Idiopathic megacolon and megarectum in an adult treated with laparoscopic modified Duhamel procedure

Idiopathic megacolon (IMC) and idiopathic megarectum (IMR) describe an abnormality of the colon or rectum, characterised by a permanent dilatation of the bowel diameter in the absence of an identifiable cause. We present a 23-year-old woman with chronic constipation and excessive straining during defecation who presented at the emergency department in partial gut obstruction with a palpable fecaloma. Manual faecal disimpaction and a sigmoid loop colostomy was initially done. A full thickness rectal biopsy was positive for ganglion cells. Further workup led to the diagnosis of chronic IMC and IMR. The patient underwent laparoscopic modified Duhamel procedure, with an uneventful postoperative course.

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Duhamel Procedure untuk Hirschsprung’s Disease Anak di RS Syaiful Anwar Malang

KELUWIH: Jurnal Kesehatan dan Kedokteran ◽

10.24123/kesdok.v2i2.2532 ◽

2021 ◽

Vol 2 (2) ◽

pp. 91-95

Author(s):

Herry Wibowo

Keyword(s):

Barium Enema ◽

Anterior Resection ◽

Low Anterior Resection ◽

Hirschsprung's Disease ◽

Hirschsprung’S Disease ◽

Rectal Biopsy ◽

Full Thickness ◽

Duhamel Procedure ◽

Pull Through ◽

Age Range

Abstract— The diagnosis of Hirschprung's disease is made with barium enema and rectal biopsy in full thickness. The Duhamel procedure was performed in 8 cases in 2008 for cases of Hirschsprung's disease. Age range of patients 7 months to 11 years. The average body weight when operated on is 7-21 kg. Morbidity and mortality after surgery were not launched. Anorectal myectomy with low anterior resection, the Duhamel-Martin procedure, and the Soave pull-through endorectal procedure are the most acceptable methods for surgical management. Hirschsprung's disease was treated in Syaiful Anwar Hospital Malang in 2008 with the following data: 1 person in January (1 year), 1 person in February (6 years), 1 person in March (7 years), 3 people in April ( 7 months, 4 years and 2 years), 2 people in August 2008 (7 months and 11 years). Enlarged abdomen with bloating, repeated constipation and sometimes aborted. Withdrawal procedures that work with endorectal withdrawal procedures, all show long results Keywords: duhamel technique, hirschsprung's disease, constipation, infant Abstrak— Diagnosis penyakit Hirschprung dibuat dengan barium enema dan full-thickness rectal biopsy. Duhamel procedure telah dilakukan pada 8 kasus pada tahun 2008 untuk kasus Hirschsprung's disease. Rentang usia penderita 7 bulan hingga 11 tahun. Berat badan rata – rata saat dioperasi 7 – 21 kg. Morbiditas dan mortalitas setelah operasi tidak dilaporkan. Anorectal myectomy dengan low anterior resection, Duhamel-Martin procedure, dan Soave endorectal pull-through procedure adalah metode yang paling dapat diterima untuk penatalaksanaan bedah. Didapatkan Kasus penyakit Hirschsprung's yang berobat di Rumah Sakit Syaiful Anwar Malang selama tahun 2008 dengan data sebagai berikut yaitu 1 orang di Januari (1 tahun) , 1 orang di Februari (6 tahun), 1 orang Maret (7 bulan), 3 orang April (7 bulan, 4 tahun dan 2 tahun), 2 orang Agustus 2008 (7 bulan dan 11 tahun). Tiap penderita mengalami riwayat abdominal distention dengan gejala perut kembung berulang, konstipasi dan kadang –kadang disertai mual. Delapan penderita yang menjalani operasi dengan prosedur endorectal pullthrough , semuanya menunjukkan hasil jangka panjang yang memuaskan. Kata kunci: tehnik duhamel, penyakit hirschsprung's , konstipasi, infant

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Image of the Month: Decision-Making in Surgery for Late Onset Hirschsprung Disease

European Journal of Pediatric Surgery Reports ◽

10.1055/s-0040-1721049 ◽

2020 ◽

Vol 08 (01) ◽

pp. e99-e101

Author(s):

Anisha Apte ◽

Elise McKenna ◽

Marc A. Levitt

Keyword(s):

Decision Making ◽

Chronic Constipation ◽

Anorectal Manometry ◽

Late Onset ◽

Hirschsprung Disease ◽

Rectal Biopsy ◽

Poor Growth

AbstractWe present a case of a 14-year-old boy with chronic distension, poor growth, and chronic constipation. He undergoes anorectal manometry and rectal biopsy, confirming the diagnosis of Hirschsprung disease (HD). The case is presented with a key image and associated questions to prompt discussion on strategies for management and treatment of HD in late-diagnosed children.

