A Rare Coexistence: Achalasia Esophagus and Acute Intestinal Pseudo-Obstruction.

Achalasia esophagus and acute intestinal pseudo-obstruction are distinct gastrointestinal motility disorders rarely found together in the same patient. We present a case of a 96-year-old woman exhibiting symptoms of both conditions, including dysphagia, regurgitation, abdominal distension, nausea, vomiting, and constipation. Diagnostic evaluations revealed esophageal dilation with a "bird beak" sign on timed barium swallows and significant bowel dilation without mechanical obstruction on computed tomography scans. Treatment involved conservative measures for acute intestinal pseudo-obstruction and palliative approaches for achalasia esophagus. The coexistence of these disorders raises questions about potential shared pathophysiological mechanisms involving the enteric nervous system or smooth muscle dysfunction. Further research is warranted to elucidate these connections and improve management strategies for such complex cases.

Time is Gut. Approaching Intestinal Leiomyositis: Case Presentation and Literature Review.

T-lymphocytic intestinal leiomyositis is a rare cause of "pediatric intestinal pseudo-obstructions." Diagnosis may be difficult and requires full-thickness bowel biopsies during laparotomy or laparoscopy with possible enterostomy. Currently, immunosuppressive therapy is the only available treatment. A delay in diagnosis and therapy may negatively affect the prognosis because of ongoing fibrotic alterations; therefore, early diagnosis and consequent treatment are crucial. This review summarizes the available information on the nosology, diagnostic steps, and treatment modalities. Here, we report the youngest case of enteric leiomyositis reported in the last two decades and analyze its management by reviewing previous cases.

Antroduodenal manometry findings in children with suspected pediatric intestinal pseudo-obstruction.

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.

Bowel pseudo-obstruction caused by goblet cell adenocarcinoma of the appendix: A case report.

INTRODUCTION: Goblet cell adenocarcinoma of the appendix is a rare diagnosis with features of both adenocarcinomas and carcinoid tumors. Commonly presenting with chronic abdominal pain, appendicitis, or abdominal distention, it can also be incidentally discovered during appendectomies. CASE PRESENTATION: A 50-year-old man with right lower abdominal pain was admitted to our hospital, which is a critical care center. A computed tomography(CT) scan showed ileal narrowing, but endoscopy found no strictures. He was admitted with suspected bowel obstruction and improved with an ileal tube. Laparoscopic surgery revealed a tumor of the appendix. Histologically, he was diagnosed goblet cell adenocarcinoma, suggesting tumor infiltration of nerve fibers impairing peristalsis. DISCUSSION: Goblet cell adenocarcinoma of the appendix has unique histology and a poor prognosis. Treatment typically involves surgery and chemotherapy. This case highlights challenges in preoperative diagnosis, with the tumor causing bowel pseudo-obstruction by invading the intestinal wall and nerve plexus. Extensive infiltration of Auerbach's plexus was observed, consistent with the length of intestinal stenosis. CONCLUSION: This case describes goblet cell adenocarcinoma of the appendix leading to bowel pseudo-obstruction due to ileal end stenosis. It emphasizes the importance of considering this diagnosis in cases of bowel obstruction without an obvious mass.

Acute colonic pseudo-obstruction: a retrospective review of the surgical outcomes.

PURPOSE: Limited data exist regarding the surgical outcomes of acute colonic pseudo-obstruction (ACPO), commonly referred to as Ogilvie syndrome, in modern clinical practice. The prevailing belief is that surgery should be avoided due to previously reported high mortality rates. We aimed to describe the surgical results of ACPO treated within our institution. METHODS: Our prospectively maintained colorectal surgery registry was queried for patients diagnosed with ACPO, who underwent surgery between 2009 and 2022. Postoperative complications were graded according to Clavien-Dindo (CD) classification. The primary outcome was postoperative mortality. RESULTS: A total of 32 patients who underwent surgery for ACPO were identified. Overall, nonoperative therapy was initially administered to 21 patients (65.6%). The surgeries performed included total abdominal colectomy (15, 43.1%), ascending colectomy with end ileostomy (8, 25%), transverse colostomy (5, 15.6%), ileostomy and transverse colostomy (3, 9.4%), and Hartmann's operation (1, 3.1%). Severe postoperative complications (CD grade 3 or 4) occurred in five patients (15.6%). No recurrence of ACPO was observed and no patient required reoperation. The average postoperative length of stay was 14.5 days, 30-day mortality was 6.3% (n = 2), and 90-day mortality was 15.6% (n = 5) due to complications of underlying comorbidities. CONCLUSIONS: Surgical treatment was effective for patients with ACPO refractory to medical therapy or presenting with acute complications. Although postoperative complications were frequent, both the 30- and 90-day mortality rates were lower than previously documented in the literature. Further investigations are warranted to determine the optimal surgical strategy, which may involve total or segmental colectomy, or diversion alone without resection.

