.

INTRODUCTION AND IMPORTANCE: Pneumatosis cystoides intestinalis (PCI) is an uncommon condition characterized by intramural gas accumulation in the intestinal submucosa. Idiopathic or secondary is presented with non-specific clinical signs; in some cases, diagnosis is incidental. Its acute presentation is uncommon, and surgical management could be performed in selected cases. CASE PRESENTATION: We present the case of an 85-year-old woman with 3 days of abdominal pain, 6 months of weight loss, and abdominal distension after meals. Abdominal computed tomography evidenced PCI at the small intestine with changes due to intestinal ischemia and internal mesenteric hernia. Intestinal resection and lateral-lateral mechanical anastomosis were performed with no complications after 90 days of follow-up. CLINICAL DISCUSSION: PCI is an infrequent and benign condition; pathophysiology is, to date, poorly understood. Idiopathic or secondary to other gastrointestinal pathologies are described. The final diagnosis is performed with histopathological analysis. Nevertheless, in some cases, the benign nature could be presented as an acute abdomen, and surgical management should be in the physician's armamentarium. CONCLUSION: PCI is an uncommon and benign entity. Nevertheless, in some cases, it could be presented as an acute abdomen. The surgical approach is appropriate, safe, and feasible.

The Challenge of Pneumatosis Intestinalis: A Contemporary Systematic Review.

PURPOSE: Pneumatosis intestinalis is a radiological finding with incompletely understood pathogenesis. To date, there are no protocols to guide surgical intervention. METHODS: A systematic review of literature, according to PRISMA criteria, was performed. Medline and PubMed were consulted to identify articles reporting on the items "emergency surgery, pneumatosis coli, and pneumatosis intestinalis" from January 2010 up to March 2022. This study has not been registered in relevant databases. RESULTS: A total of 1673 patients were included. The average age was 67.1 ± 17.6 years. The etiology was unknown in 802 (47.9%) patients. Hemodynamic instability (246/1673-14.7% of the patients) was associated with bowel ischemia, necrosis, or perforation (p = 0.019). Conservative management was performed in 824 (49.2%) patients. Surgery was performed 619 (36.9%) times, especially in unstable patients with bowel ischemia signs, lactate levels greater than 2 mmol/L, and PVG (p = 0.0026). In 155 cases, surgery was performed without pathological findings. CONCLUSIONS: Many variables should be considered in the approach to patients with pneumatosis intestinalis. The challenge facing the surgeons is in truly identifying those who really would benefit and need surgical intervention. The watch and wait policy as a first step seems reasonable, reserving surgery only for patients who are unstable or with high suspicion of bowel ischemia, necrosis, or perforation.

Rare etiology of colonic intussusception involving an adult with emphysematous cystic enteropathy: A case report and review of literature.

BACKGROUND: Pneumatosis cystoides intestinalis (PCI), characterized by a collection of gas-filled cysts in the intestinal wall, is an uncommon but well-known condition in gastroenterology. Abdominal pain is the most frequent symptom associated with PCI. Intussusception represents a potential cause of recurrent abdominal pain or emergency presentation. However, the occurrence of colonic intussusception secondary to PCI is very unusual in adulthood. CASE SUMMARY: A 52-year-old male, known with idiopathic PCI, presented seventeen months after initial diagnosis with a new right upper quadrant pain. A computed tomography-scan demonstrated a colonic intussusception at the hepatic flexure. PCI did not progress compared with initial investigation. The patient underwent an emergency right hemicolectomy. CONCLUSION: Resection was recommended in this case because PCI proved to be persisting with no identified curable cause. Surgery allowed to address the underlying pathology, the potential relapse of intussusception, and the likely cause of recurrent abdominal pain, either invagination or PCI itself.

Pneumatosis Cystoides Intestinalis in Muscular Dystrophy and Congenital Myopathies: A Report of Five Cases.