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Lactobacillus Reuteri DSM 17938 (Limosilactobacillus reuteri) in Diarrhea and Constipation: Two Sides of the Same Coin?

Medicina ◽

10.3390/medicina57070643 ◽

2021 ◽

Vol 57 (7) ◽

pp. 643

Author(s):

Angela Saviano ◽

Mattia Brigida ◽

Alessio Migneco ◽

Gayani Gunawardena ◽

Christian Zanza ◽

...

Keyword(s):

Inflammatory Bowel Disease ◽

Emergency Department ◽

Abdominal Pain ◽

Human Health ◽

Bowel Disease ◽

Chronic Constipation ◽

Lactobacillus Reuteri ◽

Current Knowledge ◽

Gastrointestinal Symptoms ◽

Inflammatory Bowel

Background and Objectives: Lactobacillus reuteri DSM 17938 (L. reuteri) is a probiotic that can colonize different human body sites, including primarily the gastrointestinal tract, but also the urinary tract, the skin, and breast milk. Literature data showed that the administration of L. reuteri can be beneficial to human health. The aim of this review was to summarize current knowledge on the role of L. reuteri in the management of gastrointestinal symptoms, abdominal pain, diarrhea and constipation, both in adults and children, which are frequent reasons for admission to the emergency department (ED), in order to promote the best selection of probiotic type in the treatment of these uncomfortable and common symptoms. Materials and Methods: We searched articles on PubMed® from January 2011 to January 2021. Results: Numerous clinical studies suggested that L. reuteri may be helpful in modulating gut microbiota, eliminating infections, and attenuating the gastrointestinal symptoms of enteric colitis, antibiotic-associated diarrhea (also related to the treatment of Helicobacter pylori (HP) infection), irritable bowel syndrome, inflammatory bowel disease, and chronic constipation. In both children and in adults, L. reuteri shortens the duration of acute infectious diarrhea and improves abdominal pain in patients with colitis or inflammatory bowel disease. It can ameliorate dyspepsia and symptoms of gastritis in patients with HP infection. Moreover, it improves gut motility and chronic constipation. Conclusion: Currently, probiotics are widely used to prevent and treat numerous gastrointestinal disorders. In our opinion, L. reuteri meets all the requirements to be considered a safe, well-tolerated, and efficacious probiotic that is able to contribute to the beneficial effects on gut-human health, preventing and treating many gastrointestinal symptoms, and speeding up the recovery and discharge of patients accessing the emergency department.

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Autopsy Study of Calretinin Immunohistochemistry in the Anorectal Canal in Young Infants and Potential Implications for Rectal Biopsy Approach in the Neonatal Period

Pediatric and Developmental Pathology ◽

10.1177/10935266211030172 ◽

2021 ◽

pp. 109352662110301

Author(s):

Heather Rytting ◽

Zachary J Dureau ◽

Jose Velazquez Vega ◽

Beverly B Rogers ◽

Hong Yin

Keyword(s):

Neonatal Period ◽

Ganglion Cells ◽

Hirschsprung Disease ◽

Rectal Biopsy ◽

Autopsy Study ◽

Pathologic Diagnosis ◽

Young Infants ◽

Autopsy Specimens ◽

Pectinate Line ◽

Age Range

Background Absent submucosal ganglion cells in biopsies 1-3 cm above the pectinate line establishes the pathologic diagnosis of Hirschsprung Disease (HD). Calretinin stains both ganglion cells and their mucosal neurites and has gained importance in HD diagnosis. Absent calretinin positive mucosal neurites in biopsies at the appropriate level above the pectinate line is highly specific for HD. Whether this applies to lower biopsies is uncertain. To address this, we studied anorectal canal autopsy specimens from infants. Methods We performed an autopsy study of infant anorectal canal specimens to describe calretinin staining in this region. Calretinin staining was correlated with histologic and gross landmarks. Results In all 15 non-HD specimens, calretinin positive mucosal neurites were present in glandular mucosa up to the anorectal line where neurites rapidly diminished. Age range was preterm 26 weeks to 3 months. Conclusions Calretinin positive mucosal neurites are present in glandular mucosa up to the anorectal line in young infants. This is potentially important regarding neonatal HD biopsy level and diagnosis. Positive calretinin staining at the anorectal line favors normal innervation making HD unlikely. Absent calretinin positive neurites in glandular mucosa is worrisome for HD in young infants, regardless of location.