A Case of Acute Colonic Pseudo-Obstruction and Anastomotic Leakage After Sigmoidectomy for Sigmoid Volvulus.

Acute colonic pseudo-obstruction (ACPO) is characterized by significant colonic distension without a mechanical obstruction. We present a case of an 83-year-old male who developed ACPO following laparoscopic surgery for sigmoid volvulus. This report details the patient's postoperative journey, highlighting the diagnostic and management challenges encountered. Despite various medical interventions, the patient's condition necessitated further surgical attention due to complications. This case underscores the importance of early diagnosis and aggressive management in ACPO to prevent life-threatening consequences and improve patient outcomes.

Recurrent Ogilvie Syndrome as a Possible Manifestation of Alpha-Gal Syndrome (AGS) Requiring Surgical Management: A Case Report.

We present a case of a male in his 60s with a history of alpha-gal syndrome (AGS) who presented with recurrent acute colonic pseudo-obstruction, also known as Ogilvie syndrome, and underwent surgical treatment for life-limiting symptoms of colonic distention, constipation, and abdominal pain. Prior to surgery, he was hospitalized multiple times after beef consumption and was diagnosed with Ogilvie syndrome, requiring a colonoscopy with rectal tube placement for symptom resolution. He later underwent a robotic subtotal colectomy with ileocolic anastomosis. Follow-up visits showed improvement in symptoms of constipation and abdominal distention. This case highlights that AGS may lead to severe manifestations, such as recurrent Ogilvie syndrome. Due to the increasing prevalence of AGS and limited data on disease course, further research is needed to determine symptom manifestations and the potential utility of surgery in management.

Bowel function and inflammation: Is motility the other side of the coin?

Digestion and intestinal absorption allow the body to sustain itself and are the emblematic functions of the bowel. On the flip side, functions also arise from its role as an interface with the environment. Indeed, the gut houses microorganisms, collectively known as the gut microbiota, which interact with the host, and is the site of complex immune activities. Its role in human pathology is complex and scientific evidence is progressively elucidating the functions of the gut, especially regarding the pathogenesis of chronic intestinal diseases and inflammatory conditions affecting various organs and systems. This editorial aims to highlight and relate the factors involved in the pathogenesis of intestinal and systemic inflammation.

Ogilvie's Syndrome in a Young Female With Chronic Constipation.

Ogilvie's syndrome, also known as acute colonic pseudo-obstruction, is often encountered in post-surgical patients or those with serious comorbidities requiring intensive care. For this reason, it has rarely been reported in patients younger than 50 years without any predisposing risk factors. Our case report highlights a unique case of Ogilvie's syndrome in a young female with no recent trauma or surgical history. To that extent, we discuss risk factors that predisposed her to this condition, including her history of chronic constipation. We also emphasize the need for outpatient workups for such patients to prevent the worsening of their symptoms.

Efficacy and safety of rifaximin in patients with chronic intestinal pseudo-obstruction: a randomized, double-blind, placebo-controlled, phase II-a exploratory trial.

