RESUMO
BACKGROUND: In 2018 diagnostic criteria for pediatric intestinal pseudo-obstruction (PIPO) were established. Neuromuscular dysfunction of the gastrointestinal tract is one of these, and often examined through antroduodenal manometry (ADM). There is little data on antroduodenal manometries in children. Our objectives were to retrospectively apply these criteria to children evaluated for suspected motility disorder, to reevaluate the ADM patterns and compare children who did and did not meet the PIPO criteria and also with healthy adults. METHODS: Children with a suspected gastrointestinal motility disorder previously investigated with 24-h 8-lead ADM were reevaluated by applying the 2018 ESPGHAN/NASPGHAN PIPO diagnostic criteria and the 2018 ANMS-NASPGHAN guidelines. ADM findings were compared between children who retrospectively fulfilled a PIPO diagnosis, children who did not, and a control group of healthy adults. KEY RESULTS: Of 34 children (age 7.9 (±5.1) years, 18 males), 12 retrospectively fulfilled the 2018 PIPO diagnostic criteria. Twenty-five children (10 in the PIPO group) had abnormal diagnostic findings on ADM, whereas 9 (2 in the PIPO group) had no such findings. A PIPO diagnosis implied a significantly higher degree of abnormal ADM patterns (2.33 vs. 1.23, p = 0.02). There were no major differences in quantitative ADM measurements between the groups except higher pressures in children. CONCLUSIONS AND INFERENCES: Children who retrospectively fulfilled a PIPO diagnosis had a significantly higher abundance of abnormal ADM findings compared with symptomatic children without PIPO and healthy adults. Our data indicate a need for set criteria for evaluation of ADM in children with suspected PIPO.
Assuntos
Pseudo-Obstrução Intestinal , Manometria , Humanos , Manometria/métodos , Masculino , Criança , Feminino , Estudos Retrospectivos , Pré-Escolar , Pseudo-Obstrução Intestinal/diagnóstico , Pseudo-Obstrução Intestinal/fisiopatologia , Adolescente , Duodeno/fisiopatologiaRESUMO
PURPOSE: Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is a well described clinical condition, but reports are focused on microcolon and intestinal hypoperistalsis, while data on bladder management are scant. Aim of the study is to present urological concerns in MMIHS. METHODS: Retrospective evaluation of clinical data on urological management of MMIHS patients treated in the last 10 years. RESULTS: Six patients were enrolled (3 male, 3 female). Three girls had prenatal diagnosis of megacystis (1 vesicoamniotic shunt was placed). All patients had genetic diagnosis: 5 had ACTG2 gene mutations and 1 MYH11 mutation. All patients were addressed to our attention for urinary symptoms, such as urinary retention, urinary tract infections, acute renal injury. Two patients presented frequent stoma prolapses. All children underwent a complete urological evaluation, and then started a bladder management protocol (clean intermittent catheterization, via urethra or cystostomy-tube placement), with improvement of urinary infections, upper urinary tract dilation and stoma prolapses, if present. All patients had good renal function at last follow-up. CONCLUSION: We believe that MMIHS patients must be addressed soon and before onset of symptoms for a multidisciplinary evaluation, including an early assessment by a pediatric urologist expert in functional disorder, to preserve renal function at its best.
Assuntos
Anormalidades Múltiplas , Colo , Colo/anormalidades , Pseudo-Obstrução Intestinal , Bexiga Urinária , Bexiga Urinária/anormalidades , Humanos , Feminino , Estudos Retrospectivos , Masculino , Anormalidades Múltiplas/cirurgia , Colo/cirurgia , Bexiga Urinária/cirurgia , Lactente , Pseudo-Obstrução Intestinal/cirurgia , Pseudo-Obstrução Intestinal/diagnóstico , Recém-Nascido , Pré-Escolar , MutaçãoRESUMO
Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare, congenital functional intestinal obstruction, characterised by megacystis (bladder distention in the absence of mechanical obstruction), microcolon and intestinal hypoperistalsis (dysmotility).We are reporting a case of a female child with normal antenatal course who presented with recurrent episodes of abdominal distension since the second day of life and underwent negative exploratory laparotomy on multiple occasions. She also had urinary retention with a grossly distended bladder, requiring drainage by clean intermittent catheterisation. Surgical procedures for bowel decompression, including gastrostomy and ileostomy, were carried out without success. Genetic analysis revealed a mutation in the human smooth muscle (enteric) gamma-actin gene (ACTG2 gene), clinching the diagnosis of MMIHS. The patient was managed with parenteral nutrition and prokinetic medications and tolerated jejunostomy feeds for a brief period before she succumbed to the illness.Female neonates or infants presenting with abdominal distension and dilated urinary tract should be investigated for MMIHS early on. A timely diagnosis will enable the early involvement of a multidisciplinary team to provide the best options available for management.
