The rare Ogilvie's Syndrome in pregnancy. How to manage? A case report and literature review.

Acute colonic pseudo-obstruction, or Ogilvie's syndrome (OS), is a complication in gynaecology and obstetrics. Its occurrence during pregnancy is rare, redefining the therapeutic decision-making and treatment options. In this review we describe the case of a 37-year-old pregnant patient who developed OS at the 30th week of gestation. A laparotomy with colonic decompression was performed. Foetal condition, regularly monitored throughout the hospital stay, remained normal. The patient experienced an uncomplicated, natural delivery at 40 weeks. A comprehensive literature search, describing the occurrence of OS during pregnancy, was conducted. We identified six cases of OS arising during pregnancy. Demographic, clinical, diagnostic and therapeutic features were analysed. Non-surgical management is generally the first-line option, with intravenous drug administration, rectal and nasogastric tube positioning and colonoscopic decompression the treatments of choice. Surgical decompression in usually performed in cases of failure of the first-line treatments. Including our own experience, in all cases, neither maternal nor foetal mortality was reported. A conservative approach is mandatory as first-line treatment, but when prompt resolution is not achieved, a multidisciplinary team, involving the gynaecologist/obstetrician, the surgeon, the radiologist and the intensivist is mandatory to avoid diagnostic delays, thereby reducing morbidity and mortality rates.

Interstitial cells of Cajal and human colon motility in health and disease.

Our understanding of human colonic motility, and autonomic reflexes that generate motor patterns, has increased markedly through high-resolution manometry. Details of the motor patterns are emerging related to frequency and propagation characteristics that allow linkage to interstitial cells of Cajal (ICC) networks. In studies on colonic motor dysfunction requiring surgery, ICC are almost always abnormal or significantly reduced. However, there are still gaps in our knowledge about the role of ICC in the control of colonic motility and there is little understanding of a mechanistic link between ICC abnormalities and colonic motor dysfunction. This review will outline the various ICC networks in the human colon and their proven and likely associations with the enteric and extrinsic autonomic nervous systems. Based on our extensive knowledge of the role of ICC in the control of gastrointestinal motility of animal models and the human stomach and small intestine, we propose how ICC networks are underlying the motor patterns of the human colon. The role of ICC will be reviewed in the autonomic neural reflexes that evoke essential motor patterns for transit and defecation. Mechanisms underlying ICC injury, maintenance, and repair will be discussed. Hypotheses are formulated as to how ICC dysfunction can lead to motor abnormalities in slow transit constipation, chronic idiopathic pseudo-obstruction, Hirschsprung's disease, fecal incontinence, diverticular disease, and inflammatory conditions. Recent studies on ICC repair after injury hold promise for future therapies.

Acute colonic pseudo-obstruction (Ogilvie syndrome) leading to respiratory compromise and death.

Here, we present an unusual case of acute colonic pseudo-obstruction (ACPO), also known as Ogilvie syndrome, which resulted in respiratory failure and sudden death. The patient was a 19-year-old man with a history of cerebral palsy (CP) and severe autism who experienced marked abdominal distension that progressed over several days in his skilled nursing facility. He developed shortness of breath and episodic diarrhea, before having an unwitnessed cardiac arrest and subsequently expiring after prehospital and emergency department care. Autopsy revealed developmental deformities consistent with CP, rectal prolapse, and significant abdominal distension. Postmortem radiography showed diffuse bowel distension with bilateral upward displacement of the diaphragm and consequent lung compression. Thorough examination of the gastrointestinal tract failed to reveal any perforation or obstruction. The cause of death was determined to be respiratory compromise secondary to diaphragmatic compression as a result of ACPO, a condition defined as colonic distension without a mechanical explanation for obstruction. This case highlights the challenges that ACPO can pose to a forensic pathologist at autopsy, and serves as a teaching point to clinicians on the warning signs and treatment measures for ACPO.

Appendiceal fat stranding on CT: a red herring in a post-caesarean section woman with Ogilvie's syndrome (OS).

A 30-year-old woman was referred for a surgical review with abdominal pain and distension 2 days post-caesearean section. Abdominal X-ray showed dilated bowel loops. CT of her abdomen however showed fat stranding around a thickened appendix, suggesting a differential diagnosis of acute appendicitis on top of a postoperative ileus. Failure to respond to intravenous antibiotics led to an emergent surgical exploratory laparotomy, by which time the progressive caecal dilatation had led to patchy necrosis and perforation of the right hemicolon intra-operatively. The patient required a right hemicolectomy and histological examination of the excised bowel supported the diagnosis of Ogilvie's syndrome. This case highlights the red herrings that one can encounter when faced with a woman with post-caesarean section abdominal pain and aims to raise awareness among clinicians of this condition-where timely diagnosis and management is key.

