Congenital peritoneal encapsulation: A review and novel classification system

Table 1 Patient demographics and key clinical characteristics

Case	Ref.	Year	Country	Age	Sex	Clinical Features	Tests	Management	Other
1	McMahon et al[23]	2018	Australia	20	M	Intermittent abdominal pain, distension	CT	Surgical resection of sac	Symptom free recovery
2	Wolski et al[41]	2017	Poland	12	M	Abdominal pain for 1 wk	XR, US	Surgical resection of sac	Post-operative complication of adhesion SBO
3	Griffith et al[13]	2017	United Kingdom	12	M	Abdominal pain and vomiting for 1 wk	XR, US	Surgical resection of sac	Gangrenous acalculous cholecystitis
4	Arumugam et al[6]	2017	India	22	F	Small bowel obstruction, assymetric distension	CT	Surgical resection of sac
5	Zoulamoglou et al[42]	2016	Greece	28	F	Intermittent abdominal pain for 1 yr, asymmetric distension	XR, CT	Surgical resection of sac
6	Teixeira et al[36]	2015	Portugal	25	M	Small bowel obstruction. Fixed, assymetrical distension	XR, CT	Surgical resection of sac
7	Stewart et al[35]	2014	Australia	16	M	Intermittent, chronic abdominal pain	XR, US	Surgical resection of sac
8	Wani et al[40]	2013	India	28	M	Generalised, intermittent abdominal pain	XR, CT, labs	Surgical resection of sac
9	Naidoo et al[26]	2013	India	40	M	Stab wound	XR, fluoro, CT	Surgical resection of sac	Stabbing injury
10	Mitrousias et al[24]	2012	Greece	78	F	3 d of abdominal pain	XR, CT, labs	Surgical resection of sac	Helical pattern on CT
11	Shamsuddin et al[30]	2012	Pakistan	16	F	Small bowel obstruction	XR	Failed conservative. Surgical resection.	Excellent recovery
12	Ince et al[15]	2012	Turkey	71	M	Small bowel obstruction	XR, US, CT	Ileocaecal resection
13	Al-Taan et al[5]	2010	United Kingdom	82	M	Asymptomatic		Surgical resection of tumour and sac	Bowel cancer
14	Kumara et al[17]	2009	Sri Lanka	44	F	Cushing’s syndrome secondary to right adrenal tumour	CT	Surgical resection of tumour and sac	Adrenal tumour
15	Sherigar et al[31]	2007	United Kingdom	85	F	Small bowel obstruction	XR, CT	Surgical resection of sac	Patient died from chest sepsis
16	Basu et al[9]	2006	India	21	F	Distension, peritonism	XR, US, labs	Resection of sac, appendix, lavage	7 yr follow up
17	Chew et al[11]	2006	Singapore	38	M	Small bowel obstruction	XR, CT	Surgical resection of sac
18	Shioya et al[32]	2005	Japan	34	M	Small bowel obstruction, right inguinal hernia	XR, labs	Surgical resection of sac	Excellent recovery
19	Okobia et al[2]	2001	Nigeria	15	F	Abdominal pain
20	Mordehai et al[25]	2001	Israel	14	F	Abdominal pain, vomiting, weight loss	XR, US	Surgical resection of sac	Post-operative ileus
21	Naraynsingh et al[27]	2001	West Indies	64	M	Abdominal pain, fixed asymmetrical distension, differential palpation		Surgical resection of sac
22	Lee et al[19]	2000	South Korea	22	F	Abdominal pain, distension	XR, CT, labs	Failed conservative management. Surgical resection of sac	Excellent recovery
23	Kyaw et al[18]	1998	Singapore	11	M	Abdominal pain for 5 d, soft mass left flank	US, CT	Surgical resection of sac	Hydronephrosis
24	Casas et al[10]	1998	Spain	43	M	Intermittent abdominal pain for 6 mo	XR, fluoro, US, CT	Surgical resection of sac	Hydronephrosis. Asymptomatic at 14 mo
25	Constantinides et al[12]	1998	Italy	49	F	Found at autopsy. Intermittent, severe abdominal pain during life.
26	Adedeji et al[4]	1994	United Kingdom	40	M	Abdominal pain, peritonism for 1 d	XR, labs	Surgical resection of sac
27	Tsunoda et al[38]	1993	Japan	52	M	Small bowel obstruction, central abdominal mass	XR, US, CT	Surgical resection of sac	Asymptomatic at 8 mo
28	Silva et al[34]	1992	Japan	29	M	Intermittent abdominal pain, scaphoid abdomen	XR, fluoro, CT, labs	Surgical resection of sac	Patient died due to gangrenous small bowel
29	Awasthi et al[8]	1991	India	16	F	Abdominal pain for 9 mo, distension	XR, fluoro	Surgical resection of sac	Discharged day 6 with resolution of symptoms.
30	Arora et al[3]	1989	India		F	Abdominal pain			Colorectal cancer
31	Askew et al[7]	1988	United Kingdom		M	Incidental finding during surgery
32	Walsh et al[39]	1988	Ireland	82	M	Small bowel obstruction	XR
33	Huddy et al[14]	1988	United Kingdom	56	M	Intermittent abdominal pain	XR	Surgical resection of sac
34	Lifschitz et al[22]	1987	Ciskei	66	M	Abdominal pain, vomiting, distension for 3 wk	XR, labs	Surgical resection of sac
35	Jamieson et al[16]	1985	United Kingdom			Incidental finding during laparotomy		Surgical resection of sac	Colorectal cancer
36	Sieck et al[33]	1983	Saudi Arabia	14	F	Nausea, vomiting, distension for 3 mo	XR, fluoro, labs	Surgical resection of sac	Patient had breast cancer
37	Sieck et al[33]	1983	Saudi Arabia	65	F	Intermittent pelvic pain, fever, abdominal distension for 5 yr		Surgical resection of sac
38	Sayfan et al[29]	1979	Israel	12	F	Abdominal pain, vomiting for 1 d	XR, labs	Surgical resection of sac	Excellent recovery. Discharged day 7
39	Lewin et al[20]	1970	United States	66	M	Post-mortem examination. Patient died of acute myocardial infarct.
40	Thorlaksen et al[37]	1953	Canada	57	M	Diarrhoea, abdominal pain for several years	XR, labs	Initial conservative management successful.
41	Thorlaksen et al[37]	1953	Canada	53	M	Incidental finding in asymptomatic patient	XR, labs	Surgical resection of sac	Car accident, intra-abdominal haemmorhage
42	Thorlaksen et al[37]	1953	Canada	64	M	Epigastric pain, constipation for 2 yr	XR, labs	Surgical resection of sac
43	Papez et al[28]	1932	United States	61	M	Cadaveric dissection
44	Lickley et al[21]	1907	United Kingdom	52	M	Autopsy. Asymptomatic during life
45	Cleland et al[1]	1868	Ireland			Cadaveric dissection

