Case report
Abdominal Cocoon Syndrome: A Rare Sequelae of Intestinal Perforation
Correspondence Address :
Dr. Abhilasha Bhargava,
Junior Resident, Department of General Surgery, Jawaharlal Nehru Medical College and Datta Meghe Institute of Higher Education and Research, Sawangi (Meghe), Wardha-442001, Maharashtra, India.
E-mail: abhilasha0318@gmail.com
Abdominal cocoon syndrome is a rare clinical presentation that has been associated with abdominal tuberculosis in rural India. It is also known as sclerosing encapsulating peritonitis, where the small bowel becomes encapsulated by a fibrous membrane due to unclear causes, leading to obstipation. Common symptoms include vomiting, nausea, and constipation. Due to these general clinical symptoms, it can be mistaken for other gastrointestinal disorders, resulting in delayed diagnosis, which may lead to adverse outcomes or even mortality. Surgery is often used to free the entrapped bowel and remove the fibrous tissue, while supportive care and problem management are crucial. The present case involves a 55-year-old male who presented with a distended abdomen and obstipation, leading to intestinal perforation and sclerosing encapsulating peritonitis, which was managed through resection and anastomosis of the small intestine. The patient was followed-up after three months with no new complaints. The present case helps in understanding the sequelae of acute intestinal perforation that can result in abdominal cocoon syndrome.
Acute abdomen, Case report, Constipation, Perforation peritonitis, Sclerosing encapsulating peritonitis
A 55-year-old male presented to the emergency ward with a distended abdomen, complaints of obstipation, and vomiting for two days. On examination, the patient had tachycardia and was normotensive. There was no history of similar complaints or any past surgeries. Perabdominal examination showed the abdomen to be tender, rigid with guarding, and distended. On percussion, a dull note was appreciated over the abdomen. On auscultation, the bowel sounds were sluggish. He presented with leucocytosis, hypoalbuminaemia, and marginally high liver enzymes. He was shifted to the Intensive Care Unit (ICU) for close monitoring. The ultrasound of the abdomen could not determine the level of perforation as most of it was obscured by bowel gas. Therefore, a Contrast-enhanced Computed Tomography (CECT) abdomen was performed, which suggested a jejunal perforation along with a mass approximately 9.2×8.4 cm adhered to small bowel loops in a capsulated form resembling a cocoon and gross ascites. Based on the CECT findings, a radiological diagnosis of abdominal cocoon syndrome with intestinal perforation and gross ascites was made (Table/Fig 1). The patient underwent emergency exploratory laparotomy. Intraoperatively, upon incising the peritoneum, 2.5-3 litres of clear fluid were suctioned out. Evidence of a mass comprising loops of small intestine covered with a thick, translucent, whitish membrane resembling a cocoon was observed (Table/Fig 2),(Table/Fig 3). After careful dissection of the membrane and probing the loops of the small intestine, a jejunal perforation of 2×2.5 cm was found, 200 cm distal to the Double J (DJ) flexure (Table/Fig 4). In the present case, jejuno-jejunal resection and anastomosis were performed to correct the intestinal perforation. After addressing the aetiology of the perforation, the patient was closely monitored until the recovery of normal peristalsis. As the patient passed stools and was asymptomatic postoperatively, no active intervention was required apart from observation and regular follow-up.
The postoperative Intensive Care Unit (ICU) stay of the patient was uneventful. The abdominal drain was removed on postoperative day 5, and suture removal was performed on day 10. During the follow-up after three months, the patient presented with a healthy scar site and no new complaints.
‘Abdominal cocoon syndrome’ is also known as ‘Encapsulating peritoneal sclerosis’. Sclerosing encapsulated peritonitis was first identified in 1907 by Owtschinnikow and was termed as ‘Abdominal cocoon’ in 1978 by Foo KT et al., (1). The disorder is called ‘cocoon syndrome’ due to an abnormal membrane surrounding some portion or all of the small intestine. Common clinical presentations of this disease include abdominal pain, nausea, vomiting, abdominal mass, or distension (2). An unclear inflammatory process has been listed as an underlying cause of this acquired disorder. Males are more likely to have abdominal cocoon syndrome compared to females with a ratio of 1.2-2:1 (3) and has been classified into primary and secondary based on disease aetiology, occurrence without any underlying cause is primary and disease presentation with known underlying reasons is termed as secondary (4). However, a female predominance has been reported in India (5). It is distinguished by a dense, concertina-like grayish-white fibrotic membrane that envelops the small intestine either entirely or partly. Acute intestinal obstruction is the most frequent manifestation of symptomatic abdominal cocoon (4),(6). Cases of abdominal cocoon syndrome reported in recent research literature have been listed in (Table/Fig 5) (2),(6),(7),(8),(9),(10),(11),(12),(13),(14),(15),(16),(17),(18),(19). On the contrary, this case presented with obstipation and abdominal distension. The pathophysiology of this illness is subject to several hypotheses, with a previous history of abdominal tuberculosis being one of the common reasons in rural areas of India (20). There was no history of abdominal tuberculosis noted in this patient. The differential diagnosis based on presenting features included encapsulating peritoneal sclerosis, small bowel obstruction, gross ascites, calcifying fibrous tumour of mesentery, omental rhabdomyosarcoma, and abdominal tuberculosis, which was ruled out intraoperatively as it was found to be a localised mass consisting of adhered bowel loops. Abdominal cocoon syndrome carries a high morbidity and mortality rate. The overall mortality rate of abdominal cocoon syndrome has been reported as 35%-74% based on the severity of the disease (21). Surgical management is recommended due to the nature of adhesions and bowel entrapment. A complete sclerosing membrane is removed for resolution of associated complications and recurrence with resection/anastomosis required only in cases of iatrogenic damage (22),(23).
The preoperative diagnosis might be incidental in cases of abdominal cocoon syndrome, as clinical symptoms are unclear and delayed diagnosis is related to adverse outcomes, including mortality. Radiological methods can aid in preoperative confirmation of the diagnosis, thereby assisting in surgical management.
DOI: 10.7860/JCDR/2024/69380.19345
Date of Submission: Jan 01, 2024
Date of Peer Review: Jan 31, 2024
Date of Acceptance: Mar 18, 2024
Date of Publishing: May 01, 2024
AUTHOR DECLARATION:
• Financial or Other Competing Interests: None
• Was informed consent obtained from the subjects involved in the study? No
• For any images presented appropriate consent has been obtained from the subjects. Yes
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ETYMOLOGY: Author
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