This is a rare clinical syndrome characterized by symptoms of intestinal obstruction but lacking a mechanical obstruction. Current theories focus on an abnormal function of the gut motility. It may be associated with several disease states. Treatments have ranged from both medical to surgical removal of the affected portion of the colon.
Disease Associations Diabetes mellitus
Pathogenesis Interstitial Cells
Smooth muscle abnormalities
Laboratory/Radiologic/Other Diagnostic Testing Manometry Gross Appearance and Clinical Variants Prognosis Treatment Surgery
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DISEASE ASSOCIATIONS CHARACTERIZATION DIABETES MELLITUS SCLERODERMA SYSTEMIC LUPUS ERYTHEMATOSUS
Chronic intestinal pseudo-obstruction in systemic lupus erythematosus due to intestinal smooth muscle myopathy.
Hill PA, Dwyer KM, Power DA.
Department of Anatomical Pathology, St. Vincent's Hospital, Fitzroy, Victoria, Australia.
Lupus 2000;9(6):458-63 Abstract quote
We report the case of a woman with systemic lupus erythematosus initially manifesting with fever, rash and arthritis, and two years later with Class IV lupus nephritis. Following treatment with cyclophosphamide she developed symptoms and signs of chronic intestinal pseudo-obstruction (CIPO) that was initially thought to be due to a neutropenic enterocolitis. However, persistence of symptoms resulted in segmental resection of the ileum which showed widespread myocyte necrosis and active inflammation within the muscularis propria.
A subsequent, more extensive ileocolic resection showed severe diffuse atrophy and fibrosis of the muscularis propria throughout the resected bowel.
The absence of mesenteric vasculitis and the clinical response of the CIPO to the immunosupressive regimen of prednisolone and cyclosporin A suggest that the bowel muscle coat changes reflect an intestinal myopathy secondary to systemic lupus erythematosus, and may have an auto-immune etiology.
PATHOGENESIS CHARACTERIZATION INTERSTITIAL CELLS
Loss of CD117 (c-kit)- and CD34-Positive ICC and Associated CD34-Positive Fibroblasts Defines a Subpopulation of Chronic Intestinal Pseudo-obstruction.
Streutker CJ, Huizinga JD, Campbell F, Ho J, Riddell RH.
Am J Surg Pathol 2003 Feb;27(2):228-35 Abstract quote
Chronic idiopathic intestinal pseudo-obstruction is a syndrome in which symptoms of intestinal obstruction are present in the absence of mechanical obstruction. Lack of normal pacemaker activity, usually generated by the interstitial cells of Cajal (ICC), could account for the apparent obstruction.
ICC are normally located around and between the myenteric plexus ganglia and within muscle and also in the deep muscular plexus of the small bowel and the submuscular plexus of the large intestine, just within the circular muscle. ICC can be demonstrated immunohistochemically with CD117 (c-kit) as well as with CD34, although this is less specific. CD34 also stains a population of fibroblasts that are intimately associated with ICC.
To determine whether there is a relative deficiency of ICC and CD34-positive fibroblasts in patients with chronic idiopathic intestinal pseudo-obstruction, tissue from 30 patients of large intestine and eight patients with small intestine pseudo-obstruction was obtained. Controls (large intestinal specimens from 12 patients, small intestinal specimens from six patients) were chosen from resections for Crohn's disease and colorectal neoplasia, both with and without dilatation. Examination of pseudo-obstruction cases identified 10 patients (nine large intestinal and one small intestinal) in which both CD117 and CD34 were absent or severely reduced in all three of the examined areas. In contrast, the control cases, including those with preobstructive dilatation, showed relatively constant ICC staining.
These results suggest that there is a proportion of pseudo-obstruction cases in which the ICC are markedly reduced. These results also demonstrate that, in these cases, loss of the kit immunoreactivity is correlated with the loss of CD34 staining: this indicates that both the ICC and the CD34-positive fibroblasts associated with the ICC are absent. These findings will allow surgical pathologists to identify this subpopulation of patients with CIIP using tissue obtained by laparoscopic biopsy of the muscularis propria or surgical resection.
SMOOTH MUSCLE ABNORMALITIES
Unusual variations of gastrointestinal smooth muscle abnormalities associated with chronic intestinal pseudo-obstruction.
Moore SW, Schneider JW, Kaschula RO.
University of Stellenbosch, Department of Paediatric Surgery, P.O. Box 19063, 7505 Tygerberg, South Africa.
Pediatr Surg Int 2002 Jan;18(1):13-20 Abstract quote
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.
SOX10 mutations in chronic intestinal pseudo-obstruction suggest a complex physiopathological mechanism.
Pingault V, Girard M, Bondurand N, Dorkins H, Van Maldergem L, Mowat D, Shimotake T, Verma I, Baumann C, Goossens M.
INSERM U468, Genetique Moleculaire et Physiopathologie, and Laboratoire de Biochimie, Hopital Henri Mondor, 94010 Creteil Cedex, France.