XR: X-Ray; CT: Computed tomography; US: Ultrasound scan; Fluoro: Fluoroscopic imaging; Labs: Laboratory investigations.

Table 2 Geographical distribution of cases

Country	Case number
United Kingdom	8
India	6
Canada	3
Japan	3
Australia	2
Greece	2
Ireland	2
Israel	2
Saudi Arabia	2
Singapore	2
United States	2
Ciskei	1
Italy	1
South Korea	1
Nigeria	1
Pakistan	1
Poland	1
Portugal	1
Spain	1
Sri Lanka	1
Turkey	1
West Indies	1

Table 3 Key differences between congenital peritoneal encapsulation and fibrotic peritoneal encapsulation

	Congenital peritoneal encapsulation	Fibrotic peritoneal encapsulation
Aetiology
Cause	Congenital	Acquired
Trigger		Primary/Idiopathic (abdominal cocoon) or secondary (encapsulating peritoneal sclerosis)
Epidemiology
Incidence	45 cases	Idiopathic: 184 cases Secondary: Based on cause
Age (yr, range)	40.8 (11-85)	34.7 (7-87)
Sex (M:F)	5:3	2:1
Geographical Geography	Europe, Sub-continental Asia	Equatorial regions
Pathology
Morphology	Identical to peritoneum. Thin, semi-transparent, vascularised, soft.	Similar to scar tissue. Thick, white, firm, fibrotic.
Histopathology	Identical to peritoneum. Mesothelial lining, fibro-connective tissue.	Dense fibro-connective tissue proliferation, chronic inflammatory cell infiltration and dilated lymphatics
Manage-ment
Treatment	Peritonectomy, adhesiolysis	Corticosteroids, tamoxifen, peritonectomy
Prognosis	Excellent. Near complete resolution of symptoms.	Up to 50% mortality at 1 yr following diagnosis.