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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.

Introduction: Small bowel diverticula is a rare condition and is rarely seen in the jejunum compared to the ileum. It is usually incidentally detected. It is mostly asymptomatic. Herein, we aimed to discuss a case of multiple jejunal diverticula that have caused pseudoobstruction in the light of the literature. Case report: A 55-year-old male patient had complaints of abdominal pain, abdominal swelling, nausea and vomiting. Mechanic intestinal obstruction was considered. Multiple giant diverticula were found in entire jejunum. Conclusion: Jejunal diverticula may produce a different clinical picture. It should be kept in mind that jejunal diverticula that is a rare condition may cause pseudoobstruction. [ABSTRACT FROM AUTHOR]

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]

Résumé L’atteinte intestinale est fréquente au cours des connectivites et des vascularites. Parmi les connectivites, l’atteinte intestinale est particulièrement prévalente dans la sclérodermie systémique (ScS), où il existe un tableau de pseudo-obstruction intestinale pouvant se compliquer de pullulation microbienne du grêle et de syndrome de malabsorption. Cette atteinte intestinale est potentiellement sévère, pouvant mener à une dénutrition sévère et une cachexie. Son traitement est difficile, avec le recours possible à des injections sous-cutanées d’octréotide, et doit associer celui d’une pullulation microbienne du grêle, basé sur une antibiothérapie séquentielle. L’atteinte intestinale des vascularites est souvent considérée comme un facteur de gravité. Certaines manifestations sont communes à l’ensemble des vascularites, comme l’ischémie, l’infarctus et les perforations digestives, ainsi que les hémorragies par ulcérations muqueuses ou les ruptures anévrismales. Certains tableaux sont plus spécifiques, et sont détaillés dans cette revue. Les manifestations cliniques sont souvent peu spécifiques et les explorations digestives n’apportent généralement pas d’élément pronostique. Le traitement de ces patients repose sur des immunosuppresseurs puissants, avec parfois nécessité de recours à la chirurgie en urgence en cas d’abdomen chirurgical. Intestinal involvement is frequent in connective tissue disorders and vasculitides. Among the connective tissue diseases, intestinal involvement is particularly prevalent in systemic sclerosis (SSc). SSc is characterized by intestinal pseudo-obstruction, which may be complicated by small intestinal bacterial overgrowth (SIBO) syndrome and malabsorption. These intestinal syndromes are potentially severe, as they can lead to severe malnutrition and cachexia. Treatment is often difficult; it might require subcutaneous octreotide injections and must associate the treatment of SIBO, based on cycled antibiotics. Intestinal involvement is often regarded as a severe complication of vasculitides. Some clinical manifestations are common to all vasculitides, including ischemia, bowel infarction and perforations, as well as hemorrhage due to mucosal ulcerations or aneurismal ruptures. Some manifestations are more specific and are detailed in this review. Clinical manifestations are often nonspecific and digestive explorations usually have no prognostic value. Treatment of these patients is based on potent immunosuppressive agents, and sometimes requires prompt surgery in case of surgical abdomen. [ABSTRACT FROM AUTHOR]

Background: Gastrointestinal complications after lung transplantation have been reported with incidence rates ranging from 3% to 51%, but the reasons are poorly understood. We aimed to investigate the correlations between pulmonary diseases leading to lung transplantation and early gastrointestinal complications requiring laparotomy after transplantation with outcomes for patients at increased risk. Methods: In this study we performed a retrospective analysis of data of patients who underwent lung transplantation at our institution from 2004 to 2012. The study period was limited to the first 90 days after transplantation. Results: Lung transplantation was performed in 258 patients, including 51 patients with α1-anti-trypsin deficiency (A1AD). Seventy-eight patients (30%) had an X-ray of the abdomen, and 23 patients (9%) required laparotomy during the first 90 days after transplantation. Patients with A1AD comprised 20% of the total recipients, 23% (18 of 78) of the patients who had an abdominal X-ray performed (p = 0.40), and 48% (11 of 23) of the patients who required laparotomy (p < 0.001). More than 1 of every 5 patients (11 of 51) with A1AD required laparotomy at a median 8 days after transplantation, and the estimated odds ratio for laparotomy for A1AD patients was 5.74 (CI 2.15 to 15.35). In the group of patients with A1AD who required laparotomy, the estimated hazard ratio for death was 1.62 (CI 0.57 to 4.62), the stay in the intensive care unit was prolonged, but no significant difference was observed for time on mechanical ventilation. Among pulmonary diseases and demographics of the patients, no other risk factors were identified for laparotomy. Conclusions: A1AD was the only significant risk factor identified for gastrointestinal complications that required laparotomy within 3 months after lung transplantation. There was a trend toward a higher risk of death after laparotomy in patients with A1AD, and the length of stay in the intensive care unit was significantly prolonged, whereas the time on mechanical ventilation was unaffected. [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]

