Chronic intestinal pseudo-obstruction: systematic histopathological approach can clinch vital clues

Abstract

The histopathological approach of chronic intestinal pseudo-obstruction (CIP) is critical, and the findings are often missed by the histopathologists for lack of awareness and nonavailability of standard criteria. We aimed to describe a detailed histopathological approach for working-up cases of CIP by citing our experience. Eight suspected cases of CIP were included in the study to determine and describe an approach for reaching the histopathological diagnosis collected over a period of the last 1.5 years. The Hirschsprung's disease was put apart from the scope of this study. A detailed light microscopic analysis was performed along with special and immunohistochemical stains. Transmission electron microscopy was carried out on tissue retrieved from paraffin embedded tissue blocks. Among the eight cases, three were neonates, one in the pediatric age group, two adolescent, and two adults. After following the described critical approach, we achieved the histological diagnoses in all the cases. The causes of CIP noted were primary intestinal neuronal dysplasia (IND) type B (in 4), mesenchymopathy (in 2), lymphocytic myenteric ganglionitis (in 1), and duplication of myenteric plexus with leiomyopathy (in 1). Desmosis was noted in all of them along with other primary pathologies. One of the IND patients also had visceral myopathy, type IV. Histopathologists need to follow a systematic approach comprising of diligent histological examination and use of immunohistochemistry, immunocytochemistry, and electron microscopy in CIP workup. Therapy and prognosis vary depending on lesions identified by pathologists. These lesions can be seen in isolation or in combinations.

Paediatric Intestinal Pseudo‐obstruction

“…those defined as "diagnostic of a well-characterised disease with established cause and/or natural history" (199). This observation is stronger for the paediatric populations (200), and especially in congenital onset cases where other confounders are usually absent (199).…”

“…counselling) (4,157,200,204,207,208)or direct other investigations toward secondary aetiology e.g. in mitochondrial disorders (203)and ganglioneuromatosis (209).…”

Acute and chronic pseudo‐obstruction: a current update

“…Combined forms have been described from analysing tissue specimens (i.e. neuro‐myopathy, neuro‐mesenchymopathy) . All three forms may be seen in the tissue, and may act synergistically.…”

“…neuro-myopathy, neuromesenchymopathy). 10,12,21 All three forms may be seen in the tissue, and may act synergistically.…”

Role of DNA Methylation in the Development and Differentiation of Intestinal Epithelial Cells and Smooth Muscle Cells

“…Aberrant growth patterns of SMC in the GI tract is associated with burdensome GI diseases such as megacystis-microcolon-intestinal hypoperistalsis syndrome [38][39][40] and intestinal pseudo-obstructions. 41,42 The combination of plasticity and the potential for the dysregulation of growth/differentiation patterns make SMC a strong candidate for phenotypic alteration through epigenetic mechanism manipulation. In this vein, several research teams have been able to manipulate the phenotypic status of SMC through alteration of DNA methylation mechanisms and enzymes, [43][44][45][46][47][48] similar to previously mentioned research into DNA methylation dynamics in intestinal epithelium.…”