Intestinal Neuronal Dysplasia Type B: Timely Diagnosis and Management

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S62-S63
Author(s):  
Mona Deerwester ◽  
Steven Drexler
Keyword(s):  
Abdominal Pain ◽  
Diagnostic Criteria ◽  
Ganglion Cells ◽  
Total Colectomy ◽  
Nerve Fibers ◽  
Intestinal Neuronal Dysplasia ◽  
Timely Diagnosis ◽  
Distended Bowel ◽  
History Of ◽  
Neuronal Dysplasia

Abstract A 23-year-old female presented to the emergency department with abdominal pain and constipation. She reported an extensive history of constipation. Imaging showed distended bowel without an obstruction. During laparotomy, no obvious mechanical cause was found and a total colectomy was performed. Gross examination of the colectomy specimen showed cobblestoning in a 10-cm portion of the colon. Microscopic examination demonstrated hypoganglionosis of the myenteric plexus, hyperganglionosis of Auerbach’s plexus, and “giant ganglia.” This case met the 2006 Meier-Ruge criteria and diagnosis of intestinal neuronal dysplasia (IND) was established. IND was first described in 1971. The frequency of IND varies widely due to lack of consensus of diagnostic criteria and has a geographic distribution with the highest rates in Europe, which correlates to published research in this region. Diagnostic criteria are controversial and require standardization. Meier-Ruge suggests a quantitative analysis of the number of ganglion cells in the submucosal plexuses and the identification of at least 20% giant ganglia with at least 8 neurons each, in 25 analyzed ganglia. More recent diagnostic criteria are conservative with differences, including (1) elimination of increased AChE-positive nerve fibers around submucosal blood vessels, (2) stipulation that a giant ganglion contains more than 8 ganglion cells, (3) the requirement that more than 20% of at least 25 ganglia be giant ganglia, and (4) diagnostic exclusion of patients <1 year. Clinical management is also controversial. Schimpl et al reported satisfactory results in 80% of 105 patients treated with dietary changes, cisapride, and laxatives with a median 7.2 years follow-up. Since colonic peristalsis is impaired by dysganglionosis, subtotal colectomy procedures have been widely successful. Clinicians should be mindful of IND in patients with a history of chronic constipation with abdominal pain and nonspecific imaging, as timely diagnosis can spare the patient from total colectomy and improve quality of life.

scholarly journals Clozapine-induced stercoral colitis: a surgical perspective

2019 ◽  
Vol 12 (8) ◽  
pp. e227718 ◽  
Author(s):  
Jayan George ◽  
Richard Hotham ◽  
William Melton ◽  
Keith Chapple
Keyword(s):  
Abdominal Pain ◽  
Ct Scan ◽  
Total Colectomy ◽  
Postoperative Recovery ◽  
Presenting Symptoms ◽  
History Of

We describe a case of a 46-year-old man with schizophrenia treated with clozapine who presented as an emergency with abdominal pain on the background of a 1 month history of constipation. The initial presenting symptoms were vague and a diagnosis was difficult to establish. Initial CT of the abdomen and pelvis demonstrated only minor abnormalities. He continued to deteriorate until a further CT scan revealed worsening stercoral colitis. He subsequently underwent an emergency total colectomy and ileostomy formation and had a complicated prolonged postoperative recovery. This case highlights the risks that clozapine can have on slowing bowel transit and the dangerous consequences that can occur if not identified early.

scholarly journals A case of single incision laparoscopic total colectomy for intestinal neuronal dysplasia type B

2017 ◽  
Vol 38 ◽  
pp. 122-127 ◽  
Author(s):  
Taro Masuda ◽  
Takashi Nonaka ◽  
Toshiyuki Adachi ◽  
Makoto Hisanaga ◽  
Shigeki Nagayoshi ◽  
...  
Keyword(s):  
Single Incision ◽  
Total Colectomy ◽  
Intestinal Neuronal Dysplasia ◽  
Type B ◽  
Neuronal Dysplasia

scholarly journals A case of severe megacolon due to acquired isolated hypoganglionosis after low anterior resection for lower rectal cancer

2019 ◽  
Vol 13 (3) ◽  
pp. 328-333
Author(s):  
Tetsuro Tominaga ◽  
Satoshi Nagayama ◽  
Manabu Takamatsu ◽  
Shun Miyanari ◽  
Toshiya Nagasaki ◽  
...  
Keyword(s):  
Rectal Cancer ◽  
Anterior Resection ◽  
Low Anterior Resection ◽  
Ganglion Cells ◽  
Total Colectomy ◽  
Locally Advanced ◽  
Nerve Fibers ◽  
Lower Rectal Cancer ◽  
Stoma Closure ◽  
Isolated Hypoganglionosis

