A Case Report on 2 years Child: Hirschprung’s Disease

Introduction: Hirschsprung's disease is the most common cause of large intestinal obstructing in neonates. Hirschsprung's disease is a congenital anomaly caused by migratory failure of neural crest cells leading to abnormal innervations of the bowel .The defect begins in the internal and sphincter and extends proximally for a variable length of gut. Hirschprung's disease is a disorder of the gut caused due to congenital absence of ganglion cells in the sub-mucosal and myenteric plexus of intestine. This disease is also known as megacolon or congenital Aganglionic megacolon. Case presentation: A 2 years old male child, known case of Hirschprung’s disease, was brought to AVBRH for further management. As narrated by the mother, the child was not passing stool since birth. There was no history of abdominal distention, vomiting or fever. The child pass the stool with the help of enema which was given to the child in the morning and evening. Mast. Devansh has past history of NICU stay. In View of not passing stools, USG of abdomen and pelvis done. Endorectal pull through surgery was done on 4/02/2021 under general anesthesia. No any past surgical history. There is a past history of rectal biopsy done in 1 1/2 month of age. Conclusion:The patient was admitted in AVBRH for further management. Then the report mainly focused on surgical management and quality nursing care due to which patient was discharged without any further complication and satisfaction.

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Hirschsprung’s Disease: Diagnosis and Management

Journal of Enam Medical College ◽

10.3329/jemc.v10i2.53536 ◽

2021 ◽

Vol 10 (2) ◽

pp. 104-113

Author(s):

Md Benzamin ◽

Md Rukunuzzaman ◽

Md Wahiduzzaman Mazumder ◽

ASM Bazlul Karim

Keyword(s):

Hirschsprung's Disease ◽

Hirschsprung’S Disease ◽

Rectal Biopsy ◽

Disease Diagnosis ◽

Radiological Investigation ◽

Functional Intestinal Obstruction ◽

The Common ◽

High Index Of Suspicion ◽

Hirschprung's Disease ◽

Related Mortality

Hirschsprung’s disease (HD) is a rare genetic congenital defect of intestine causing failure of migration of parasympathetic ganglionic cells in some definite part of intestine, resulting in functional intestinal obstruction. It commonly involves rectosigmoid region of colon but other parts of colon or total colon, even small intestine may be affected. Incidence is 1/5000 live births. It is one of the common pediatric surgical problems and 2nd most organic cause of constipation. Symptoms may be evident from 1st day of life. About 90% infants with Hirschprung’s disease fail to pass meconium in 1st 24 hours of life. About 80% HD cases are diagnosed in early few months of life and present with abdominal distention, constipation, poor feeding, vomiting etc. HD enterocolitis is a devastating condition related to mortality. HD may be associated with some congenital anomalies and syndrome. High index of suspicion is the main key to diagnosis. Radiological investigation supports the diagnosis and rectal biopsy for histopathology is confirmatory. Although it is a surgical problem, physician can play a key role in early diagnosis and thus help to prevent HD enterocolitis-related mortality and restore near-normal life. J Enam Med Col 2020; 10(2): 104-113

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Hirschsprung's disease

10.1093/med/9780198569862.003.0039 ◽

2011 ◽

Author(s):

R. Mark Beattie ◽

Anil Dhawan ◽

John W.L. Puntis

Keyword(s):

Myenteric Plexus ◽

High Frequency ◽

Hirschsprung's Disease ◽

Hirschsprung’S Disease ◽

Congenital Abnormalities ◽

Ganglion Cells ◽

Chromosome 10 ◽

Neuronal Intestinal Dysplasia ◽

Intestinal Pseudo Obstruction ◽

Hirschprung's Disease

Hirschprung's disease 280Neuronal intestinal dysplasia 281Intestinal pseudo-obstruction 281Hirschsprung's disease is the absence of ganglion cells in the myenteric plexus of the most distal bowel. Presentation is with constipation. Incidence is 1 in 5000. Long-segment Hirschsprung's disease is familial, with equal sex incidence. The gene is on chromosome 10. It is associated with Down's syndrome and there is a high frequency of other congenital abnormalities....

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Clinicopathological Characteristics of Hirschsprung’s Disease With Emphasis on Diagnosis and Management: A Single-Center Study in the Kingdom of Saudi Arabia

Global Pediatric Health ◽

10.1177/2333794x19848865 ◽

2019 ◽

Vol 6 ◽

pp. 2333794X1984886

Author(s):

Abdulaziz Howsawi ◽

Hanaa Bamefleh ◽

Saud Al Jadaan ◽

Stanley Crankson ◽

Rakan Alkhilaiwi ◽

...

