Guideline sources The following summarized guidelines for the evaluation and management of chronic intestinal pseudo-obstruction are prepared by our editorial team based on guidelines from the British Society of Gastroenterology (BSG 2020) and the European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN 2018). 1 2 Guidelines 1. Screening and diagnosis Diagnosis: make a definite diagnosis only when a detailed history, symptoms, and investigations (including histology in a unit with expertise in this area of pathology) confirm the diagnosis. Consider making an empirical working diagnosis of probable severe dysmotility if a definitive diagnosis is not possible. B Etiology: Recognize that enteric myopathies are often a primary condition and have multi-visceral involvement (especially of the urinary tract). Recognize that enteric myopathies may be https://web.pathway.md/diseases/recZYm6OFeqQ3Q6OM 1/6 6/29/23, 3:54 AM Chronic intestinal pseudo-obstruction Pathway secondary to a muscular disease (such as muscular dystrophy) and an awareness of this is needed when managing the primary illness. B Recognize that patients with an enteric neuropathy often have a serious underlying condition (often neurological or metabolic). B 2. Diagnostic investigations Initial evaluation: obtain workup judiciously, including radiology, radioisotope studies, manometry, autoimmune screens, and histology, in accordance with current guidelines. Take into account the presence of some drugs (such as opioids, cyclizine, or anticholinergic drugs), severe malnutrition, or an eating disorder when interpreting results. B Show 4 more 3. Medical management General principles: ensure multidisciplinary team management (likely to include a gastroenterologist, gastrointestinal physiologist, gastrointestinal surgeon, pain team, psychiatrist/psychologist, rheumatologist, urologist, gynecologist, radiologist, dietitian, specialist nurses, clinical biochemist, histopathologic, and pharmacist) in patients with chronic small intestinal dysmotility as these patients are complex with multiple issues contributing to their presentation. Encourage regional networking (can be via a virtual multidisciplinary team) both for support/guidance and to collect data. B Show 3 more 4. Nonpharmacologic interventions Nutritional support: offer oral supplements/dietary adjustments in patients with or at risk of malnutrition. B Show 4 more 5. Therapeutic procedures Venting gastrostomy: consider placing venting gastrostomy to reduce vomiting in patients with chronic small intestinal dysmotility recognizing associated problems (leakage, not draining, or poor body image). B 6. Specific circumstances Pediatric patients (definition): view chronic intestinal pseudo-obstruction occurring in infants and children as a separate entity from that in adults and refer to as pediatric intestinal pseudo- obstruction. B https://web.pathway.md/diseases/recZYm6OFeqQ3Q6OM 2/6 6/29/23, 3:54 AM Chronic intestinal pseudo-obstruction Pathway Show 2 more Pediatric patients (diagnosis): Suspect pediatric intestinal pseudo-obstruction in the following situations: Situation Guidance Presenting with symptoms of intestinal obstruction without an occluding lesion All children Prenatal diagnosis of megacystis/enlarged bladder Neonates Persistent or recurrent obstructive symptoms after exclusion of Hirschsprung disease and hypothyroidism Persistent vomiting after a Ladd's procedure for malrotation Symptoms of intestinal obstruction associated with bladder dysmotility Persistent or recurrent obstructive symptoms after exclusion of Hirschsprung disease Infants or children Persistent vomiting/intestinal obstruction after correction of malrotation Symptoms of intestinal obstruction associated with ptosis, deafness, or abnormal cardiac rhythm/function. B Pediatric patients (differential diagnosis): exclude Hirschsprung disease in patients with suspected pediatric intestinal pseudo-obstruction. B Pediatric patients (initial imaging): obtain routine abdominal XR as first-line imaging in all patients with suspected pediatric intestinal pseudo-obstruction to identify dilated small intestinal loops. B Show 2 more Pediatric patients (assessment of bowel transit): do not use radiopaque marker studies for the assessment of small bowel transit in the diagnostic work-up of pediatric intestinal pseudo- obstruction. D Show 5 more Pediatric patients (intestinal biopsy): perform full-thickness intestinal biopsy for histopathological analysis if therapeutic surgery (such as intestinal resection, ostomy formation, ostomy revision) is performed in patients with pediatric intestinal pseudo-obstruction. B Show 2 more Pediatric patients (laboratory tests): https://web.pathway.md/diseases/recZYm6OFeqQ3Q6OM 