Assuntos
Anormalidades Múltiplas , Colo/anormalidades , Doenças Fetais , Pseudo-Obstrução Intestinal , Bexiga Urinária/anormalidades , Retenção Urinária , Lactente , Recém-Nascido , Criança , Humanos , Feminino , Gravidez , Pseudo-Obstrução Intestinal/diagnóstico , Pseudo-Obstrução Intestinal/terapia , Pseudo-Obstrução Intestinal/genética , Anormalidades Múltiplas/diagnóstico , Anormalidades Múltiplas/terapia , Anormalidades Múltiplas/genética , Colo/cirurgia , PeristaltismoRESUMO
Idiopathic chronic intestinal pseudo-obstruction (CIPO) is associated with intestinal inflammation and malabsorption and may cause serum vitamin D deficiency. We aimed to assess whether there is an association between idiopathic CIPO and serum levels of 25-hydroxy-vitamin D. Consecutive patients with confirmed diagnosis of idiopathic CIPO were prospectively enrolled and matched with healthy controls by gender, age, and BMI. Median serum level of 25-hydroxy-vitamin D of patients with CIPO was compared with that of healthy subjects using the Wilcoxon signed-rank test for matched samples. A total of 35 patients with CIPO and 35 matched healthy subjects were enrolled. All patients with CIPO had a 25-hydroxy-vitamin D deficiency with serum levels <12â ng/ml. The median serum level of vitamin D was significantly lower in patients with CIPO than in healthy controls (5.7 vs. 29.7â ng/ml, P â <â 0.0001). Serum level of vitamin D was not associated with gender ( P â =â 0.27), age ( P â =â 0.22), BMI ( P â =â 0.95), high (>10â 000â ×â ml) WBC count ( P â =â 0.08), or high (>5â mg/l) C-reactive protein ( P â =â 0.87) among patients with CIPO. CIPO seems to be strongly associated with low serum levels of 25-hydroxy-vitamin D.
Assuntos
Pseudo-Obstrução Intestinal , Deficiência de Vitamina D , Humanos , Vitamina D , Pseudo-Obstrução Intestinal/diagnóstico , Pseudo-Obstrução Intestinal/etiologia , Deficiência de Vitamina D/complicações , Deficiência de Vitamina D/diagnóstico , Doença CrônicaRESUMO
Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is an uncommon genetic disorder inherited in an autosomal recessive pattern that affects the muscles that line the bladder and intestines. The most common genes associated with MMIHS mutations are ACTG2, LMOD1, MYH11, MYL9, MYLK, and PDCL3. However, the complete genetic landscape of MMIHS still needs to be fully understood. The diagnosis of MMIHS can be challenging. However, advances in prenatal and diagnostic techniques, such as ultrasound and fetal urine analysis, have improved the ability to detect the syndrome early. Targeted next-generation sequencing (NGS) and other diagnostic tests can also diagnose MMIHS. The management of MMIHS involves addressing severe intestinal dysmotility, which often necessitates total parenteral nutrition (TPN), which can lead to complications such as hepatotoxicity and nutritional deficiencies. Multivisceral and intestinal transplantation has emerged as therapeutic options, offering the potential for improved outcomes and enteral autonomy. Understanding the genetic underpinnings of MMIHS is crucial for personalized care. While the prognosis varies, timely interventions and careful monitoring enhance patient outcomes. Genetic studies have given us valuable insights into the molecular mechanisms of MMIHS. These studies have identified mutations in genes involved in the development and function of smooth muscle cells. They have also shown that MMIHS is associated with defects in the signaling pathways that control muscle contraction. Continued research in the genetics of MMIHS holds promise for unraveling the complexities of MMIHS and improving the lives of affected individuals.