Keyword(s):

Saudi Arabia ◽

Hirschsprung's Disease ◽

Hirschsprung’S Disease ◽

Neonatal Period ◽

Ganglion Cells ◽

Care Center ◽

Tertiary Care ◽

Motor Disorder ◽

Permanent Section ◽

Pull Through

Introduction: Hirschsprung’s Disease (HD) is a motor disorder of the gut caused by the failure of neural crest cells to migrate craniocaudally into the bowel during intestinal development, resulting in a functional obstruction. The majority of patients with HD are diagnosed in the neonatal period when they present with symptoms of distal intestinal obstruction. Aim: This study aims to identify the clinic-pathological characteristic of HD patients in our institution in KSA and comparing it with local and international data. Materials and Methods: This retrospective cohort study was conducted in King Abdulaziz Medical City (KAMC), a tertiary care center in Riyadh, Kingdome of Saudi Arabia (KSA). Results: A total of 54 patients (72% male) were diagnosed with HD. Forty-eight patients (89%) were born at term, and 6 were pre-term. Sixty-three percent of the patients presented in the neonatal period. Twenty-two patients (41%) underwent one-stage endorectal pull-through procedure, 23 patients (43%) two-stage endorectal pull-through, and 9 patients (16%) had three-stage endorectal pull-through. Five out of 54 patients had ganglion cells seen on FS but were absent in the permanent section. Therefore, the concordance rate was 90.8%. Conclusion: FS biopsy is a necessary method to determine the level of aganglionosis intraoperatively in HD, but the definitive diagnosis should be with permanent section. Also, the choice of surgical operation type (single-stage or multi-stage pull-through) depends on the patient’s clinical condition.

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Rectal Biopsy as an Aid in the Diagnosis of Hirschsprung's Disease

PEDIATRICS ◽

10.1542/peds.18.2.176 ◽

1956 ◽

Vol 18 (2) ◽

pp. 176-176

Keyword(s):

Chronic Constipation ◽

Hirschsprung's Disease ◽

Hirschsprung’S Disease ◽

Rectal Wall ◽

Rectal Biopsy ◽

Distal Segment ◽

Large Bowel Obstruction ◽

Atypical Symptoms ◽

Typical History ◽

History Of

The child with a typical history of chronic constipation, and with physical signs of a distended abdomen and an empty rectal ampulla, who is found to have a widely dilated colon with a narrow distal segment by the radiologist, presents no diagnostic problem. However, the diagnosis of Hirschsprung's disease may be very difficult to establish if part of the classical diagnostic criteria are absent or inconclusive. In such cases, the authors have found rectal biopsy to be a useful diagnostic procedure. They cite as indications for biopsy: (1) suspected Hirschsprung's disease in the newborn infant, because the colon does not become dilated and hypertrophied until the intestinal tract has been functioning for several months; (2) symptoms of upper large bowel obstruction developing soon after birth, because barium enema studies of infants whose entire colons are aganglionic reveal no obstruction of the lumen of the large intestine; (3) radiologic evidence of dilatation of the entire distal segment of the colon, because, in patients with a short aganglionic lesion, the obstruction may be so low that the radiologist is unable to demonstrate a narrow segment extending through the rectosigmoid, and (4) atypical symptoms of megacolon, including diarrhea, episodes of intestinal obstruction, or other symptoms of colonic dysfunction. This procedure has been performed by Swenson and his colleagues on 40 patients. Biopsy specimens obtained from 19 patients, subsequently proved to have Hirschsprung's disease by study of the resected rectum and sigmoid, contained no ganglionic cells. In the other 21 patients, biopsy was performed because Hirschsprung's disease was suspected. Ganglionic cells were present in all of these specimens, and the success of subsequent conservative management of these patients confirmed the diagnosis of chronic constipation. Control material was obtained from 10 cadavers with normal colons, and ganglionic cells were found in all of these specimens. The authors emphasize that this technique is only as good as the specimen obtained. An adequate biopsy of rectal wall must be removed. Multiple histologic sections must be carefully examined before the diagnosis of Hirschsprung's disease is confirmed.

