Sclerosing encapsulating peritonitis associated withpropranolol usage: A case report and review of the literature

Sumit KALRA,* Antwan ATIA,* Jason McKINNEY,* Thomas R BORTHWICK† & Roger D SMALLIGAN‡

Department of *Internal Medicine and †Gastroenterology, James H. Quillen VA Medical Center, and ‡GeneralInternal Medicine, Quillen College of Medicine, East Tennessee State University, Johnson City, Tennessee, USA

INTRODUCTION

Sclerosing encapsulating peritonitis is a rare cause ofintestinal obstruction that is characterized by thick,white, fibrous membrane formation in the perito-neum that partially or completely encases the bowelloops (the abdominal cocoon syndrome). The mostcommonly identified cause of this condition is con-tinuous ambulatory peritoneal dialysis, but some betaadrenergic receptor blocker-associated cases have alsobeen reported in the literature. We present a case andreview the literature of sclerosing encapsulating peri-tonitis associated with propranolol use and discussthe presentation, etiopathogenesis and managementof this rare condition.

CASE REPORT

A 59-year-old man presented with a 5-day history ofepisodic colicky abdominal pain, abdominal disten-sion and vomiting. This was his ninth admission inthe past year with similar symptoms. On each previ-ous occasion he was managed conservatively andrecovered, only to have a recurrence a few weeks tomonths later. His past history was also significant forcryptogenic cirrhosis of the liver with portal hyperten-sion, diabetes mellitus and hypertension. He had nohistory of abdominal surgery, peritoneal dialysis, orspontaneous bacterial peritonitis. His medicationsincluded propranolol, lisinopril, furosemide, spirono-lactone, insulin, gabapentin, lactulose and sertraline.He was a non-smoker, drank no alcohol and had no

history of illicit drug use. On physical examinationhe was a middle-aged man of average build. Hispulse was 78/minute, regular, blood pressure 116/76 mm Hg and he was afebrile. His chest examinationrevealed clear lungs bilaterally and his heart soundswere normal without any murmurs, gallops or rubs.His abdomen was distended, tympanitic, firm butnon-tender. Bowel sounds were present and mildlyincreased and there was no obvious ascites by clinicalexamination. Laboratory testing showed a hemo-globin of 10.9 g/dL, white blood cell count 7800cells/mm3, serum sodium 140 mmol/L, potassium4.2 mmol/L, chloride 110 mmol/L, bicarbonate24 mmol/L, glucose 10.6 mmol/L, bilirubin18.81 mmol/L, aspartate aminotransferase 31 U/L,alanine aminotransferase 32 U/L and alkaline phos-phatase 183 U/L. An abdominal computed tomogra-phy (CT) scan (Fig. 1) showed dilated loops of thesmall bowel with air fluid levels and a small amountof ascites but no other significant pathology. He wasdiagnosed with partial small bowel obstruction andmanaged conservatively with bowel rest, i.v. fluids,anti-emetics and parenteral nutrition. When thepatient failed to improve as quickly as he had onprevious admissions, the surgery service was consultedand an exploratory laparotomy was performed. Uponopening the abdominal cavity thick fibrous tissue wasfound involving the peritoneum that encapsulatedthe abdominal contents. A biopsy of the peritonealcapsule revealed diffuse fibrosis and inflammatorycells consistent with peritoneal fibrosis and sclerosis(Fig. 2). The patient underwent extensive lysis of theadhesions during the laparotomy had an uneventfulrecovery and at the time of writing has been symptomfree for over 9 months.

DISCUSSION

A partial small bowel obstruction is a clinical pro-blem that internists, gastroenterologists and general

Correspondence to: Sumit KALRA, Department of Internal Medicine,James H. Quillen VA Medical Center, Johnson City, Mountain Home,TN, 37684, USA. Email: drsumitkalra@gmail.comFinancial disclosure: None of the authors have any financialconflict of interest related to this manuscript.© 2009 The AuthorsJournal compilation © 2009 Chinese Medical AssociationShanghai Branch, Chinese Society of Gastroenterology andBlackwell Publishing Asia Pty Ltd.

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surgeons face on a regular basis. While adhesions,malignancy and hernias are the most common causes,sclerosing encapsulating peritonitis is a rare clinicalentity that can cause acute, sub-acute or chronic intes-tinal obstruction. Sclerosing encapsulating peritonitiswas first described in 1907 by Owtschinnikow, whoused the term ‘peritonitis chronica fibrosa encapsu-late’.1 Foo et al. coined the term ‘abdominal cocoonsyndrome’ in 1978 due to the cocoon-like appearanceof the fibrotic capsule that is encountered when anexploratory laparotomy is performed.2 The condition

is characterized by the formation of a thick grayish-white fibrous membrane that encases the small bowelpartially or totally.3