Assuntos
Anormalidades Múltiplas , Colo , Pseudo-Obstrução Intestinal , Mutação , Bexiga Urinária , Humanos , Pseudo-Obstrução Intestinal/genética , Pseudo-Obstrução Intestinal/terapia , Pseudo-Obstrução Intestinal/diagnóstico , Bexiga Urinária/anormalidades , Colo/anormalidades , Anormalidades Múltiplas/genética , Sequenciamento de Nucleotídeos em Larga EscalaRESUMO
Gastrointestinal involvement in systemic sclerosis can be severe, reaching the critical point of chronic intestinal pseudo-obstruction, secondary to major disorders of small bowel motility. It is associated with some clinical and biological characteristics, in particular the positivity of anti-fibrillarin/U3RNP antibodies. Chronic intestinal pseudo-obstruction (CIPO) is complicated by a small intestinal bacterial overgrowth that requires cyclic antibiotic therapy. CIPO leads to a reduction of the food intake, due to painful symptoms, nausea and vomiting caused by meals, and ultimately to severe malnutrition. Meal splitting is often transiently effective and patients require exogenous nutritional support, mostly parenteral. Systemic sclerosis is not an obstacle to initiation and long-term continuation of parenteral nutrition and central venous catheter implantation is not associated with an increased risk of cutaneous or infectious complications. However, continuation of long-term parenteral nutrition requires monitoring in an expert nutrition center in order to adapt nutritional volumes and intakes and to limit potentially fatal cardiac and hepatobiliary complications. In addition to nutrition, prokinetic treatments, whose side effects must be known, can be associated. Invasive procedures, whose risk-benefit ratio must be carefully assessed, can also be used to treat symptoms exclusively.
Assuntos
Pseudo-Obstrução Intestinal , Escleroderma Sistêmico , Humanos , Pseudo-Obstrução Intestinal/diagnóstico , Pseudo-Obstrução Intestinal/etiologia , Pseudo-Obstrução Intestinal/terapia , Nutrição Parenteral/efeitos adversos , Intestino Delgado , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/terapia , Medição de Risco , Doença CrônicaRESUMO
BACKGROUND: Pediatric chronic intestinal pseudo-obstruction (PIPO) is a rare disease characterized by symptoms and radiological signs suggestive of intestinal obstruction, in the absence of lumen-occluding lesions. It results from an extremely severe impairment of propulsive motility. The intestinal endocrine system (IES) jointly with the enteric nervous system (ENS) regulates secreto-motor functions via different hormones and bioactive messengers/neurotransmitters. The neurotransmitter 5-hydroxytryptamine (5-HT) (or serotonin) is linked to intestinal peristalsis and secretory reflexes. Gut microbiota and its interplay with ENS affect 5-HT synthesis, release, and the subsequent serotonin receptor activation. To date, the interplay between 5-HT and gut microbiota in PIPO remains largely unclear. This study aimed to assess correlations between mucosa associated microbiota (MAM), intestinal serotonin-related genes expression in PIPO. To this purpose, biopsies of the colon, ileum and duodenum have been collected from 7 PIPO patients, and 7 age-/sex-matched healthy controls. After DNA extraction, the MAM was assessed by next generation sequencing (NGS) of the V3-V4 region of the bacterial RNA 16 S, on an Illumina Miseq platform. The expression of genes implicated in serotoninergic pathway (TPH1, SLC6A4, 5-HTR3 and 5-HTR4) was established by qPCR, and correlations with MAM and clinical parameters of PIPO have been evaluated. RESULTS: Our results revealed that PIPO patients exhibit a MAM with a different composition and with dysbiosis, i.e. with a lower biodiversity and fewer less connected species with a greater number of non-synergistic relationships, compared to controls. qPCR results revealed modifications in the expression of serotonin-related intestinal genes in PIPO patients, when compared to controls. Correlation analysis do not reveal any kind of connection. CONCLUSIONS: For the first time, we report in PIPO patients a specific MAM associated to underlying pathology and an altered intestinal serotonin pathway. A possible dysfunction of the serotonin pathway, possibly related to or triggered by an altered microbiota, may contribute to dysmotility in PIPO patients. The results of our pilot study provide the basis for new biomarkers and innovative therapies targeting the microbiota or serotonin pathways in PIPO patients.