Acute colonic pseudoobstruction (Ogilvie's syndrome) in gynecologic and obstetric patients: case report and systematic review of the literature.

BACKGROUND: Acute colonic pseudo-obstruction or Ogilvie's syndrome (OS) is a rare form of postsurgical or posttraumatic complication. OS rarely occurs in the postoperative course of gynecologic and obstetric patients and is difficult to diagnose. CASE PRESENTATION: We present the case of an 83-years-old patient with carcinosarcoma of the uterus who developed OS with non-obstructive dilation of the right hemicolon and intraabdominal compression after total abdominal hysterectomy, omentectomy, and lymphadenectomy. Laparotomy with colonic decompression and abdominal dressing was performed. Subsequently, the patient developed pneumonia and peritonitis and died due to septic shock. SYSTEMATIC LITERATURE REVIEW: We identified 49 case reports and 10 case series describing 17 gynecologic (cervical cancer, n = 2; carcinosarcoma of the uterus, n = 1; benign gynecologic condition, n = 14) and 76 obstetric patients (cesarean section, n = 66; OS during pregnancy or after vaginal delivery, n = 10). Outcome data were available for 59 patients. First-line treatment was conservative in 22/59 (37%) cases, laparotomy with decompression or colon resection was performed in 20/59 (34%) cases, endoscopic decompression in 12/59 (20%) cases, and i.v. neostigmine in 4/59 (7%) cases. Resolution was achieved in 22/59 (37%) of patients. The most common second-line treatment was right hemicolectomy. Adverse events grade 3 and 4 were observed in 8/59 and 31/59 patients (together 66%), respectively, mortality was 3/59 (5%). CONCLUSION: OS is a rare postoperative complication of gynecologic and obstetric patients with a good prognosis, but a high morbidity. Pregnancy seems to be a predisposing factor for OS. Conservative treatment is a successful first-line approach.

Histological characteristics of eosinophilic myenteric ganglionitis: an under-recognised cause of chronic intestinal pseudo-obstruction.

Eosinophilic myenteric ganglionitis (EMG) is characterised by eosinophilic infiltration of the myenteric plexus. EMG has been rarely reported as a cause of chronic intestinal pseudo-obstruction (CIPO), and its histopathological features are not fully elucidated. We analysed seven patients with CIPO. Three of them were diagnosed with EMG and four patients were categorised as non-EMG. Clinicopathological features were similar in both groups. These features included subtle to mild lymphocytic infiltration at the myenteric ganglia/muscularis propria, loss of myenteric ganglions and interstitial cells of Cajal (ICC), and no significant findings in the mucosa. The exceptions were moderate to severe degree of eosinophilic infiltration at the myenteric ganglia/muscularis propria in EMG. Functional gastrointestinal obstruction may be associated with inflammatory cell infiltration at the myenteric ganglia/muscularis propria, leading to subsequent hypoganglionosis and deficiency of ICC in EMG. Pathologists and clinicians should be aware of this distinction during differential diagnosis of patients with CIPO.

Ogilvie's syndrome: management and outcomes.

Ogilvie's syndrome, also known as acute colonic pseudo-obstruction, refers to pathologic dilation of the colon without underlying mechanical obstruction, occurring primarily in patients with serious comorbidities. Diagnosis of Ogilvie's syndrome is based on clinical and radiologic grounds, and can be treated conservatively or with interventions such as acetylcholinesterase inhibitors (such as neostigmine), decompressive procedures including colonoscopy, and even surgery. Based on our clinical experience we hypothesized that conservative management yields similar, if not superior, results to interventional management. Therefore, we retrospectively examined all patients over the age of 18 with Ogilvie's syndrome who presented to the Medical University of South Carolina (MUSC). The diagnosis of Ogilvie's syndrome was confirmed by clinical criteria, including imaging evidence of colonic dilation ≥9 cm. Patients were divided and analyzed in 2 groups based on management: conservative (observation, rectal tube, nasogastric tube, fluid resuscitation, and correction of electrolytes) and interventional (neostigmine, colonoscopy, and surgery). Use of narcotics in relation to maximal bowel size was also analyzed. Over the 11-year study period (2005-2015), 37 patients with Ogilvie's syndrome were identified. The average age was 67 years and the average maximal bowel diameter was 12.5 cm. Overall, 19 patients (51%) were managed conservatively and 18 (49%) underwent interventional management. There was no significant difference in bowel dilation (12.0 cm vs 13.0 cm; P = .21), comorbidities (based on the Charlson Comorbidity Index (CCI), 3.2 vs 3.4; P = .74), or narcotic use (P = .79) between the conservative and interventional management groups, respectively. Of the 18 patients undergoing interventional management, 11 (61%) had Ogilvie's-syndrome-related complications compared to 4 (21%) of the 19 patients in the conservative management group (P < .01). There was no difference in overall length of stay in the 2 groups. Two patients, one in each group, died from complications unrelated to their Ogilvie's syndrome. We conclude that Ogilvie's syndrome, although uncommon, and typically associated with severe underlying disease, is currently associated with a low inpatient mortality. While interventional management is often alluded to in the literature, we found no evidence that aggressive measures lead to improved outcomes.