A number of hypotheses have been proposed toexplain the pathogenesis of sclerosing peritonitis. Thecondition was described initially in adolescent girlsfrom tropical and subtropical countries.4 This led tothe hypothesis that retrograde menstruation2 or a ret-rograde viral infection through the fallopian tubeswas the underlying etiology of sclerosing peritonitis.5

However, since then this condition has also beendescribed in men and in other age groups,6–8 this origi-nal theory appears to be unfounded. The proposedetiologies include dietary factors such as ergot-infestedpearl millet, greater omentum hypoplasia and mesen-teric vessel malformation, though each of these issimply speculative.9–11 There are a number of otherconditions and factors associated with its occurrence,the most common of which is continuous ambulatoryperitoneal dialysis.12,13 Other associations reported inthe literature include ovarian thecomas, tuberculouspelvic inflammatory disease, ventriculoperitonealshunts, systemic lupus erythematosus, orthotopic livertransplantation, protein S deficiency, ovarian cysts,keratoconjunctivitis sicca syndrome and various betaadrenergic blocking agents.14–21 The exact mechanismby which beta blockers induce sclerosing peritonitis isunknown, but two suggested hypotheses include apossible allergic type reaction to the class of drug orperhaps an excess production of intraperitoneal col-lagen, which has been shown to be induced by theseagents.22,23

The clinical manifestations of sclerosing peritonitiscan be nonspecific and include vague, colicky abdomi-nal pain, weight loss, nausea and vomiting, all largelyrelated to the partial small bowel obstruction the con-dition induces.9 Sclerosing peritonitis can also presentwith recurrent ascites or as an abdominal mass.Although the diagnosis is most commonly made atlaparotomy, occasionally it is suspected on radiologi-cal imaging. On CT scan, sclerosing peritonitis canappear as peritoneal thickening or calcification, locu-lated fluid collections or matting of the small bowel.Thickening of the small bowel loops and calcificationsof the liver capsule, spleen, posterior peritoneal wallor bowel can also be seen.24 Ultrasound and magneticresonance imaging have also been useful in makingthis diagnosis.25–27

Our patient did not have a history of any of the mostcommonly associated conditions, but had been taking

Figure 1. Computerd tomography scan of the abdomenshows dilated loops of small bowel with air fluid levels andsmall amount of ascites.

Figure 2. Biopsy of the peritoneal capsule shows diffusefibrosis and inflammatory cells.

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propranolol for over 4 years at the time of diagnosis.Although he was also taking other medications, noneof these have been linked to sclerosing peritonitis. Areview of the literature revealed that several beta block-ers have been associated with sclerosing encapsulatingperitonitis, the first of which was practolol in 1971.21

Practolol was also linked to a diffuse fibrotic disordercalled the oculomucocutaneous syndrome, whichcauses fibrotic changes in the eyes, skin, peritonealand pleural membranes. The median latency periodbetween usage of practolol and the development ofsclerosing peritonitis was 4 years in reported cases.28

These adverse reactions led to practolol being with-drawn from the market in 1975. Since then, casereports involving other beta blockers have been pub-lished including timolol, metoprolol, atenolol, oxpre-nolol, sotalol and propranolol.22,23,29–31 Two such casereports describing the association of propranolol andsclerosing peritonitis are summarized in Table 1.22,31

Each of these cases involved men taking propranololfor 3 months to 2 years for treatment of chronic angina.

The treatment of sclerosing peritonitis is mainly sur-gical with lysis of the adhesions at laparotomy. A ret-rospective analysis of 32 patients with this conditionwas done to determine the best surgical managementcomparing membrane resection, enterolysis, partialexcision of the membrane, intestinal resection andexploratory laparotomy only. Membrane resectionresulted in less patients having persistent bowelobstruction or death and the study recommended thisbe attempted when feasible. No surgical treatment wasrecommended in cases of associated ascites, asymp-tomatic sclerosing peritonitis, or sub-acute intestinalobstruction.32

While this is a rare clinical entity and the causative roleof propranolol cannot be established with absolutecertainty, such clinical observations build on currentknowledge and it is hoped they will eventually con-

tribute to a better understanding of the etiology of thissyndrome. Our case reminds physicians to considerthe abdominal cocoon syndrome in the differentialdiagnosis of a patient with recurrent small bowelobstruction, especially in patients on propranololtherapy or with any of the other predisposing condi-tions mentioned.

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Table 1. Summary of patients describing association between propranolol and sclerosing peritonitis

Patient 1 Patient 2

Patient’s age 56 43Patient’s sex Male MaleDose of propranolol 320 mg/day 20 mg q.i.d.Indication for propranolol usage Hypertension and angina pectoris Angina pectorisDuration of propranolol usage 2 years 2 monthsClinical picture Discovered during laparotomy for rectal

hemorrhageSmall bowel obstruction and ascites

Diagnostic method During laparotomy During laparotomyOutcome Patient recovered Patient recovered

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