Assuntos
Microbioma Gastrointestinal , Pseudo-Obstrução Intestinal , Humanos , Criança , Serotonina/metabolismo , Projetos Piloto , Intestinos , Pseudo-Obstrução Intestinal/genética , Pseudo-Obstrução Intestinal/diagnóstico , Proteínas da Membrana Plasmática de Transporte de SerotoninaRESUMO
BACKGROUND: Chronic intestinal pseudo-obstruction (CIPO) is a rare intestinal disorder characterized by impaired propulsion of the digestive tract and associated with symptoms of intestinal obstruction, despite the absence of obstructive lesions. CIPO includes several diseases. However, definitive diagnosis of its etiology is difficult only with symptoms or imaging findings. CASE PRESENTATION: A 56-year-old man was referred to our hospital due to a 3-year history of continuous abdominal distention. Imaging, including computed tomography of the abdomen, and endoscopy revealed marked dilatation of the entire small intestine without any obstruction point. Therefore, he was diagnosed with CIPO. Since medical therapy didn't improve his symptoms, enterostomy and percutaneous endoscopic gastro-jejunostomy were performed. These procedures improved abdominal symptoms. However, he required home central venous nutrition due to dehydration. The pathological findings of full-thickness biopsies of the small intestine taken during surgery revealed decreased number and degeneration of ganglion cells in the normal plexus. These findings led to a final diagnosis of CIPO due to acquired isolated hypoganglionosis (AIHG). CONCLUSIONS: Here, we report the case of a patient with CIPO secondary to adult-onset AIHG of the small intestine. Since AIHG cannot be solely diagnosed using clinical findings, biopsy is important for its diagnosis.
Assuntos
Obstrução Intestinal , Pseudo-Obstrução Intestinal , Masculino , Adulto , Humanos , Pessoa de Meia-Idade , Pseudo-Obstrução Intestinal/etiologia , Pseudo-Obstrução Intestinal/cirurgia , Pseudo-Obstrução Intestinal/diagnóstico , Dilatação Patológica , Atrofia Muscular , Intestino Delgado/cirurgia , Doença CrônicaRESUMO
Chronic intestinal dysmotility is a rare and debilitating digestive disorder characterized by symptoms of mechanical obstruction without an organic lesion. It has diverse causes and involves various pathological mechanisms. Small bowel manometry is the preferred diagnostic method, particularly for patients with severe and progressive symptoms. The condition can be categorized as intestinal pseudo-obstruction and enteric dysmotility, both entities share abnormal small bowel motility, but with important differences in prognosis and management.
Assuntos
Íleus , Pseudo-Obstrução Intestinal , Humanos , Motilidade Gastrointestinal , Pseudo-Obstrução Intestinal/diagnóstico , Pseudo-Obstrução Intestinal/terapia , Pseudo-Obstrução Intestinal/etiologia , Intestino Delgado/patologia , Prognóstico , Doença CrônicaRESUMO
Chronic intestinal pseudo-obstruction is a rare and heterogeneous syndrome characterized by recurrent symptoms of intestinal obstruction with radiological features of dilated small or large intestine with air/fluid levels in the absence of any mechanical occlusive lesion. Several diseases may be associated with chronic intestinal pseudo-obstruction and in these cases, the prognosis and treatment are related to the underlying disease. Also, in its "primary or idiopathic" form, two subgroups of patients should be determined as they require a more specific therapeutic approach: patients whose chronic intestinal pseudo-obstruction is due to sporadic autoimmune/inflammatory mechanisms and patients whose neuromuscular changes are genetically determined. In a context of a widely heterogeneous adult population presenting chronic intestinal pseudo-obstruction, this review aims to summarize a practical diagnostic workup for identifying definite subgroups of patients who might benefit from more specific treatments, based on the etiology of their underlying condition.