Abstract: Purpose: Erythromycin is successfully used as a gastroduodenal prokinetic agent. Given the limited available treatments for colonic dysmotility, further investigation into erythromycin''s effect on colonic motility is warranted. We aimed to study the effect of erythromycin on colonic motility in pediatric patients with recalcitrant chronic constipation/encopresis and other suspected colonic motility disorders. Methods: Patients referred for colonic manometry were eligible for enrollment. Fasting motility was recorded for 1 to 2 hours, then erythromycin lactobionate (EL), 3 mg/kg, was administered intravenously, and colonic motility was monitored for 1 to 2 hours after erythromycin. Manometry was then continued per routine. The motility index (MI) of pressure tracings at each pressure transducer was calculated for each patient for a period of 15 and 60 minutes before and after EL infusion. Change in MI was compared by Wilcoxon signed rank test. Results: Twenty patients were enrolled. The most common indication was constipation with encopresis. Seventy percent of patients had normal colonic manometry, and 30% of patients demonstrated a neuropathy. Average MI for the 60-minute period before and after EL infusion were 254 ± 74 mm Hg/h and 253 ± 94 mm Hg/h, respectively (P = .55). Average MI for the 15-minute period before and after EL infusion were 64 ± 23 mm Hg/15 min and 69 ± 32 mm Hg/15 min, respectively (P = .45). Conclusions: Administration of intravenous EL resulted in no changes in colonic MI in pediatric patients referred for colonic manometry. Further studies on potential colokinetic agents are warranted in this population of patients. [Copyright &y& Elsevier]

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]

Summary: Chronic intestinal pseudoobstruction due to visceral myopathy may cause intestinal failure. The anatomical pathology of visceral myopathy is dominated by an atrophic muscularis propria. We present a case that is overwhelmingly dominated by a hypertrophic muscularis propria. The hypertrophic pattern was documented from ages 2 to 28 years. Most descriptions of visceral myopathy focus on the atrophic pattern of injury to the muscularis propria, typified by vacuolar degeneration of myocytes, loss of muscle fibers, and frequently with a honeycomb pattern of fibrosis. Hypertrophic changes can be observed in visceral myopathy, but the hypertrophy typically represents only a minor feature, typically limited to the internal layer of the muscularis propria. In our patient, the observed muscular hypertrophy differed from most descriptions because it (a) was the dominant pattern of injury and (b) involved both layers of muscularis propria rather than just the inner circular layer. The patient had intestinal failure and the complications induced cirrhosis, eventually treated by multivisceral transplantation to correct the intestinal failure and cirrhosis. [Copyright &y& Elsevier]

This section provides abstracts of poster sessions presented at the 21st International Symposium on Neurogastroenterology and Motility held in Jeju, South Korea in September 2007, including sessions on the sensitivity of guinea-pig small intestinal spinal afferents, telemetric recording of electrogastrogram, and changes in serum gastrin levels.

Degenerative leiomyopathy (DL) is a distinctive form of acquired degenerative visceral myopathy of uncertain etiology that occurs largely in Africa and results in intestinal pseudo-obstruction (IP). In this review of 39 patients from the Western Cape region of South Africa, the mean age at presentation was 9.5 years (range 6 months to 16 years). Characteristic clinical features included a chronic, insidious history of repeated attacks of abdominal distension, abdominal pain, and vomiting. Marked gaseous distension with atony and IP, especially of the colon, was noted on X-ray films. Megacolon was the most common radiologic feature, but pseudo-obstruction extended proximally into the small intestine in some patients with advanced disease. In the majority of cases the condition was progressive and eventually affected the entire gastrointestinal (GI) tract. [ABSTRACT FROM AUTHOR]

The clinicopathological spectrum of gastrointestinal (GI) smooth-muscle abnormalities associated with chronic intestinal pseudo-obstruction (CIPO) includes numerous heterogeneous conditions that are often ill-defined and poorly understood. Primary GI smooth-muscle abnormalities include familial and sporadic forms. Secondary involvement of GI smooth-muscle may result from associated GI and systemic conditions, but is less frequent than in adults. This study documents the clinicopathological findings observed in 12 South African patients with unusual forms of visceral smooth-muscle abnormalities not conforming to the diagnostic criteria of known primary visceral myopathies at the Tygerberg and Red Cross Childrens' Hospitals over a 14-year period (July 1985 through January 1999). Congenital muscle defects occurred in 5 patients where layers of bowel-wall muscle were absent or attenuated. Idiopathic fibrosis and ultrastructural features of perinuclear and mitochondrial vacuolisation were noted in 2 patients. A 21-year-old female with long-standing pseudo-obstruction demonstrated diminished immunohistochemical expression of enteric alpha-smooth-muscle actin without associated muscular degeneration or fibrosis. A secondary complication of dermatomyositis (bowel perforation) occurred twice in 1 patient. In 3 further patients (1 each with anorectal malformation, long-segment Hirschsprung's disease, and intestinal neuronal dysplasia), muscle fibrosis appeared during progression of the pre-existing disease. Visceral myopathies are poorly understood conditions that may present with CIPO. Unusual variations occur that do not conform to the usual recognised histological patterns. Secondary involvement may also be more common than anticipated in children. The challenge to further understanding these uncommon conditions lies in timely diagnosis and identification of early, subtle signs. Optimal and extensive application of various diagnostic modalities, including the development of new diagnostic tools, is of considerable importance in identifying hitherto unexplained CIPO due to GI smooth-muscle abnormalities. [ABSTRACT FROM AUTHOR]

