|CASE IN POINT - CLINICS IN SURGERY
|Year : 2018 | Volume
| Issue : 4 | Page : 259-261
Revisiting the enigmatic abdominal cocoon
Rushil Jain, Vimal Jain, Shalit Jolly
Department of General Surgery, Mata Chanan Devi Hospital, Janakpuri, New Delhi, India
|Date of Web Publication||29-Oct-2018|
Department of General Surgery, Mata Chanan Devi Hospital, Janakpuri, New Delhi
Source of Support: None, Conflict of Interest: None
Abdominal cocoon, the idiopathic form of sclerosing encapsulating peritonitis (SEP) is a rare condition of unknown etiology that results in intestinal obstruction due to partial or total encapsulation of the small bowel by a thick fibrotic membrane in a concertina like fashion, hence the name cocoon. It primarily affects adolescent girls living in tropical and subtropical regions. The early clinical features and findings of imaging diagnostic tools are non specific and difficult to make definitive diagnosis preoperatively. Surprisingly, with proper surgical management, there is complete recovery and recurrence-free later life. We report an unusual presentation in an elderly female who presented with acute intestinal obstruction. The diagnosis could be made only peroperatively when on laparotomy the entire small bowel was cocooned and enclosed in a yellowish white fibrotic membrane resulting in obstruction of small intestine. The membrane was carefully peeled off the intestines and adhesiolysis was done. Patient had complete recovery. Histopathology report was consistent with the diagnosis of primary abdominal cocoon. We are reporting this case so that surgeons are better aware of this condition as a possible cause of intestinal obstruction to facilitate preoperative diagnosis and prevent unnecessary bowel resection at laparotomy for good surgical outcome.
Keywords: Abdominal cocoon, adhesiolysis, idiopathic sclerosing encapsulating peritonitis, intestinal obstruction
|How to cite this article:|
Jain R, Jain V, Jolly S. Revisiting the enigmatic abdominal cocoon. Astrocyte 2018;4:259-61
| Introduction|| |
Abdominal cocoon, a rare cause of intestinal obstruction, was first observed by Owstschinnikow as early as1907 who termed it peritonitis chronic fibrosa incapsulata., Sclerosing encapsulating peritonitis (SEP) can be classified as idiopathic or secondary. The idiopathic form known as abdominal cocoon was first described by Foo et al. in 1978. It primarily affects young adolescent girls in tropical and subtropical zones. Till date, only few cases have been reported. Thick fibrotic peritoneum encasing the small bowel partially or completely is a pathognomonic feature. Preoperative diagnosis requires very high index of suspicion. Ultrasonography, contrast-enhanced computed tomography (CECT) of the abdomen are useful tools to diagnose this condition and rule out other causes of intestinal obstruction. Peritoneal sac excision and adhesiolysis the treatment with satisfactory outcome.
| Case Report|| |
An unusual presentation in an elderly female is reported who presented with acute intestinal obstruction. A 45-year-old lady presented with complaints of pain in the abdomen for 2 days. Pain was generalized, dull aching, and continuous type. Pain was neither radiated nor referred to any other site. She also complained of non passage of stools/flatus for the last 2 days. There was no history of previous abdominal surgery/intervention, lump abdomen, jaundice, anorexia or weight loss, no urinary complaints, or any menstrual irregularities. On examination, she had a sick and anxious look, was febrile, pulse rate was 100/min and blood pressure was 110/70 mmHg. Abdomen was distended with guarding, rigidity. Bowel sounds were sluggish. Other organ systems evaluation was normal. Ryles tube was put in and immediately 1200 ml fluid was aspirated, contents of which were bilious and contained partially digested food material. X-ray of the abdomen showed centrally located multiple air fluid levels. Ultrasound of the abdomen showed mild free intraperitoneal fluid and dilated small bowels. CECT of the abdomen showed small bowel obstruction and thick fibrotic bands with free fluid [Figure 1]. Complete blood counts, liver function test, and kidney function test were within normal limits. Diagnosis of acute intestinal obstruction due to adhesions was made. On exploratory laparotomy, the entire small bowel was cocooned and enclosed in a yellowish white fibrotic membrane resulting in obstruction of small intestines [Figure 2]. The membrane was carefully peeled off the small intestines and adhesiolysis was done. The diagnosis of abdominal cocoon was made on laparotomy. Operative phase was uneventful. Patient however had prolonged ileus in postoperative period, was managed in ICU with intravenous (IV) antibiotics, IV fluids, fresh frozenplasma, and blood transfusion. Patient passed flatus on 6th day. On 10th postoperative day patient was discharged in stable condition. The histopathology report was of nonspecific chronic inflammatory reaction consistent with primary abdominal cocoon.