Hum Genet 2002 Aug;111(2):198-206 Abstract quote
The type IV Waardenburg syndrome (WS4), also referred to as Shah-Waardenburg syndrome or Waardenburg-Hirschsprung disease, is characterised by the association of Waardenburg features (WS, depigmentation and deafness) and the absence of enteric ganglia in the distal part of the intestine (Hirschsprung disease). Mutations in the EDN3, EDNRB, and SOX10 genes have been reported in this syndrome. Recently, a new SOX10 mutation was observed in a girl with a neural crest disorder without evidence of depigmentation, but with severe constipation due to a chronic intestinal pseudo-obstruction and persistence of enteric ganglia.
To refine the nosology of WS, we studied patients with typical WS4 (including Hirschsprung disease) or with WS and intestinal pseudo-obstruction. We found three SOX10 mutations, one EDNRB and one EDN3 mutations in patients presenting with the classical form of WS4, and two SOX10 mutations in patients displaying chronic intestinal pseudo-obstruction and WS features.
These results show that chronic intestinal pseudo-obstruction may be a manifestation associated with WS, and indicate that aganglionosis is not the only mechanism underlying the intestinal dysfunction of patients with SOX10 mutations.
CHARACTERIZATION RADIOLOGIC LABORATORY MARKERS MANOMETRY
Colonic manometry in children with chronic intestinal pseudo-obstruction.
Di Lorenzo C, Flores AF, Reddy SN, Snape WJ Jr, Bazzocchi G, Hyman PE.
Department of Pediatrics, UCLA Medical Center, Martin Luther King Jr General Hospital, Charles Drew University of Medicine and Science, Torrance 90502.
Gut 1993 Jun;34(6):803-7 Abstract quote
Pressure changes were evaluated in the transverse, descending, and rectosigmoid colon of 30 children with chronic intestinal pseudo-obstruction. Twenty two had severe lifelong constipation and eight had symptoms suggesting a motility disorder exclusively of the upper gastrointestinal tract.
Based on prior antroduodenal manometry, 24 children were diagnosed as having a neuropathic and six a myopathic form of intestinal pseudo-obstruction. On the day of study, endoscopy was used to place a manometry catheter into the transverse colon and intraluminal pressure was recorded for more than four hours. After a baseline recording, we gave a meal to assess the gastrocolonic response. Colonic contractions were noted in 24 children. The six children with no colonic contractions had a hollow visceral myopathy and constipation. In the children with colonic contractions, fasting motility did not differentiate children with and without constipation. After the meal, in all eight children without constipation there was (1) an increase in motility index (3.2 (SEM 0.3) mm Hg/min basal v 8.4 (SEM 1.1) mm Hg/min postprandial; p < 0.001), and (2) at least one high amplitude propagated contraction (HAPC). In the 16 constipated children with colonic contractions the motility index did not significantly increase after the meal (2.1 (SEM 0.3) mm Hg/min basal v 3.1 (SEM 0.4) mm Hg/min postprandial) and 12 of them had no HAPCs (p < 0.01 v group without constipation).
In summary, in children with a clinical diagnosis of chronic intestinal pseudo-obstruction, constipation is associated with absence of HAPCs, and the gastrocolonic response or with total absence of colonic contractions.
CHARACTERIZATION GENERAL VARIANTS GENITOURINARY
Urological manifestations associated with chronic intestinal pseudo-obstructions in children.
Lapointe SP, Rivet C, Goulet O, Fekete CN, Lortat-Jacob S.
Hospital of St. Justine, Montreal, Canada.
J Urol 2002 Oct;168(4 Pt 2):1768-70 Abstract quote
PURPOSE: Chronic intestinal pseudo-obstruction syndrome is a rare pathological condition characterized by chronic intestinal occlusion without any anatomical or mechanical lesion. Recent progress in the treatment of these children has led to improved survival and development of associated urological complications. We define urological manifestations of chronic intestinal pseudo-obstruction to optimize evaluation and treatment.
MATERIALS AND METHODS: We evaluated retrospectively the charts of 20 girls and 19 boys with a mean age of 11.2 years (range 3 days to 26 years) diagnosed with chronic intestinal pseudo-obstruction from January 1975 to February 2000. Urological evaluation included prenatal and postpartum ultrasonography, serum renal function assessment, voiding cystourethrography and urodynamics.
RESULTS: Urological involvement was present in 27 of the 39 children (69%). Megacystis in 26 children (96.3%) was isolated in 12 (46.2%) and associated with ureterohydronephrosis in 14 (53.8%), including 4 with vesicoureteral reflux. One patient presented with a complex urinary anomaly. Prenatal diagnosis of megacystis was made in 16 patients (59%), including 4 with neonatal urinary retention and 12 with urinary tract infection. At followup 24 patients had 1 or greater febrile urinary tract infections while 3 had afebrile infections. In the 14 patients with megacystis and ureterohydronephrosis, 3 or greater febrile urinary tract infections developed while 10 patients with isolated megacystis had only 1 febrile urinary tract infection. Twelve patients underwent 17 urodynamic studies, all of whom initially presented with hypocontractile detrusor, increased age corrected capacity (194% +/- 44%) and compliance (42 ml./cm. H O, range 13 to 214). Bladder proprioception was also diminished. Presently voiding is achieved by abdominal pressure in 14 cases and intermittent catheterization in 10. Antibioprophylaxis was given to 17 patients, at one point in time. Renal failure (1 iatrogenic, 1 complex anomaly) developed in 2 patients.