Assuntos
Obstrução Intestinal , Pseudo-Obstrução Intestinal , Adulto , Humanos , Pseudo-Obstrução Intestinal/diagnóstico , Pseudo-Obstrução Intestinal/etiologia , Pseudo-Obstrução Intestinal/terapia , Obstrução Intestinal/complicações , Prognóstico , Doença Crônica , SíndromeRESUMO
OBJECTIVES: Antroduodenal manometry (ADM) measures antral and small bowel motility and is clinically used to evaluate upper gastrointestinal (UGI) symptoms. We aimed to evaluate its utility in guiding treatment, predicting response, and association with clinical findings. METHODS: Retrospective review of 200 children undergoing ADM. ADM interpretation and parameters were compared to outcomes (response to first therapy after ADM and overall response), predominant symptom (group A, abdominal distention and/or vomiting and group B, abdominal pain and/or nausea), etiology (idiopathic or with known comorbidity), and ADM indication [suspected chronic intestinal pseudo-obstruction (CIPO) or unexplained UGI symptoms]. RESULTS: We found an association between a normal intestinal phase III of the migrating motor complex (MMC) and idiopathic etiology, group B symptoms and unexplained UGI symptoms. No variable was associated with initial successful response. However, normal small bowel phase III of the MMC and idiopathic etiology were associated with overall successful response to treatment (including feeding tolerance and weaning of parenteral nutrition). No antral ADM parameter was associated with outcomes or other comparisons. The time to overall successful treatment response was significantly shorter in patients with a normal ADM and presence of a normal phase III of the MMC. CONCLUSIONS: The presence of the phase III of the MMC was the single ADM parameter predictive of overall treatment response, also associated to group B symptoms and idiopathic etiology. Our findings suggest that small bowel ADM parameters are more useful to predict outcomes and ADM should be performed primarily in patients presenting with abdominal distention and/or vomiting and those being evaluated for CIPO.
Assuntos
Gastroenteropatias , Pseudo-Obstrução Intestinal , Trato Gastrointestinal Superior , Criança , Humanos , Gastroenteropatias/diagnóstico , Motilidade Gastrointestinal/fisiologia , Manometria , Vômito/diagnóstico , Vômito/etiologia , Pseudo-Obstrução Intestinal/diagnóstico , Pseudo-Obstrução Intestinal/terapia , Doença Crônica , DuodenoRESUMO
BACKGROUND: Intestinal pseudo-obstruction (IPO) is a rare disease, and its clinical manifestations can resemble mechanical intestinal obstruction leading to unnecessary and potentially harmful surgery. Certain autoimmune diseases have been associated with IPO, however, cases secondary to Sjögren's syndrome (SjS) are especially rare. CASE PRESENTATION: We described the first case of SjS-associated acute IPO in pregnancy, which was successfully treated with combined immunosuppressive therapy and resulted in an uneventful caesarean delivery. CONCLUSIONS: Women with SjS is likely to experience more complications during pregnancy, and IPO rather than the classic symptoms could be the first sign of SjS flares. IPO should be suspected in patients with unrelenting symptoms of small bowel obstruction, and a multidisciplinary approach can provide optimal management of such high-risk pregnancies.
Assuntos
Doenças Autoimunes , Pseudo-Obstrução Intestinal , Síndrome de Sjogren , Gravidez , Humanos , Feminino , Síndrome de Sjogren/complicações , Pseudo-Obstrução Intestinal/etiologia , Pseudo-Obstrução Intestinal/diagnóstico , Pseudo-Obstrução Intestinal/tratamento farmacológicoRESUMO
BACKGROUND: Little is known about ileal motility patterns and their utility in children. Here, we present our experience with children undergoing ileal manometry (IM). METHODS: A retrospective review of children with ileostomy comparing IM between 2 groups: A [chronic intestinal pseudo-obstruction (CIPO)] and B (feasibility of ileostomy closure in children with defecation disorders). We also compared the IM findings with those from antroduodenal manometry (ADM), and evaluated the joint effect of age, sex, and study indication group on IM results. RESULTS: A total of 27 children (median age 5.8 years old, range 0.5-16.74 years, 16 were female) were included (12 in group A and 15 in group B). There was no association between IM interpretation and sex; however younger age was associated with abnormal IM ( P = 0.021). We found a significantly higher proportion of patients with presence of phase III of the migrating motor complex (MMC) during fasting and normal postprandial response in group B than in group A ( P < 0.001). Logistic regression analysis revealed that only Group B was associated with normal IM ( P < 0.001). We found a moderate agreement for the presence of phase III MMC and postprandial response between IM and ADM (kappa = 0.698, P = 0.008 and kappa = 0.683, P = 0.009, respectively). CONCLUSION: IM is abnormal in patients with CIPO and normal in patients with defecation disorders, suggesting that IM may be not needed for ostomy closure in those with defecation disorders. IM has a moderate agreement with ADM and could be used as a surrogate for small bowel motility.