AbstractAcquired isolated hypoganglionosis is a rare intestinal neurological disease, which presents in adulthood with the clinical symptoms of chronic constipation. A 39-year-old man underwent laparoscopic low anterior resection and covering ileostomy for locally advanced-rectal cancer. A 6-month course of postoperative adjuvant chemotherapy was completed, followed by closure of the ileostoma. After the closure, he developed severe colitis which required 1-month of hospitalization. Mucosal erosions and pseudo-membrane formation were evident on colonoscopy and severe mucosal damage characterized by infiltration of inflammatory cells and crypt degeneration were pathologically confirmed. Even after the remission of the colitis, he suffered from severe constipation and distention. At 4 years after the stoma closure, he decided to undergo laparoscopic total colectomy. Histopathologically, the nerve fibers and ganglion cells became gradually scarcer from the non-dilated to dilated regions. Immunohistochemical staining examination confirmed that the ganglion cells gradually decreased and became degenerated from the normal to dilated region, thereby arriving at the final diagnosis of isolated hypoganglionosis. The patient recovered without any complications and there has been no evidence of any relapse of the symptoms. We present a case of acquired isolated hypoganglionosis-related megacolon, which required laparoscopic total colectomy, due to severe enterocolitis following stoma closure.

scholarly journals Challenges in the diagnosis of intestinal neuronal dysplasia type B: A look beyond the number of ganglion cells

2021 ◽  
Vol 27 (44) ◽  
pp. 7649-7660
Author(s):  
Simone Antunes Terra ◽  
Anderson Cesar Gonçalves ◽  
Pedro Luiz Toledo de Arruda Lourenção ◽  
Maria Aparecida Marchesan Rodrigues
Keyword(s):  
Ganglion Cells ◽  
Intestinal Neuronal Dysplasia ◽  
Type B ◽  
Neuronal Dysplasia

scholarly journals Intestinal Neuronal Dysplasia Type B: An Updated Review of a Problematic Diagnosis

2018 ◽  
Vol 143 (2) ◽  
pp. 235-243 ◽  
Author(s):  
Raj P. Kapur ◽  
Miguel Reyes-Mugica
Keyword(s):  
Diagnostic Criteria ◽  
Large Body ◽  
Hirschsprung Disease ◽  
Scientific Basis ◽  
Intestinal Neuronal Dysplasia ◽  
Published Data ◽  
Supporting Evidence ◽  
Type B ◽  
Intestinal Dysmotility ◽  
Neuronal Dysplasia

Context.— Intestinal neuronal dysplasia type B (IND B) is a controversial histopathologic phenotype that has been associated with intestinal dysmotility, either as an isolated condition or in conjunction with established pathologic disorders (eg, Hirschsprung disease). Many factors contribute to the debate over the existence and/or clinical significance of IND B, including a large body of published data based on inconsistent diagnostic criteria and methods, which have fostered many unwarranted conclusions that lack sufficient scientific basis. Objective.— To critically analyze existing published data regarding IND B to provide supporting evidence-based diagnostic practice and to stimulate necessary and scientifically sound research. Data Sources.— This update focuses on published literature related to the pathology of IND B because without a reliable pathologic diagnosis, studies of epidemiology, pathogenesis, natural history, management, and outcome are all suspect. Problems with existing data are identified explicitly with suggestions as to how future investigations should be designed and evaluated to better understand this entity. Conclusions.— Inconsistencies in diagnostic criteria and methods used to define IND B justifiably encumber the universal acceptance of IND B as a neuropathologic etiology for intestinal dysmotility. IND B will remain a controversial diagnosis until rigorous, well-controlled scientific studies are conducted to establish reproducible and reliable diagnostic criteria that reliably translate from one laboratory to another.

Hypercellular/Heterotopic Ganglia in Omphalomesenteric Duct Remnants

2001 ◽  
Vol 4 (3) ◽  
pp. 246-251
Author(s):  
Sandy S. Wu ◽  
Susan Coventry ◽  
Jean-Pierre de Chadarevian ◽  
Margaret H. Collins
Keyword(s):  
Acute Abdomen ◽  
Bowel Obstruction ◽  
Ganglion Cells ◽  
Pancreatic Tissue ◽  
Intestinal Neuronal Dysplasia ◽  
Muscularis Mucosa ◽  
Type B ◽  
Omphalomesenteric Duct ◽  
Neuronal Dysplasia ◽  
Similar Incidence

