Congenital peritoneal encapsulation: A review and novel classification system

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.