Gastrointestinal manometry has gained wide acceptance in the approach to patients with suspected enteric neuromuscular disorders. However, performing gastrointestinal manometry in these subjects without a previous exhaustive diagnostic evaluation is unjustified. Twelve children (median age: 7.0 years; range: 8 months–13 years), with clinical and x-ray features suggesting chronic intestinal pseudoobstruction, were referred to our unit for gastrointestinal manometry. The latter was performed with a perfused catheter for 5 hr in the fasting state and for 90 min after feeding. Data were compared with those recorded in eight age-matched controls. In all patients and controls, interdigestive motor complexes with propagated phases III were detected; a regular postprandial antroduodenal motor activity was also recorded. Patients and controls did not differ for fed antral and duodenal motility indexes, fed antroduodenal coordination, and length of duodenal phase III. Most of the patients showed short or prolonged bursts of nonpropagated activity in the fasting and/or fed states; in four cases fasting and/or fed sustained phasic activity was recorded. Manometric evidence of migrating motor complexes and postfeeding activity did not support the diagnosis of intestinal pseudoobstruction and suggested redirecting the diagnostic evaluation. Final diagnoses were: Munchausen syndrome-by-proxy (four cases), celiac disease (two cases), intestinal malrotation (two cases), Crohn’s disease (two cases), multiple food intolerance (one case), and congenital chloride-losing diarrhea (one case). It is concluded that in children with suspected chronic intestinal pseudoobstruction manometric evidence of migrating motor complexes and fed motor activity excludes an enteric neuromuscular disorder and suggests a reassessment of the diagnostic work-up. Furthermore, if gastrointestinal manometry shows migrating motor complexes and postfeeding motor activity, qualitative abnormalities of the manometric tracings do not indicate an underlying enteric neuromuscular disorder and must not be overemphasized. Patients referred for gastrointestinal manometry should previously undergo an extensive diagnostic investigation to exclude disorders mimicking chronic intestinal pseudoobstruction. [ABSTRACT FROM AUTHOR]

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We performed simultaneous fasting and fedantroduodenal manometry and EGG in 25 children withfunctional bowel disorders. Three patients (12%) had anuninterpretable EGG. The manometric studies showedsevere neuropathy in six patients; milder neuropathicchanges in five patients; postprandial hypomotility inone patient; myopathy in four patients, and normalmotility in the remaining six patients. The percentage of tachygastria time (frequency >3.5cycles/min) was higher in the patients with mild (44.1± 15.8%) and severe (48 ± 19.1%)neuropathy than in the patients with myopathy (20± 16.2%, P < 0.05) or with normal motility (23 ± 13.3%, P< 0.05). There was a considerable overlap in thepercentage of tachygastria and total arrhythmia timeamong the different study groups. The ratio of post- to preprandial power was significantly higher (2.5± 0.07) in children with normal motility than inthe other patients groups. Every child with totalarrhythmia time <35% and a ratio of post-topreprandial power >2.4 had normal manometry. In summary,EGG differentiated groups of children with normalmanometry from others with neuropathic or myopathicchanges, but in a minority of patients the study was not interpretable and there was overlap in EGGresults between children with normal and abnormalmanometry. [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]

Celiac Disease (CD) is gluten-sensitive, autoimmune chronic enteropathy. Diarrhea, abdominal distention, vomiting and failure to thrive are the classical symptoms of celiac disease. "Atypical" celiac disease can present with uncommon gastrointestinal findings, such as abdominal pain, constipation and non gastrointestinal manifestations. Here we report child with celiac disease presenting with recurrent small bowel intussusception. Delayed short bowel transit time and reduced motility could be responsible for clinical symptoms, which resolved after gluten free diet. Celiac disease should be kept in mind in children with symptoms of small bowel intususception or pseudoobstruction without underlying cause. [ABSTRACT FROM AUTHOR]

Abstract: Ogilvie''s syndrome is an uncommon disorder of acute colonic pseudoobstruction that is often associated with concomitant medical disease or psychiatric medication. Therapeutic interventions include cholinesterase inhibitors, colonic decompression, and, in severe cases, surgery. We report a case of functional obstruction that was resolved after spinal anesthesia. The effect of spinal anesthesia on the autonomic control of colonic motility is discussed, and the literature on neuraxial blockade and Ogilvie''s syndrome is reviewed. [Copyright &y& Elsevier]

Abstract: A pair of 2 serial abdominal images show Ogilvie syndrome in a patient after cardiac surgery with the subtle but classic x-ray transition from a nonperforated to a perforated colon. [Copyright &y& Elsevier]