Omphalomesenteric duct remnants (ODR) including Meckel diverticula often present with symptoms of bowel obstruction. Their histologic features are varied and include heterotopic gastrointestinal mucosa and/or pancreatic tissue within the wall. Abnormalities of the submucosal plexus of Meissner, however, have not been documented in the literature. Thus, we have examined a number of ODR for evidence of ganglion abnormalities. Fifty-three cases of ODR were retrieved from our archives, along with 25 nonduodenal small bowel control specimens obtained from autopsies of patients without clinical or pathologic evidence of enteropathy. Histologic criteria for the diagnosis of abnormal hypercellular/heterotopic ganglia (HHG) in ODR were defined as a single submucosal ganglion/plexus containing >10 neurons, or >5 submucosal ganglions per high-power (X40) field, or heterotopic ganglion cells within the muscularis mucosa or the lamina propria. HHG, histologically indistinguishable from intestinal neuronal dysplasia type B, were found in more than half of the ODR (ODR: 51.9%, n = 50 vs. control: 4%, n = 25, P = 3.6 X 10−6), particularly those excised for obstructive complications (ODR with acute abdomen: 65.7%, n = 35, vs. ODR without acute abdomen: 33.3%, n = 15, P = 0.035). HHG were present in equal numbers of inflamed and noninflamed ODR (inflamed: 53.6%, n = 28, vs. noninflamed: 59.1%, n = 22, P = 0.70). A similar incidence was found in ODR with heterotopia and without (with heterotopia: 61.1%, n = 18, vs. without: 53.1%, n = 32, P = 0.59). ODR frequently contained HHG histomorphologically similar to those found in intestinal neuronal dysplasia, type B (IND-B). The overrepresentation of HHG in symptomatic ODR patients suggests an association with bowel obstruction. The data did not demonstrate a relationship with either inflammation or heterotopia.

scholarly journals Isolated Intestinal Neuronal Dysplasia- Type B of Ileum: a Rare Occurrence

2021 ◽  
Author(s):  
Ram Nawal Rao ◽  
Pratishtha Sengar
Keyword(s):  
Ganglion Cells ◽  
Hirschsprung Disease ◽  
Exploratory Laparotomy ◽  
Distal Colon ◽  
Intestinal Neuronal Dysplasia ◽  
Rare Occurrence ◽  
Type B ◽  
Frequent Site ◽  
Intestinal Pseudo Obstruction ◽  
Neuronal Dysplasia

Abstract Background: Intestinal neuronal dysplasia type B in the gastrointestinal tract is a rare occurrence and may occur alone or in combination with Hirschsprung disease. Distal colon seems to be frequent site for isolated IND-B cases, however small bowel involvement is scarcely reported. Case presentation: We report a case of 9 years old boy presenting with features of intestinal pseudo-obstruction for 5 years. Exploratory laparotomy revealed narrowed distal ileum with huge proximal dilatation. Histopathology of the resected terminal ileum revealed giant submucosal ganglion, hyperplastic submucosal nerves and ectopic ganglion cells in the lamina propria suggestive of IND-B.Conclusions: Although IND-B involving ileum is a rare occurrence, suspicion should be kept in cases of intestinal obstruction with minimal response to conventional treatment.

Conversion Reactions in Adolescents

1987 ◽  
Vol 8 (9) ◽  
pp. 279-286
Author(s):  
Gregory Prazar
Keyword(s):  
Abdominal Pain ◽  
Physical Examination ◽  
Physical Symptom ◽  
Secondary Gain ◽  
Conversion Reaction ◽  
Mental Symptom ◽  
Timely Diagnosis ◽  
History Of ◽  
The Individual ◽  
Minute Walk

Few situations in practice present as more frustrating for a busy pediatrician than an adolescent who appears for a ten-minute walk-in visit with a prolonged history of unexplained abdominal pain or headache. Obtaining a brief history of the symptom may contribute little to a timely diagnosis, and physical examination is often unrewarding in understanding the symptom. If the adolescent's demeanor suggests little discomfort, the pediatrician is often left with the impression that the pain is "psychosomatic" and/or that the adolescent is exploiting a physical symptom for some secondary gain. A diagnosis of conversion reaction is seldom considered. The focus of this article will be to aid the pediatrician in identifying conversion reactions, differentiating conversion reactions from other disorders (eg, hypochondriasis, malingering), and outlining an approach to the treatment and referral of patients with conversion reactions. DESCRIPTION AND PREVALENCE Conversion reactions represent a form of communication of the uncomfortable, or as Engel1 writes: "a psychic mechanism whereby an idea, fantasy, or wish is expressed in bodily rather than in verbal terms and is experienced by the patient as a physical symptom rather than as a mental symptom." The idea or wish is psychologically threatening to the individual or unacceptable for him or her to express directly.

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