Pneumatosis intestinalis due to gastrointestinal amyloidosis: A case report &amp; review of literature

Khalid, Filza; Kaiyasah, Hadiel; Binfadil, Wafa; Majid, Maiyasa; Hazim, Wessam; Eltayeb, Yousif H

doi:10.1016/j.ijscr.2016.03.044

Cited by 10 publications

(14 citation statements)

References 13 publications

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“…Other cases have been reported of pneumatosis intestinalis as the presenting symptom of amyloidosis. These patients also did well, usually with conservative management 12 13. It is important to note that none of these patients had mention of severe liver dysfunction at the time of diagnosis, or throughout their hospital stays.…”

Section: Discussionmentioning

confidence: 90%

“…Patients with solid organ, namely hepatic and splenic ruptures have been mentioned previously. Gastrointestinal tract perforations have also been noted, with an inciting event infrequently identified 10–13. Patients with these issues generally had favourable short-term outcomes, with reversal of the acute issue often enough to return them to their previous state of health, although one patient died from postoperative complications 10 11.…”

Section: Discussionmentioning

confidence: 99%

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Spontaneous hepatic rupture due to primary amyloidosis

et al. 2019

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Spontaneous hepatic rupture is an uncommon cause of haemorrhagic shock and very rarely happens due to amyloidosis. This report describes one such case in which a middle-aged man presented in extremis. He was managed initially with massive transfusion, interventional radiology embolisation and decompressive laparotomy for abdominal compartment syndrome. Subsequent coagulopathy was treated with activated factor VII due to deficient native activity. Serum protein electrophoresis and liver biopsy during his hospital course yielded a diagnosis of amyloidosis, which was treated palliatively with steroids and bortezomib. Despite supportive care, he died 10 days after presentation. This case illustrates the importance of considering an uncommon pathology when a patient presents with a condition in an uncommon way.

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Section: Discussionmentioning

confidence: 90%

Section: Discussionmentioning

confidence: 99%

Spontaneous hepatic rupture due to primary amyloidosis

et al. 2019

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“…Although the phenomenon of PI has been described since over 100 years [2,7], hematologists and specialists in other disciplines (outside the field of gastroenterology) are less familiar with this rare condition. PI does not represent a closed entity, but rather has to be interpreted as a radiological finding that can be caused by benign conditions up to fulminant gastrointestinal diseases with a lethal outcome.…”

Section: Discussionmentioning

confidence: 99%

“…Most patients develop secondary PI caused by various underlying disorders including autoimmune disorders like systemic sclerosis [1], gastrointestinal amyloidosis [2], mechanical intestinal obstruction, and acute mesenteric ischemia [3]. Compared to these disorders, only a few patients develop primary benign idiopathic PI [4].…”

Section: Introductionmentioning

confidence: 99%

Intestinal Pneumatosis Associated with Tuberculosis after Allogeneic Hematopoietic Stem Cell Transplantation

Venkataramani

Hosseini²,

Schulze³

et al. 2016

Acta Haematol

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Pneumatosis intestinalis (PI), defined as intestinal intra- and extramural gas accumulation, is a rare radiographic finding in conditions of intestinal wall damage of varied etiology. Here, we report on a 56-year-old female with multiple myeloma who presented with undulating fever, fluctuating abdominal symptoms, and a distended abdomen 5 months after allogeneic hematopoietic stem cell transplantation (HSCT). Abdominal X-ray and CT scan documented PI with gas accumulation both in the intestinal and colonic bowel walls. Concurrently, thoracic CT revealed mediastinal and bihilar lymphadenopathy associated with bilateral pleural effusions. Microscopy of bronchoalveolar lavage fluid (BALF) revealed acid-fast bacilli, which were identified as Mycobacterium tuberculosis. Tuberculostatic treatment resulted in timely clinical improvement, a complete clearance of the radiological and clinical findings of PI, and the control of the tuberculosis (Tbc), determined by multiple negative BALF results. Taken together, PI occurred as the initial symptom of Tbc in an allogeneic stem cell recipient, achieving complete recovery by tuberculostatic treatment only.

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“…Pneumatosis intestinalis (PI) is a radiological finding, and its pathogenesis is not entirely understood 1. PI is presented by the presence of free gas in subserosal or submucosal layers of the intestinal wall and cyst formation within the gastrointestinal (GI) tract and tissues around it 2 3…”

Section: Introductionmentioning

confidence: 99%

Pneumatosis intestinalis in an adult patient with antral stenosis and midgut malrotation

Mehrkhu

Dehkordi

et al. 2018

BMJ Case Reports

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Pneumatosisintestinalis (PI) is a radiological finding with about 0.03% incidence and incompletely understood pathogenesis. We report a case of PI with a rare presentation of pneumoperitoneum that underwent diagnostic surgery and finally diagnosed by midgut malrotation and antral stenosis. A 40-year-old man with 1-year history of dyspepsia and vomiting which was aggravated by 20 kg underweight since 3 months ago, despite medications was presented. His imaging examinations showed partial antral obstruction besides pneumoperitoneum and gas collection in the small intestine wall, which were in favour of PI. Samplings of the small intestine during laparotomy reported simple serosal cysts. Persistence of his symptoms forced him to recourse to another hospital and their new imaging revealed Ladds' band in addition to mentioned findings in previous studies; he underwent subtotal gastrectomy plus Ladds' band division by Braun gastrojejunostomy. PI could be a presentation of antral stenosis and midgut malrotation. PI with pneumoperitoneum needs surgical interventions. Considering that most of the midgut malrotation cases are diagnosed in the first year of life and it is very rare in adults, it may bethat these new findings are due to adhesion band formation after the first surgery in this case.

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Pneumatosis intestinalis due to gastrointestinal amyloidosis: A case report & review of literature

Cited by 10 publications

References 13 publications

Spontaneous hepatic rupture due to primary amyloidosis

Spontaneous hepatic rupture due to primary amyloidosis

Intestinal Pneumatosis Associated with Tuberculosis after Allogeneic Hematopoietic Stem Cell Transplantation

Pneumatosis intestinalis in an adult patient with antral stenosis and midgut malrotation

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