Diagnostic use of endoscopic full-thickness wall resection (eFTR)-a novel minimally invasive technique for colonic tissue sampling in patients with severe gastrointestinal motility disorders.

BACKGROUND: Complex gastrointestinal (GI) motility disorders such as chronic intestinal pseudo-obstruction (CIPO) or Hirschsprung's disease (HD) are challenging to diagnose and treat appropriately. Thorough assessment of patient history, radiographic exams, endoscopy, and motility measurements aid in diagnostic workup, yet underlying histology is the cornerstone to enable a more distinct diagnosis of neuromuscular GI disorders. Traditionally, surgical procedures have been performed to obtain specimen suitable for accurate histologic analysis. METHODS: We performed endoscopic full-thickness resection (eFTR) using a full-thickness-resection device (FTRD) under moderate propofol sedation in four patients with suspected severe neuromuscular gut disorders including CIPO. KEY RESULTS: The mean age of the four patients was 43 y (range 19-56 y). Technical and histological success providing large colonic full-thickness tissue samples of excellent quality was achieved in all four patients (success rate 100%). The mean procedure time was 12 min (range 5-20 min). The mean diameter of the resected specimen was 21 mm (range 20-22 mm). No adverse events connected to the procedure itself occurred. Histology ranged from aganglionosis such as Hirschsprung's disease (HD) to hypoganglionosis and eosinophilic leiomyositis combined with lymphocytic ganglionitis in a third patient. Histology was unspecific in one patient. CONCLUSION AND INFERENCES: EFTR allows safe and minimal invasive harvesting of ample full-thickness tissue samples for accurate histological analysis in patients with suspicion of neuromuscular gut disorders.

A Novel Mutation in Nucleoporin 35 Causes Murine Degenerative Colonic Smooth Muscle Myopathy.

Chronic intestinal pseudo-obstruction (CIPO) is a rare but life-threatening disease characterized by severe intestinal dysmotility. Histopathologic studies in CIPO patients have identified several different mechanisms that appear to be involved in the dysmotility, including defects in neurons, smooth muscle, or interstitial cells of Cajal. Currently there are few mouse models of the various forms of CIPO. We generated a mouse with a point mutation in the RNA recognition motif of the Nup35 gene, which encodes a component of the nuclear pore complex. Nup35 mutants developed a severe megacolon and exhibited a reduced lifespan. Histopathologic examination revealed a degenerative myopathy that developed after birth and specifically affected smooth muscle in the colon; smooth muscle in the small bowel and the bladder were not affected. Furthermore, no defects were found in enteric neurons or interstitial cells of Cajal. Nup35 mice are likely to be a valuable model for the subtype of CIPO characterized by degenerative myopathy. Our study also raises the possibility that Nup35 polymorphisms could contribute to some cases of CIPO.

Spread of tumor microenvironment contributes to colonic obstruction through subperitoneal fibroblast activation in colon cancer.

We evaluated the influence of the cancer microenvironment formed by peritoneal invasion (CMPI) on clinical findings in colon cancer patients. In addition to the association with poor prognosis, we discovered a relationship with bowel obstruction. Detailed analysis revealed that clinical findings related to bowel obstruction occurred more frequently in patients with an elevated type tumor, which had peritoneal elastic laminal elevation to the tumor surface, compared to those with non-elevated type tumors among those with elastic laminal invasion (ELI). Lateral tumor spread and increase of tumor annularity rate in ELI-positive elevated type cases suggested the morphological progression from ELI-positive non-elevated type to elevated type. In addition, α-smooth muscle actin expression was the highest in ELI-positive elevated type, and prominent expressions were found not only in the deep tumor area but also in the shallow tumor area. Furthermore, contraction assays revealed the robust contractile ability of subperitoneal fibroblasts stimulated by cancer cell-conditioned medium. Our findings suggest that CMPI spread into the luminal side of the colonic wall along with tumor progression, which caused bowel obstruction through the activation of subperitoneal fibroblasts. However, although the clinical outcome was not different between the two types, the clinical findings were affected by the spread of CMPI. We are the first to explore how the alteration of the tumor-promoting microenvironment, along with tumor progression, contributes to the development of clinical findings.