Chronic intestinal pseudo-obstruction (CIPO) is a rare intractable disease with limited treatment options. Small intestinal bacterial overgrowth (SIBO) often co-occurs with several diseases, including CIPO. While rifaximin (RFX) is effective in treating SIBO, its efficacy for CIPO remains unclear. Here, we aimed to investigate the efficacy and safety of RFX in adult patients with CIPO. Twelve patients were randomly assigned to receive RFX (400 mg three times daily, n=8) or a placebo (PBO, n=4) for 4 weeks. The global symptom score for abdominal bloating (GSS-bloating) and an original whole gastrointestinal symptoms score (O-WGSS) were collected, and a glucose hydrogen breath test (GHBT) and abdominal computed tomography (CT) were performed. No significant differences were observed in the primary endpoint. GSS-bloating improved by 75% and 25% in the PBO and RFX groups, respectively, and O-WGSS improved by 25% in both groups. No significant differences were observed in secondary and other endpoints, including the SIBO eradication rate in the GHBT and small intestinal volume on CT. In a post hoc analysis of SIBO-positive patients with CIPO (4/4 and 4/8 in the PBO and RFX groups), SIBO was eradicated in 25% and 75% of the patients (PBO and RFX groups, respectively) at the end of treatment, indicating a high eradication rate in the RFX group. Furthermore, the small intestinal gas volume decreased in the RFX group, and no severe adverse events occurred. Although no significant improvements were observed in subjective indicators, RFX may be beneficial in alleviating SIBO and reducing the small intestinal gas volume in SIBO-positive patients with CIPO.

Enteric Nervous System Striped Patterning and Disease: Unexplored Pathophysiology.

The enteric nervous system (ENS) controls gastrointestinal (GI) motility, and defects in ENS development underlie pediatric GI motility disorders. In disorders such as Hirschsprung's disease (HSCR), pediatric intestinal pseudo-obstruction (PIPO), and intestinal neuronal dysplasia type B (INDB), ENS structure is altered with noted decreased neuronal density in HSCR and reports of increased neuronal density in PIPO and INDB. The developmental origin of these structural deficits is not fully understood. Here, we review the current understanding of ENS development and pediatric GI motility disorders incorporating new data on ENS structure. In particular, emerging evidence demonstrates that enteric neurons are patterned into circumferential stripes along the longitudinal axis of the intestine during mouse and human development. This novel understanding of ENS structure proposes new questions about the pathophysiology of pediatric GI motility disorders. If the ENS is organized into stripes, could the observed changes in enteric neuron density in HSCR, PIPO, and INDB represent differences in the distribution of enteric neuronal stripes? We review mechanisms of striped patterning from other biological systems and propose how defects in striped ENS patterning could explain structural deficits observed in pediatric GI motility disorders.

Megacystis-Microcolon-Intestinal Hypoperistalsis Syndrome: A Case Report of an Uncommon Condition.

The megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS), also known as Berdon syndrome, is a rare congenital condition that falls within the spectrum of visceral myopathies. It is characterized by the presence of megacystis, microcolon, and hypoperistalsis, which are secondary to gastrointestinal and urinary system dysmotility. It is frequently associated with other alterations in the gastrointestinal and genitourinary tracts. Although it is possible to make the diagnosis in the prenatal period, most cases are diagnosed after birth through genetic and imaging studies. Advances in treatment have led to a progressive increase in survival rates. We present the case of a newborn with congenital alterations described prenatally and with imaging findings characteristic of the syndrome.

Clozapine-Induced Refractory Colonic Pseudo-Obstruction.

The management of treatment-resistant schizophrenia (TRS) is challenging as the medications involved, often atypical antipsychotics, have a host of associated adverse effects. While complications such as agranulocytosis are well established and necessitate close hematological monitoring, the gastrointestinal effects of particular atypical antipsychotics, such as clozapine, are recognized to a lesser extent. The following case of TRS leading to chronic treatment-resistant pseudo-obstruction, eventually requiring total colectomy, highlights the considerable sequelae of clozapine on the gastrointestinal tract. Beyond the effects of severe constipation, the possible implications of ischemic colitis, stercoral perforation, and intraabdominal sepsis warrant a degree of caution when prescribing such medication. This study sheds light on the importance of monitoring bowel motility when administering antipsychotics, particularly clozapine, to avoid these deleterious consequences.

A case of acute colonic pseudo-obstruction, sigmoid volvulus, and massive pneumoperitoneum in a young female patient.