|Figure 1: CECT Showing Small Bowel Obstruction Secondary to Thick Fibrotic Bands.|
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|Figure 2: Intraoperative Photograph Showing Thick Fibrotic Layer Encasing Small Intestines.|
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| Discussion|| |
SEP is an uncommon cause of intestinal obstruction. It can be classified as primary or idiopathic and secondary. The idiopathic form known as abdominal cocoon was first described by Foo et al. in 1978. It primarily affects young adolescent girls in tropical and subtropical zones. Till date, only few cases have been reported. Thick fibrotic peritoneum encasing the small bowel partially or completely is a pathognomonic feature. The etiology of abdominal cocoon or idiopathic SEP has remained largely unknown. Various hypotheses have been proposed. These include retrograde menstruation with a superimposed viral infection, retrograde peritonitis, and cell-mediated immunological tissue damage incited by gynecological infection.,,, Greater omentum hypoplasia and mesenteric vessel malformation have been demonstrated in some cases. The secondary form has been reported in continuous ambulatory chronic peritoneal dialysis. Other causes include prior abdominal surgery, use of povidone iodine for abdominal washout, recurrent peritonitis, practolol treatment (practolol has been withdrawn from use because of this), PV shunting, and more rarely abdominal tuberculosis, sarcoidosis, familial Mediterranean fever, intraperitoneal chemotherapy, cirrhosis, liver transplantation, gastrointestinal malignancy, endometriosis, luteinized ovarian thecomas, protein S deficiency, dermoid cyst rupture, and fibrogenic foreign material. The clinical presentation of abdominal cocoon mostly occurs as acute abdomen, which in most cases requires surgical intervention. The early clinical features of SEP are nonspecific and often not recognized. Patient presents with recurrent abdominal pain, nausea, vomiting, anorexia, weight loss, malnutrition, recurrent episodes of acute, subacute or chronic small bowel, incomplete or complete obstruction and rarely with a palpable soft, and nontender abdominal mass. Diagnosis needs a high index of suspicion as signs and symptoms are nonspecific. Yip and Lee suggested four main clinical features for diagnosis –relatively young female patient without an obvious cause of bowel obstruction, past history of similar episodes with symptomatic relief, symptoms of bowel obstruction but absence of cardinal signs such as distention, and presence of soft nontender mass. Differential diagnosis is mainly from internal hernias, voluminous intussusception, simple peritoneal adhesions. Findings of ultrasonography and CT are not conclusive. Conventional radiographs may show dilated bowel loops and air fluid levels. Contrast studies may show varying lengths of small bowel tightly enclosed in a cocoon of thickened peritoneum, proximal small bowel dilatation and increased transit time. It may show fixed cluster of dilated bowel loops lying in a concertina fashion giving a cauliflower-like appearance –cauliflower sign. Ultrasound findings described include a trilaminar appearance of bowel wall, tethering of bowel to posterior abdominal wall, dilatation and fixation of small bowel loops, thick-walled mass containing bowel loops best visible only in presence of ascites, and small bowel loops arranged in concertina shape –cauliflower appearance. CT of the abdomen may help in noninvasive diagnosis by demonstrating small bowel loops congregated to the centre encased by a thick membrane and also exclude other causes of obstruction. Tethering or matting of the small bowel loops posterior to loculated fluid collection or floating within the fluid may be seen. Retraction of mesentery can lead to tethered bowel loops dubbed as “gingerbread man” sign. Abdominal exploration is usually needed for final diagnosis and treatment. Careful dissection and excision of sac –thick fibrotic peritoneal wrap with release of small intestine and lysis of interloop adhesions is the best treatment and leads to complete recovery., Resection of bowel is needed only if it is nonviable, because resection increases morbidity and mortality. An excellent long-term post operative prognosis, most times guaranteed with little risk of recurrence in the long-term follow-up. Better awareness of this entity is needed for suspecting it preoperatively, proper management, prevent unnecessary bowel resections and bad outcomes.
| Conclusions|| |
Abdominal cocoon is a relatively rare cause of intestinal obstruction usually seen in adolescent girls; the case reported here is an unusual presentation in an elderly woman. A high index of clinical suspicion may be generated by recurrent attacks of non strangulating obstruction in the same individual combined with relevant imaging findings and lack of secondary causes. A better awareness and preoperative recognition of entity is important as it results in proper management i.e., surgical removal of wrap –cause of cocoon and prevent unnecessary bowel resection. Prognosis is good and complete recovery is usually the result after surgery.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]