The clinical characteristics of colonic pseudo-obstruction and the factors associated with medical treatment response: a study based on a multicenter database in Korea.

Colonic pseudo-obstruction (CPO) is defined as marked colonic distension in the absence of mechanical obstruction. We aimed to investigate the clinical characteristics of CPO and the factors associated with the response to medical treatment by using a multicenter database in Korea. CPO was diagnosed as colonic dilatation without mechanical obstruction by using radiologic and/or endoscopic examinations. Acute CPO occurring in the postoperative period in surgical patients or as a response to an acute illness was excluded. CPO cases were identified in 15 tertiary referral hospitals between 2000 and 2011. The patients' data were retrospectively reviewed and analyzed. In total, 104 patients (53 men; mean age at diagnosis, 47 yr) were identified. Seventy-seven of 104 patients (74%) showed a transition zone on abdominal computed tomography. Sixty of 104 patients (58%) showed poor responses to medical treatment and underwent surgery at the mean follow-up of 7.4 months (0.5-61 months). Younger age at the time of diagnosis, abdominal distension as a chief complaint, and greater cecal diameter were independently associated with the poor responses to medical treatment. These may be risk factors for a poor response to medical treatment.

Varicella-zoster virus (VZV) infection as a possible cause of Ogilvie's syndrome in an immunocompromised host.

We describe an immunodeficient adult with Ogilvie's syndrome preceding a disseminated papulovesicular skin rash in whom varicella-zoster virus infection was demonstrated by PCR assay in cutaneous and colonic biopsy specimens. In view of the significant morbidity and mortality that this condition carries, early and accurate molecular diagnosis and timely treatment are strongly recommended.

Hand-assisted laparoscopic subtotal colectomy with cecorectal anastomosis for chronic idiopathic colonic pseudo-obstruction: report of a case.

Chronic idiopathic colonic pseudo-obstruction (CICP) is characterized by the chronic disturbance of colonic motility without mechanical obstruction, any underlying disease or medication. Currently, there are no established medical treatments for CICP. A 62-year-old female who had undergone right hemicolectomy for splenic flexure syndrome caused by idiopathic megacolon was referred to our hospital with relapse, experiencing palpitation and abdominal fullness. She was diagnosed with CICP according to findings of marked dilation of the colon without mechanical obstruction, dilation of other parts of the gastrointestinal tract, or underlying disease. The dilated colon was surgically removed by hand-assisted laparoscopic subtotal colectomy, followed by cecorectal anastomosis. Histopathologically, there was no degeneration or lack of ganglion cells in Auerbach's plexus. The patient has experienced no severe symptoms after undergoing the present operation.

Chronic intestinal pseudo-obstruction in a horse: a case of myenteric ganglionitis.

An 11-year-old Quarter horse mare was presented for recurrent episodes of colic. A chronic intestinal pseudo-obstruction was diagnosed. Medical treatment and surgical resection of the colon were performed but the condition did not improve and the horse was euthanized. Histopathological examination revealed a myenteric ganglionitis of the small intestine and ascending colon.

Colonic pseudo-obstruction with distinct transitional zone in adult constipation patients: pathological analysis and results of surgical treatment.

There are subsets of chronic constipation patients showing features of colonic pseudo-obstruction (CPO) with distinct transitional zone (TZ). We intended to analyze the clinicopathologic characteristics and surgical outcomes of these patients. Twenty-five consecutive patients who underwent surgery for constipation over the 9-year period were analyzed. TZ (+) group was defined as patients showing symptoms or signs of large bowel obstruction with dilated proximal and collapsed distal colon around the TZ at the time of operation, but without any evidence of mechanical causes of obstruction. Nineteen (76%) patients had features of CPO with TZ. All TZs were located in the left colon. Pathologically, segmental hypoganglionosis was identified at the TZ in all TZ (+) patients. On the other hand, pathologic diagnosis was intestinal neuronal dysplasia type B in the remaining six (24%) patients having a uniform colon diameter without demonstrable dilatations (TZ (-) group). Among TZ (+) patients, 17 (90%) underwent total colectomy with ileorectal anastomosis and two (10%) underwent enterostomy. Long-term follow-up (median 56 months) showed no recurrence of constipation in this group of patients. All six TZ (-) patients underwent total colectomy with ileorectal anastomosis and two (33%) of them had persistent symptoms of constipation on long-term follow-up (median 60 months). In a subset of adult constipation patients presenting with features of CPO with TZ, segmental hypoganglionosis was the final pathologic diagnosis. Constipation patients with features of CPO with distinct TZ in the left colon are expected to benefit from surgical intervention.