3/6 6/29/23, 3:54 AM Chronic intestinal pseudo-obstruction Pathway Obtain the following laboratory tests in the diagnostic evaluation of patients with suspected pediatric intestinal pseudo-obstruction: CBC electrolytes renal function tests LFTs albumin inflammatory indices (ESR and CRP) celiac serology fasting cortisol thyroid function tests basic metabolic panel (ammonia, lactate, urinary organic acids). B Decide on obtaining other laboratory tests, particularly aimed at identifying secondary causes of pediatric intestinal pseudo-obstruction, based on the presence of specific clinical signs and symptoms. Pay special attention to patients with 1 of the following: familial history of similar problems deafness cardiac involvement (cardiomyopathy, abnormal conduction or rhythm) neurological symptoms and neurological impairment in the absence of a specific cause dermatological involvement (scleroderma, Raynaud's phenomenon, palmar erythema, nodules) known or suspected mitochondrial disorders sudden onset of intestinal sub-occlusive episodes (idiopathic or paraneoplastic inflammatory neuro myopathy). B Pediatric patients (non-operative management): ensure a multidisciplinary team approach (including general pediatrician/adolescent medicine, gastroenterologist, surgeon, nutritionist, psychologist, urologist, geneticist/metabolic medicine specialist, and social worker) for the management of patients with pediatric intestinal pseudo-obstruction. B Show 2 more Pediatric patients (nutrition): optimize the nutrition status in patients with pediatric intestinal pseudo-obstruction using the guidance of a nutritionist as part of a hospital-based multidisciplinary team. Attempt to optimize oral and enteral nutrition without compromising intestinal function, even though the nutrition may comprise parenteral nutrition. B Pediatric patients (stoma creation): Consider creating a venting/feeding gastrostomy and/or jejunostomy in all patients with pediatric intestinal pseudo-obstruction. B Consider creating a decompressive ileostomy in all patients with pediatric intestinal pseudo- obstruction on parenteral nutrition. B https://web.pathway.md/diseases/recZYm6OFeqQ3Q6OM 4/6 6/29/23, 3:54 AM Chronic intestinal pseudo-obstruction Pathway Pediatric patients (surgical management): minimize the number of surgical interventions in patients with pediatric intestinal pseudo-obstruction in order to avoid potential complications (such as adhesion formation, prolonged paralytic ileus post-surgery) and diagnostic uncertainty regarding the pseudo obstructive or true occlusive nature of future obstructive episodes. B Show 2 more Pediatric patients (transition of care): Transfer patients with pediatric intestinal pseudo-obstruction to specialist adult gastroenterology centers with appropriate expertise in neurogastroenterology and motility as well as intestinal failure and parenteral nutrition (including home parenteral nutrition). B Initiate transition of care by the age of 15 years and initially involve both the pediatric and adult gastroenterology consultants as well as local gastrointestinal expertise. B Pediatric patients (national regulations): establish national referral centers for the diagnosis and management of pediatric intestinal pseudo-obstruction. B Show 2 more Clinical findings Symptoms Past medical history Abdominal cramps Alpers-huttenlocher syndrome Abdominal pain Carcinoid syndrome Bloating Chagas disease Constipation Dermatomyositis Diarrhea Diabetes mellitus Early satiety Ehlers-danlos syndrome Epigastric pain Exposure to chemotherapy Heartburn Fabry disease Loss of appetite Lung cancer Nausea Melas syndrome Vomiting Merrf syndrome Weight loss Multiple system atrophy Paraneoplastic syndrome Anthropomorphic exam Parkinson's disease Pelvic radiation therapy Malnutrition Prostate cancer Lab findings SIBO SLE Hypoalbuminemia Scleroderma Hypokalemia Thymoma Metabolic acidosis Vitamin B12 deficiency Waardenburg syndRome type 4 Imaging findings Distended bowel loops https://web.pathway.md/diseases/recZYm6OFeqQ3Q6OM 5/6 6/29/23, 3:54 AM Chronic intestinal pseudo-obstruction Pathway Abdominal exam Intestinal dysmotility Abdominal distension Abdominal tenderness Succussion splash Serological findings References 1. Jeremy M D Nightingale, Peter Paine, John McLaughlin et al. The management of adult patients with severe chronic small intestinal dysmotility. Gut. 2020 Dec;69 12 2074 2092. Open 2. Nikhil Thapar, Efstratios Saliakellis, Marc A Benninga et al. Paediatric Intestinal Pseudo-obstruction: Evidence and Consensus-based Recommendations From an ESPGHAN Led Expert Group. J Pediatr Gastroenterol Nutr. 2018 Jun;66 6 991 1019. Open https://web.pathway.md/diseases/recZYm6OFeqQ3Q6OM 6/6