CONCLUSIONS: The presence of megacystis on prenatal ultrasound should alert the physician to the possibility of chronic intestinal pseudo-obstruction. Bladder adynamia represents the dominant urological complication. Urological evaluation and management are required to ensure adequate bladder emptying to reduce the risk of urinary tract infection and preserve long-term renal function.
PROGNOSIS CHARACTERIZATION GENERAL
Quality of life outcomes in congenital chronic intestinal pseudo-obstruction.
Schwankovsky L, Mousa H, Rowhani A, DI Lorenzo C, Hyman PE.
University of Ohio, Columbus, USA.
Dig Dis Sci 2002 Sep;47(9):1965-8 Abstract quote
The goal of this study was to assess the quality of life for children with chronic intestinal pseudoobstruction.
We used a retrospective chart review to identify children with congenital chronic intestinal pseudoobstruction, then a structured telephone interview with parents that included the Child Health Questionnaire to gather information about the current status and quality of life for each patient and family. Children with chronic intestinal pseudo-obstruction had less freedom from pain, depression, and anxiety than healthy children or children with juvenile rheumatoid arthritis (P < 0.05 for all three parameters). Parents of children with chronic intestinal pseudo-obstruction had poorer emotional status than parents of healthy children or children with juvenile rheumatoid arthritis. The time required for parents to care for children with chronic intestinal pseudo-obstruction was greater than the time required to care for healthy children or children with juvenile rheumatoid arthritis (P < 0.01).
In conclusion, the quality of life for children with chronic intestinal pseudo-obstruction lags behind that of healthy children and children with another chronic illness. Appropriate treatment of chronic pain may improve the quality of life for children with chronic intestinal pseudo-obstruction and their families. Moreover, attention to reducing each family's burden of time and emotional distress may help them cope better with their chronically ill child.
TREATMENT CHARACTERIZATION GENERAL SURGERY
Surgical treatment of chronic intestinal pseudo-obstruction: report of three cases.
Shibata C, Naito H, Funayama Y, Fukushima K, Hashimoto A, Kitayama T, Nagao M, Matsuno S, Sasaki I.
Division of Biological Regulation and Oncology, Department of Surgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai 980-8574, Japan.
Surg Today 2003;33(1):58-61 Abstract quote
We report the cases of three patients with chronic intestinal pseudo-obstruction (CIP) whose quality of life (QOL) was improved by palliative surgery in combination with home parenteral nutrition (HPN). Loop enterostomy and shortening of the gastrointestinal tract was performed with good results in all three patients to relieve abdominal distension that was progressive despite treatment with prokinetic drugs.
Oral intake was also improved after surgical treatment in one patient who underwent massive resection of the jejunoileum. HPN maintained a satisfactory nutritional state in all patients.
These three case reports serve to demonstrate that surgical intervention to improve QOL should be considered for patients with CIP if medication is not effective and symptoms are progressive.
Long-term results of intestinal transplantation for pseudo-obstruction in children.
Iyer K, Kaufman S, Sudan D, Horslen S, Shaw B, Fox I, Langnas A.
Organ Transplantation Program, University of Nebraska Medical Center, Omaha, NE 68198-3285, USA.
J Pediatr Surg 2001 Jan;36(1):174-7 Abstract quote
PURPOSE: The aim of this study was to determine long-term results of intestinal transplantation in children with pseudo-obstruction, particularly when stomach and colon are not part of the allograft.
METHODS: The authors conducted a case-record review of all children who underwent transplantation at our center for a primary diagnosis of pseudo-obstruction. Supplementary information was obtained from outpatient charts, computerized database, and telephone survey of parents.
RESULTS: Six small bowel and 3 liver-small bowel transplants were carried out in 8 patients between 1993 and 1999. Median follow-up is 40 months (range, 13 to 73 months). Median age at transplantation was 2.7 years (range, 0.7 to 12.8 years). Median graft survival in this series is 15 months (range, 1 day to 71 months). Stomach and colon were excluded from all allografts. Two children died 5 and 368 days after transplant and 2 graft losses occurred in 1 patient. Two children had lymphoproliferative disease; both are alive with functioning grafts. Five survivors with functioning grafts receive full enteral feedings at home. Four of the 5 have had ileostomies closed, and 3 have normal bowel movements.
CONCLUSIONS: Intestinal transplantation without stomach or colon provides children with chronic intestinal pseudo-obstruction with a good quality of life. The underlying disease poses special challenges in management.
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