Abnormal layering of muscularis propria as a cause of chronic intestinal pseudo-obstruction: A case report and literature review

Table 1 Previously reported cases of visceral myopathy due to abnormal layering of muscularis propria

Case	Sex/age	Presenting symptom	Site	Muscularis propria pathology		Other GI abnormality	Other findings	Remark	Ref.
				Additional layer	Location
1	M/11 d	CIPO	S	Oblique	External to OL	Short small intestine + malrotation	Sclerocornea, cryptorchidism, scoliosis, deformity of extremities		[19]
2	M	CIPO	S and L	Circular	Between IC and OL	Short intestine + malrotation	Megacystis	X-linked	[9]
3	M	CIPO	S and L	Circular	Between IC and OL	Short intestine + malrotation	Megacystis	X-linked	[9]
4	M	CIPO	S and L	Circular	Between IC and OL	Short intestine + malrotation	Megacystis	X-linked	[9]
5	F	Constipation	L	Circular	Internal to IC	-	-		[9]
6	F	Constipation	L	Circular	Internal to IC	-	Megaureter		[9]
7	M/16 yr	Constipation	L	Circular	Internal to IC	-	Dysmorphic facies and toes, seizures, leukoencephalopathy, cataract	Clinically improved after surgery	[22]
8	M/birth	Respiratory problem	S	Circular	External to OL	Short small intestine + malrotation	Diaphragmatic defect, High-arched palate, ASD, ventriculomegaly, Subependymal heterotopias, arachnoid cyst, spina bifida, proximally placed thumbs	Dead	[22]
9	M/9 mo	CIPO	S	Oblique	Between IC and OL	Short small intestine	Brachydactyly, absent 2nd to 4th middle phalanges of both hands	Clinically improved by medication	Present case

CIPO: Chronic intestinal pseudo-obstruction; GI: Gastrointestinal; S: Small intestine; L: Large intestine; IC: Inner circular layer; OL: Outer longitudinal layer.