Assuntos
Defecação , Pseudo-Obstrução Intestinal , Criança , Humanos , Feminino , Lactente , Pré-Escolar , Adolescente , Masculino , Pseudo-Obstrução Intestinal/diagnóstico , Pseudo-Obstrução Intestinal/cirurgia , Motilidade Gastrointestinal/fisiologia , Complexo Mioelétrico Migratório/fisiologia , Intestino Delgado , Doença Crônica , Manometria/métodosRESUMO
BACKGROUND: Chronic intestinal pseudo-obstruction (CIPO) may be a primary or secondary phenomenon and is often multifactorial. Treatment is largely directed at improving colonic motility. The use of cholinesterase inhibitors such as pyridostigmine has been hypothesized to increase acetylcholine in the bowel, improving symptoms and transit times. METHODS: A systematic review of the use of pyridostigmine in CIPO was conducted using scientific and commercial search engines identifying scientific studies enrolling adult human subjects, published from 2000 to 2022 in the English language. RESULTS: Four studies were identified including two randomized controlled trials (RCT) and two observational studies. The studies had heterogenous inclusion criteria, dosing regimens and reported outcomes. Two studies were identified as being at high risk of bias. All studies reported improved patient outcomes with use of pyridostigmine, and low rates (4.3%) of mild cholinergic side effects. No major side effects were reported. CONCLUSION: The use of pyridostigmine in management of CIPO is biologically plausible due to its ability to increase colonic motility, and early studies on its role are uniformly suggestive of benefit with low side-effect profile. Four clinical studies have been conducted to date, with small sample sizes, heterogeneity and high risk of bias. Further high-quality studies are required to enable assessment of pyridostigmine's utility as an effective management strategy in CIPO.
Assuntos
Pseudo-Obstrução Intestinal , Brometo de Piridostigmina , Adulto , Humanos , Brometo de Piridostigmina/uso terapêutico , Brometo de Piridostigmina/farmacologia , Motilidade Gastrointestinal , Pseudo-Obstrução Intestinal/tratamento farmacológico , Pseudo-Obstrução Intestinal/diagnóstico , Inibidores da Colinesterase/uso terapêutico , Inibidores da Colinesterase/farmacologia , Doença CrônicaRESUMO
Chronic intestinal pseudo-obstruction (CIPO) is caused by a plethora of primary and secondary causes, dominantly involving the neuromuscular tissue, interstitial cells of Cajal, or the connective tissue framework. The lack of the connective tissue framework, known as desmosis, is evaluated by Masson's trichrome (MT) or picrosirius red stains, both of which are recommended in the London classification. We evaluated the orcein stain in detecting desmosis in comparison to the MT stain. We performed both orcein and MT stains in six previously published cases of complete or partial desmosis along with six age-matched controls. Our results showed comparable results of the orcein stain as compared to the MT stain. Additional advantages of lower cost and a clearer background in orcein stain were noteworthy, whereas MT stain can be used for the detection of additional pathology. We believe that orcein stain can be used as a cheap alternative in resource-limited settings.
Assuntos
Pseudo-Obstrução Intestinal , Verde de Metila , Humanos , Coloração e Rotulagem , Pseudo-Obstrução Intestinal/diagnóstico , Doença CrônicaRESUMO
OBJECTIVES: Pediatric intestinal pseudo-obstruction (PIPO) management is based on nutritional, medical, and surgical care while available evidence is scarce. The aim of this study was to outline the current diagnostic and management strategies in intestinal failure (IF) teams of the European Reference Network for rare Inherited and Congenital Anomalies (ERNICA) and to compare these practices to the latest PIPO international guidelines. METHODS: An online survey on institutional diagnostic and management strategies of PIPO was conducted among the ERNICA IF teams. RESULTS: In total, 11 of 21 ERNICA IF centers from 8 countries participated. On average, 64% of teams had ≥6 and 36% had 1-5 PIPO patients under active follow-up. In total, 80 of 102 PIPO patients were parenteral nutrition (PN) dependent while each IF team had median 4 (range 0-19) PN dependent PIPO patients under follow-up. On average, each center received 1-2 new PIPO patients per year. Diagnostics mostly followed current guidelines while medical and surgical management strategies were diverse. CONCLUSIONS: Numbers of PIPO patients are low and management strategies are diverse among ERNICA IF teams. To improve PIPO patient care, regional reference centers with specialized multidisciplinary IF teams and continuous collaboration across centers are needed.