Pneumatosis cystoides intestinalis (PCI) is a rare disease wherein air accumulates in the intestinal subserosa and submucosa, causing multiple gaseous cysts within the gastrointestinal wall. While PCI has various known risk factors, reports identifying muscular diseases as a factor are scarce. The aim of this study is to elucidate the clinical characteristics of PCI in muscle disease. We present a case series of five cases, including two cases of Duchenne muscular dystrophy (DMD) and three cases of rare congenital myopathies. All cases are of male patients, with poor intestinal peristalsis and constipation, who underwent tube feeding and mechanical ventilation via tracheostomy. They had no signs of severe complications, such as intestinal necrosis, and all of them improved with conservative treatment. Case 1 is a 23-year-old man with DMD who developed cardiopulmonary arrest at the age of 20 years. Pulmonary hemorrhage occurred three months before the incidental detection of PCI in the ascending colon, which resolved with conservative oxygen treatment. Case 2 is a 25-year-old man with DMD who progressed to immobility necessitating tracheostomy at the age of 20 years. He experienced persistent abdominal pain and nausea, and PCI was detected in the cecum and ascending colon. He showed near-complete resolution of PCI after three months of conservative treatment. Case 3 is a six-year-old boy with reducing body myopathy. Constipation was diagnosed at four years of age. He experienced intermittent bloody stools, leading to the incidental detection of PCI at six years of age. After two months of conservative treatment, the PCI resolved with no subsequent recurrence. Case 4 is a 33-year-old man with infantile severe myotubular myopathy. He required mechanical ventilation immediately after birth and later underwent tracheostomy and tube feeding due to complications. At the age of 27 years, PCI was incidentally detected on abdominal CT. He had episodes of remission and worsening for a few years; however, PCI completely resolved after three years. Case 5 is a 27-year-old man with nemaline myopathy. At the age of 14 years, he had persistent bloody stools. After lower gastrointestinal endoscopy, he was diagnosed with PCI with numerous rectal cysts. PCI required no specific therapeutic intervention. There was spontaneous resolution of PCI and bloody stools. Given that PCI lacks specific symptoms and cases with muscular diseases often experience abdominal issues, many cases are liable to be overlooked or misdiagnosed. Cases with muscular diseases complaining of persistent abdominal symptoms should undergo radiographic imaging to rule out PCI.

Systemic lupus erythematosus presenting with pneumoperitoneum without evidence of pneumatosis cystoides intestinalis-when not to operate-a case report.

Pneumoperitoneum in patients with systemic lupus erythematosus is commonly recognized as a surgical emergency that requires exploration. However, it may not be associated with bowel perforation and may be a benign disease manifestation. We present a case of a young patient who developed spontaneous pneumoperitoneum after pulse steroid therapy for lupus enteritis and was successfully managed conservatively. Patients with connective tissue disease may present with pneumoperitoneum, with or without pneumatosis cystoides intestinalis. Therefore, a detailed clinical history, thorough clinical examination, and laboratory parameters should be evaluated before proceeding with surgical intervention. A conservative approach may be attempted in patients with spontaneous pneumoperitoneum, and surgery should only be considered if clinical deterioration occurs.

Intestinal Emphysema and Gut Bacterial Microbiota Composition.

Pneumatosis cystoides intestinalis, or intestinal emphysema, is a condition characterized by the presence of multiple cystic structures within the gut wall and on the serosal surface of the intestine. Intestinal emphysema represents an accidental finding in swine, although it can be clinically relevant in humans. Its etiology is unknown, and many theories have been proposed. Among them, a bacterial etiology is considered the most likely. Therefore, in this study, the V3-V4 region of the 16S rRNA gene was sequenced from 19 swine ileal tracts, 12 with intestinal emphysema and 7 without lesions, to detect a possible bacterial agent. In parallel, prevalence was estimated. Escherichia-Shigella (13.15%), Clostridium_sensu_stricto_1; s__uncultured_bacterium (7.09%), and Fusobacterium; s_uncultured bacterium (6.60%) were the most abundant species identified. No statistically relevant differences were observed between the pathological and physiological groups. Prevalence ranged from 1.25 to 5.12% depending on the batch. Our results suggest that the gut wall bacterial microbiota greatly match the normal gut microbiota, and that the etiological agent of intestinal emphysema may be (1) undetectable due to the chronicity of the lesions, (2) not considered statistically relevant in comparing the two groups (p < 0.05) and likewise in causing lesions, and (3) undetectable due to contamination. Regarding prevalence, the condition is moderately frequent.