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TREATMENT OF CONGENITAL MEGACOLON IN 50 INFANTS

PEDIATRICS ◽

10.1542/peds.38.2.185 ◽

1966 ◽

Vol 38 (2) ◽

pp. 185-193

Author(s):

Walton K. T. Shim ◽

Orvar Swenson

Keyword(s):

Hirschsprung's Disease ◽

Hirschsprung’S Disease ◽

Average Weight ◽

Older Children ◽

The Past ◽

History Of ◽

Pull Through ◽

Definitive Surgery ◽

Criteria For Selection ◽

Selection Of

Fifty infants less than 1 year old with Hirschsprung's disease were treated at Children's Memorial Hospital in the past 6 years. Thirty-two of the 35 barium enema studies yielded "diagnostic" roentgenographic studies, and 3 were "negative" for Hirschsprung's disease. Fourteen of the 50 infants had a history of diarrhea. Enterocolitis in infants may be extremely rapid in onset and progression. It should be vigorously treated with rectal irrigations and intravenous fluids. Nineteen patients (38%) had aganglionic segments extending above the rectosigmoid colon, and eight (16%) were aganglionic proximal to the splenic flexure. This includes three patients with aganglionosis of the entire colon and one that was aganglionic from the jejunum to anus. Emphasis is placed on an aggressive combined attack by pediatrician, radiologist, pathologist, and surgeon to diagnose and prepare the sick infant as early as possible for colostomy or definitive abdominal-perineal pull-through operation. Twelve infants with an average weight of 12.5 lb were operated on primarily with no deaths or complications. It is our belief that definitive surgery can be done with greater ease in an infant than in older children, but careful selection and preparation of the patient and meticulous operative technique must be exercised. The important criteria for selection of infants for immediate resection are the absence of emaciation and enterocolitis, although the infant may be small. Twenty-six infants had colostomy and resection with no deaths or complications. Our raw mortality figures show 10 deaths in 50 patients (20%). There were 7 deaths in 47 surgical patients (15%).

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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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Accuracy of Suction Rectal Biopsy for Diagnosis of Hirschsprung's Disease in Neonates

European Journal of Pediatric Surgery ◽

10.1055/s-0038-1667040 ◽

2018 ◽

Vol 29 (05) ◽

pp. 425-430 ◽

Cited By ~ 4

Author(s):

Ashley Rebekah Allen ◽

Angelica R. Putnam ◽

Angela P. Presson ◽

Chelsea McCarty Allen ◽

Douglas C. Barnhart ◽

...

Keyword(s):

Hirschsprung's Disease ◽

Hirschsprung’S Disease ◽

Age Groups ◽

Newborn Infants ◽

Rectal Biopsy ◽

Suction Rectal Biopsy ◽

Postmenstrual Age ◽

Pull Through ◽

One Year

Introduction Recent publications have questioned the sensitivity of suction rectal biopsy (SRB) for diagnosis of Hirschsprung's disease (HD) in newborns. A recent European survey reported that 39% of pediatric surgeons performed full-thickness transanal biopsies due to concerns about the accuracy of SRB. We sought to examine our contemporary SRB experience in infants. Materials and Methods A review was performed (2007–2016) of patients under 6 months of age who had a SRB at our children's hospital. The cohort was subdivided by postmenstrual age at time of SRB: preterm (< 40 weeks, A), term neonate (40–44 weeks, B), and infant (> 44 weeks, C). The pathology reports from endorectal pull-through were used as gold standard confirmation. One-year follow-up of patients with negative SRB was used to confirm accurate diagnosis. Results A total of 153 patients met the criteria and a total of 159 SRBs (< 2,500 g; n = 26) were performed (A = 60, B = 58, C = 35). Forty-three patients were diagnosed with HD (A = 25, B = 15, C = 3). A second SRB was performed in 6 (3.9%) patients due to inadequate tissue (A = 2, B = 2, C = 2) with HD diagnosed in 5. No complications occurred. Sensitivity and specificity of SRB was 100% in all age groups. Half of the patients with a negative SRB had at least 1 year follow-up, with none subsequently diagnosed with HD. Conclusion SRB results in adequate tissue for evaluation of HD in nearly all patients less than 6 months of age on the first attempt and is highly accurate in the preterm and newborn infants. No complications occurred, even among infants less than 2,500 g.

