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Acute colonic pseudoobstruction (ACPO) is a medical emergency defined as acute colonic distention without mechanical obstruction or inflammatory status. The initial treatment for ACPO includes conservative therapy and gastrointestinal tube decompression. Colonic endoscopic decompression is indicated in patients who fail to respond to conservative treatment. If none of the above treatments are effective, surgeries such as cecostomy must be considered. We report the case of a 77-year-old man with a history of Parkinson's disease who was receiving anti-Parkinson agent therapy. The patient presented with abdominal distention for 1 week, and ACPO was diagnosed. The patient received conservative treatment after admission; however, the symptoms and imaging findings did not improve. Endoscopic decompression was unsuccessful. Subsequently, we successfully treated the patient with a novel technique by placing a decompression tube into the proximal colon under the guidance of a colonoscope.

The gastrointestinal (GI) tract can be involved in up to 90% of patients with systemic sclerosis (SSc) and is the leading cause of morbidity and third most common cause of mortality in these patients. The GI involvement can occur in the absence of cutaneous manifestations in 10% of patients. Vasculopathy, cellular and humoral immunity, and diffuse fibrosis are the principal pathogenetic mechanisms in SSc and begin with autoantibody-mediated neuronal damage followed by muscular damage and fibrosis. This leads to progressive dysmotility of the entire GI tract from mouth to anus and is responsible for the clinical manifestations including gastroesophageal reflux disease and dysphagia due to esophageal involvement, gastroparesis, small intestinal bacterial overgrowth and chronic intestinal pseudo-obstruction, and constipation due to colonic and fecal incontinence due to anorectal involvement. The clinical features resulting from the involvement of these organs often overlap and multiple areas may be involved simultaneously. The treatment remains mostly symptomatic because effective disease-modifying therapies are lacking. These patients are at a risk of malnutrition and nutritional screening, and thus rehabilitation is very important. Refractory cases require nutritional support in the form of enteral nutrition and/or home parenteral nutrition. Future research is needed in the pathogenesis, development of biomarkers for early identification of GI involvement at the asymptomatic stage, and targeted disease-modifying therapies, which can alter/halt the disease progression.

The gastrointestinal (GI) tract can be involved in up to 90% of patients with systemic sclerosis (SSc) and is the leading cause of morbidity and third most common cause of mortality in these patients. The GI involvement can occur in the absence of cutaneous manifestations in 10% of patients. Vasculopathy, cellular and humoral immunity, and diffuse fibrosis are the principal pathogenetic mechanisms in SSc and begin with autoantibody-mediated neuronal damage followed by muscular damage and fibrosis. This leads to progressive dysmotility of the entire GI tract from mouth to anus and is responsible for the clinical manifestations including gastroesophageal reflux disease and dysphagia due to esophageal involvement, gastroparesis, small intestinal bacterial overgrowth and chronic intestinal pseudo-obstruction, and constipation due to colonic and fecal incontinence due to anorectal involvement. The clinical features resulting from the involvement of these organs often overlap and multiple areas may be involved simultaneously. The treatment remains mostly symptomatic because effective disease-modifying therapies are lacking. These patients are at a risk of malnutrition and nutritional screening, and thus rehabilitation is very important. Refractory cases require nutritional support in the form of enteral nutrition and/or home parenteral nutrition. Future research is needed in the pathogenesis, development of biomarkers for early identification of GI involvement at the asymptomatic stage, and targeted disease-modifying therapies, which can alter/halt the disease progression. [ABSTRACT FROM AUTHOR]

The enteric nervous system (ENS) provides the intrinsic innervation of the bowel and is the most neurochemically diverse branch of the peripheral nervous system, consisting of two layers of ganglia and fibers encircling the gastrointestinal tract. The ENS is vital for life and is capable of autonomous regulation of motility and secretion. Developmental studies in model organisms and genetic studies of the most common congenital disease of the ENS, Hirschsprung disease, have provided a detailed understanding of ENS development. The ENS originates in the neural crest, mostly from the vagal levels of the neuraxis, which invades, proliferates, and migrates within the intestinal wall until the entire bowel is colonized with enteric neural crest-derived cells (ENCDCs). After initial migration, the ENS develops further by responding to guidance factors and morphogens that pattern the bowel concentrically, differentiating into glia and neuronal subtypes and wiring together to form a functional nervous system. Molecules controlling this process, including glial cell line-derived neurotrophic factor and its receptor RET, endothelin (ET)-3 and its receptor endothelin receptor type B, and transcription factors such as SOX10 and PHOX2B, are required for ENS development in humans. Important areas of active investigation include mechanisms that guide ENCDC migration, the role and signals downstream of endothelin receptor type B, and control of differentiation, neurochemical coding, and axonal targeting. Recent work also focuses on disease treatment by exploring the natural role of ENS stem cells and investigating potential therapeutic uses. Disease prevention may also be possible by modifying the fetal microenvironment to reduce the penetrance of Hirschsprung diseasecausing mutations. [ABSTRACT FROM AUTHOR]

Pseudoobstruction is an uncommon manifestation of hypothyroidism. However, hypotony of the bladder in myxedema is very rare, and there were only four such cases reported previously. We present a 45-year-old man with myxedema and ileus, who was started on thyroxine therapy and was detected to have bladder hypotony on the 5th day of admission. There was no evidence of bladder outflow obstruction, and both bowel and urinary symptoms improved with thyroxine therapy. Bladder hypotony may be more prevalent in patients of hypothyroidism than previously reported; hence, it is important to have a high clinical suspicion and perform early urodynamic studies to avoid back-pressure damage to the upper tracts. [ABSTRACT FROM AUTHOR]

Mitochondrial neurogastrointestinal encephalomyopathy is a rare disorder affecting the pediatric age group with a heterogenous multisystem involvement. We happen to manage a young child with symptoms of constipation since infancy alongwith cachexia, seizures and peripheral neuropathy. The child later went into encephalopathy preterminally. This clinical syndrome fitted very well with mitochondrial neurogastrointestinal encephalomyopathy. The child had elevated lactate levels and electron microscopy of the rectal biopsy was suggestive of a mitochondrial disorder To the best of our knowledge there is no case report of this syndrome from India and since this presents with diagnostic difficulties so is being reported. [ABSTRACT FROM AUTHOR]