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Total Colonic Aganglionosis: Case Report, Practical Diagnostic Approach and Pitfalls

Archives of Pathology & Laboratory Medicine ◽

10.5858/2010-0279-cr.1 ◽

2010 ◽

Vol 134 (10) ◽

pp. 1467-1473 ◽

Cited By ~ 3

Author(s):

Raja Rabah

Keyword(s):

Transition Zone ◽

Ganglion Cells ◽

Hirschsprung Disease ◽

Rectal Biopsy ◽

Nerve Fibers ◽

Submucosal Plexus ◽

Total Colonic Aganglionosis ◽

Biopsy Specimens ◽

Suction Rectal Biopsy ◽

Acetylcholinesterase Staining

Abstract Hirschsprung disease remains a challenging diagnosis for many pathologists. The disease is characterized by a lack of ganglion cells in the myenteric and submucosal plexus, associated with increased numbers of acetylcholinesterase-positive nerve fibers. Hypertrophic nerve fibers are present in most but not all patients. Total colonic aganglionosis (TCA) is an uncommon form of Hirschsprung disease with clinical, histologic, and genetic differences and is even more difficult to diagnose and manage. This case illustrates some of the difficulties frequently faced by the pathologists dealing with total colonic aganglionosis. Suction rectal biopsy specimens often lack significant nerve hypertrophy and positive acetylcholinesterase staining, which aid in the diagnosis. Pathologists have to depend mainly on the lack of ganglion cells in adequate submucosa to establish the diagnosis. Transition zone is often long in total colonic aganglionosis and interpretation of frozen sections can be difficult. The presence of several uniformly distributed clusters of mature ganglion cells and lack of nerve hypertrophy are required to avoid connections at the transition zone.

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Megacystis-Microcolon-Intestinal Hypoperistalsis Syndrome

PEDIATRICS ◽

10.1542/peds.63.5.805 ◽

1979 ◽

Vol 63 (5) ◽

pp. 805-808

Author(s):

Thomas E. Wiswell ◽

James S. Rawlings ◽

James L. Wilson ◽

Gary Pettett

Keyword(s):

Neonatal Period ◽

Ganglion Cells ◽

Plain Film ◽

Rectal Biopsy ◽

Exploratory Laparotomy ◽

Nerve Fibers ◽

Bladder Obstruction ◽

Distended Abdomen ◽

Biopsy Specimens ◽

Hypoperistalsis Syndrome

The syndrome of megacystis-microcolon-intestinal hypoperistalsis has been reported in a total of seven female infants. Massive abdominal distention secondary to a distended urinary Fig 1. Plain film of markedly distended abdomen with no air beyond stomach. Stomach is displaced upward toward left. der was the major presenting characteristic. Iutestinal hypoperistalsis, apparent in the early neonatal period, persists without improvement. Exploratory laparotomy reveals malrotation and malfixation of a small microcolon. No anatomic cause of intestinal or bladder obstruction can be found. Intestinal and/or rectal biopsy specimens contain abundant ganglion cells and nerve fibers. The outcome has been uniformly fatal, with survival in the reported cases ranging from two days to 34 months.

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Pullthrough Operation for Hirschsprung's Disease: Importance of a Circumferential (Donut) Biopsy at the Level of the Anastomosis

European Journal of Pediatric Surgery Reports ◽

10.1055/s-0039-1693494 ◽

2019 ◽

Vol 07 (01) ◽

pp. e55-e57

Author(s):

Susan Jehangir ◽

Soundappan Venkatraman Sannappa Soundappan ◽

Micheal Krivanek ◽

Susan Arbuckle ◽

Nicole Graf

Keyword(s):

Transition Zone ◽

Transverse Colon ◽

Hirschsprung's Disease ◽

Hirschsprung’S Disease ◽

Ganglion Cells ◽

Distal Colon ◽

Dentate Line ◽

Full Thickness ◽

Mesenteric Border ◽

Antimesenteric Border

AbstractHirschsprung's disease is characterized by the absence of ganglia in the distal colon, resulting in a functional obstruction. It is managed by excision of the aganglionic segment and anastomosis of the ganglionated bowel just above the dentate line. The level of aganglionosis is determined by performing multiple seromuscular biopsies and/or full thickness biopsy on the antimesenteric border of the bowel to determine the level of pullthrough. The transition zone is described as being irregular, and hence a doughnut biopsy is recommended so that the complete circumference can be assessed. Herein, we described a child in whom there was a selective absence of ganglion cells in 30% of the circumference of the bowel along the mesenteric border for most of the transverse colon. This case defies the known concept of neural migration in an intramural and transmesenteric fashion and emphasizes the importance of a doughnut biopsy of the pulled-down segment.

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