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Hirschsprung’s Disease: A Rare Adult Diagnosis

Clinical Practice and Cases in Emergency Medicine ◽

10.5811/cpcem.2020.6.46492 ◽

2020 ◽

Vol 4 (3) ◽

pp. 480-481

Author(s):

Kaitlyn Schmutz ◽

Gaea McGaig ◽

B. Jason Theiling

Keyword(s):

Computed Tomography ◽

Emergency Department ◽

Young Adult ◽

Chronic Constipation ◽

Hirschsprung's Disease ◽

Hirschsprung’S Disease ◽

Failure To Thrive ◽

Case Presentation ◽

Gut Colonization ◽

History Of

Case Presentation: Approximately 94% of patients with Hirschsprung’s disease (HD) are diagnosed before the age of five. In our case, a young adult with years of constipation presented to the emergency department with significant abdominal distention. He was ultimately diagnosed with HD, which was identified using computed tomography (CT). Discussion: In HD, we find defects in gastric motility due to improper gut colonization. Without childhood recognition, HD often leads to chronic constipation and failure to thrive in adulthood. CT is a key step in identifying this rare adult diagnosis that should be considered in all patients with a history of chronic constipation.

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Is Single-stage Primary Transanal Endorectal Pull through (TEP) feasible for Short Segment Hirschsprung's Disease in Neonates?- Our Experience in a Tertiary Hospital

Journal of Surgical Sciences ◽

10.3329/jss.v18i2.43755 ◽

2019 ◽

Vol 18 (2) ◽

pp. 45-50

Author(s):

Md Shahjahan ◽

Kazi Md Noor ul Ferdous ◽

M Kabirul Lslam

Keyword(s):

Hirschsprung's Disease ◽

Hirschsprung’S Disease ◽

Anastomotic Stricture ◽

Pediatric Anesthesia ◽

Rectal Biopsy ◽

Tertiary Hospital ◽

Single Stage ◽

Short Segment ◽

Pull Through

Background: The surgical management of Hirschsprung's Disease (HD) includes so many procedures. TransanalEndorectal Pull through (TEP) represents the latest development in the concept of the minimally invasive surgery for HD. The purpose of this study was to evaluate the out come of the procedure in neonates. Methods: This retrospective study was carried out in a tertiary pediatric hospital during the period from January 2007 to December 2012 (5 years). The study included neonates of both sex, weight more than 2 kg, who were clinically suspected HD, radiologically transition zone at rectosigmoid and midsigmoid region and rectal biopsy proven HD and no evidence of sepsis or entrocolitis. Short segment HD with associated anomalies, and operated cases with less than 6 months' or irregular follow up were excluded. Results: During study period, single-stage transanalendorectal pull through (TEP) operation was done for short segment HD in 63 neonates, 9 patients were excluded from the study for irregular follow up. The mean operative time, mean blood loss, postoperative hospital stay, follow up period were 113 minutes, 20ml, 6.8 days and 19.6 months respectively. Transverse colostomy was needed in 6 patients for anastomotic leakage, thereafter developed anastomotic stricture, managed with regular anastomotic dilatation. Conclusion: Advancement in pediatric anesthesia, improvement of pediatric surgical expertise, perioperative management and nursing care has made single-stage primary transanalendorectal pull-through a feasible and safe surgical procedure for the treatment of short segment Hirschsprung's disease in neonate. Journal of Surgical Sciences (2014) Vol. 18 (2) : 45-50

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HIRSCHSPRUNG’S DISEASE;

The Professional Medical Journal ◽

10.29309/tpmj/2014.21.02.2096 ◽

2018 ◽

Vol 21 (02) ◽

pp. 391-394

Author(s):

Muhammad Ishfaq ◽

Saima Manzoor ◽

Muhammad Azim Khan ◽

Umar Farooq Ahmad

Keyword(s):

Early Diagnosis ◽

Hirschsprung's Disease ◽

Hirschsprung’S Disease ◽

Rectal Biopsy ◽

Anorectal Stenosis ◽

Pull Through

Objective: To determine the frequency of Hirschsprung’s Disease associatedenterocolitis in pre and post operative patients. Place and Duration of Study: Nishtar Hospitaland Ibn-e-Sina Hospital, Multan from January 2008 to June 2012. Methodology: A total numberof 114 patients aged from less than 6 months to more than 5 years were diagnosed and treated forHirschsprung’s Disease after rectal biopsy and internal sphinterotomy. Results: In total of 114patients 32 (28%) developed enterocolitis before and 5 (18.5%) after definite surgery.Conclusions: The results of this study implicates that the early diagnosis and to make measuresto decrease the post pull-through anastomostic anorectal stenosis can reduce the frequency ofpre and post operative Hirschsprung’s disease associated enterocolitis.

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