INTRODUCTION AND IMPORTANCE: Acute colonic pseudo-obstruction (ACPO) is an uncommon phenomenon that is especially rare in young patients and can result in bowl ischemia and perforation if left untreated. Furthermore, pneumoperitoneum is almost always a concerning imaging finding and in the context of recent colonic resection may be a sign of anastomotic leakage. CASE PRESENTATION: We describe a case of a young female patient with postpartum ACPO who subsequently underwent a hemicolectomy with colorectal anastomosis. The patient's hospital course was complicated by massive postoperative pneumoperitoneum that resulted in resection of the anastomosis and creation of an end colostomy. However, despite this measure, there was recurrent pneumoperitoneum on cross-sectional imaging 36 h later. This was treated non-operatively and the remainder of their hospital course was uneventful. CLINICAL DISCUSSION: A potential etiology for ACPO during pregnancy may be due to compression of parasympathetic plexus nerves by the gravid uterus. Idiopathic pneumoperitoneum has been documented on a number of occasions, though this is generally in older patients. It can present with signs of peritonitis or can be asymptomatic. Simultaneous pneumothorax and pneumoperitoneum is rare and may be due to the transmission of air from the peritoneum to the mediastinum and thorax. The pneumoperitoneum itself may be due the air leakage through the significantly distended colon into the peritoneum. CONCLUSION: The combination of ACPO following pregnancy and associated pneumothorax, pneumomediastinum, and recurrent pneumoperitoneum suggest a communicating defect between the thoracic, mediastinal, and peritoneal cavities. Furthermore, the possibility of underlying colonic dysmotility should be considered prior to the restoration of large bowel continuity.

Chronic intestinal pseudo-obstruction: associations with gut microbiota and genes expression of intestinal serotonergic pathway.

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.

[Severe small bowel involvement and chronic intestinal pseudo-obstruction in systemic sclerosis (scleroderma): Pathophysiological, diagnostic and therapeutic basis, including parenteral nutrition]. / Atteinte sévère de l'intestin grêle et pseudo-obstruction intestinale chronique au cours de la sclérodermie systémique : bases physiopathologiques, diagnostiques et thérapeutiques, dont la nutrition parentérale.

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.

Chronic intestinal pseudo-obstruction due to adult-onset acquired isolated hypoganglionosis with muscular atrophy in the small intestine: a case report and review of literature.

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.

Acute colonic pseudo-obstruction post-cesarean section is not a benign entity: A case series and review of the literature.

Acute colonic pseudo-obstruction (ACPO) is an infrequent occurrence after cesarean section. Anecdotal evidence suggests that the clinical course of ACPO in the obstetric setting is different to that seen in non-pregnant adult patients with ACPO secondary to alternative causes, such as systemic illnesses, the use of certain medications, and after non-abdominal surgery. The risk of progression to ischemia and perforation, as well as the need for emergency surgery, appears to be higher after cesarean section. Here we describe the clinical course of ACPO in four patients after cesarean section from our institution, followed by a review of the literature and a discussion of the important issues surrounding this condition in the postpartum time period. The findings from our cohort of patients and the reports from the medical literature support a hands-on combined approach from a group of specialists including obstetricians, surgeons, radiologists, and enterostomal therapists. Immediate imaging followed by regular observation is mandatory for any patient being managed conservatively. Early use of endoscopic decompression should be considered for patients who are not resolving with a conservative approach. Clinical signs of peritonism or radiological signs of ischemia or perforation in patients with ACPO mandate immediate surgical intervention. Appropriate postoperative care is necessary to deal with the complex physiological and psychological consequences of emergency surgery and potential stoma formation so soon after cesarean section.

Pheochromocytoma leading to Takotsubo and Ogilvie syndrome, a pathophysiological triad.

Takotsubo syndrome (TTS) is a transient left ventricle dysfunction usually caused by a stressful trigger (emotional or physical). We report the case of a 77 year-old female patient who presented with TTS caused by a pheochromocytoma, a catecholamine-producing neuroendocrine tumour. Diagnosis was facilitated by acute kidney injury prompting renal ultrasound, recurrence of TTS and symptoms of episodic palpitations, profuse sweating and labile blood pressure. Furthermore, during her hospitalisation the patient also developed an Ogilvie syndrome, an acute colonic pseudo-obstruction, due to the catecholamine-excess. Treatment consisted of betablocker and angiotensin-converting enzyme inhibitor for TTS, neostigmine for Ogilvie syndrome, in combination with alpha-blocker and surgical removal of the tumour after recuperation of left ventricular function and colonic pseudo-obstruction. To our knowledge, this is the first case report of the pathophysiological triad of pheochromocytoma leading to Takotsubo and Ogilvie syndrome in a single patient.

Chronic intestinal pseudo-obstruction in adults: A practical guide to identify patient subgroups that are suitable for more specific treatments.

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.