scholarly journals PRESENTATION AND MANAGEMENT OF HIRSCHSPRUNG'S DISEASE IN INFANTS AT A TERTIARY CARE CENTRE AND OUTCOME OF PRIMARY PULL-THROUGH IN THESE PATIENTS.

2021 ◽  
pp. 1-4
Author(s):  
Magray Mudasir A ◽  
Mufti, Gowhar N ◽  
Quyoom Nazia ◽  
Bhat, Nisar A. ◽  
Baba, Aejaz A
Keyword(s):  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Frozen Section ◽  
Tertiary Care ◽  
Treatment Protocol ◽  
Rectal Biopsy ◽  
Water Soluble ◽  
Tertiary Care Centre ◽  
Young Infants ◽  
Pull Through

Hirschsprung's disease is a common cause of neonatal intestinal obstruction. The treatment of Hirschsprung's disease has evolved to a single stage pull-through. . The aim of study was to evaluate the feasibility of performing the primary pull-through surgery and to develop an optimum treatment protocol for patients of Hirschsprung's disease (HD) presenting to our institution. It was a prospective cohort study of newborn and young infants with features of Hirschsprung's disease conducted in our department from Oct. 2014, till Oct. 2018. The diagnosis was based on history, clinical examination, water soluble contrast enema and a definitive rectal biopsy. Transition zone was confirmed by intra-operative frozen section. Patients with weight of more than 5kgs and absence of features of concurrent enterocolitis, were taken for surgery. Patients included in the study underwent primary pull-through by different methods were put on strict follow-up protocol for 24 months and results were evaluated. Out of 40 patients of Hirschsprung's disease presented to SKIMS from Oct. 2014 till Oct. 2017,only 25 patients underwent primary pull-through. Mean age of patients at the time of pull-through was6.9±7.12 months and mean weight was 8.76 ±2.67kg. Delayed passage of meconium was seen in23 patients (92%). Preoperative rectal biopsy was done for confirmation of diagnosis and intra-operative frozen section for delineation level of aganglionosis. Laparoscopic transanal pull-through was most common operation done in 13 (52%) patients. Anastomotic leak was seen in 2(8%) patients, wound infection occurred 2(8%) patients, enterocolitis in1(4%) and constipation in 2 (8%) patients. One patient who had undergone trans-anal pull-through underwent a redo for a retained cuff. Primary pull-through is feasible and safe option with excellent results in neonates and infants with Hirschsprungs disease.

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

2021 ◽  
pp. 558-564
Author(s):  
Anushri Kale ◽  
Aditi Badwaik ◽  
Pallavi Dhulse ◽  
Archana Maurya ◽  
Bibin Kurian
Keyword(s):  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Ganglion Cells ◽  
Past History ◽  
Rectal Biopsy ◽  
Case Presentation ◽  
Surgical History ◽  
History Of ◽  
Pull Through ◽  
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.

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

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.

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

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

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

2018 ◽  
Vol 29 (05) ◽  
pp. 425-430 ◽  
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.

Robotic Swenson Pull-Through for Hirschsprung's Disease in Infants

2011 ◽  
Vol 77 (7) ◽  
pp. 937-941 ◽  
Author(s):  
André Hebra ◽  
Valerie A. Smith ◽  
Aaron P. Lesher
Keyword(s):  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Average Length ◽  
Robotic Technology ◽  
Average Length Of Stay ◽  
Young Infants ◽  
Bowel Movements ◽  
Da Vinci Robotic System ◽  
Average Operative Time ◽  
Pull Through

It has been demonstrated that infants with Hirschsprung's disease can be treated with a one-stage laparoscopic resection and coloanal pull-through. However, the feasibility and benefits of performing this operation using robotic technology have not yet been evaluated. We reviewed our experience with 12 infants diagnosed with Hirschsprung's disease and treated with laparoscopic-robotic assisted colonic resection with proctectomy and pull-through using the da Vinci robotic system. Patients were treated at a mean age/weight of 16 weeks/5.5 kg. The average operative time for the robotic procedure was 230 minutes, and average length of stay was 3 days. At discharge, all patients were having regular bowel movements and tolerating a completely oral diet. All patients received early postoperative anorectal dilation and six patients required dilations for an average of 12 weeks after surgery for management of minor rectal strictures. Only two patients developed postoperative enterocolitis with a mean follow-up of 36 months. A robotic approach for performing a Swenson-type resection and pull-through procedure can be performed safely and successfully in young infants. Robotic technology provided superior dexterity and visualization, essential in performing a more complete rectal dissection, thus allowing for a complete proctectomy and eliminating the risk of leaving a segment of aganglionic rectum behind.

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

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

scholarly journals HIRSCHSPRUNG’S DISEASE;

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.

scholarly journals The follow-up of the robotic-assisted Soave procedure for Hirschsprung’s disease in children

2021 ◽  
Author(s):  
Tran Anh Quynh ◽  
Pham Duy Hien ◽  
Le Quang Du ◽  
Le Hoang Long ◽  
Nguyen Thi Ngoc Tran ◽  
...  
Keyword(s):  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Pediatric Patients ◽  
Three Dimensional ◽  
Dentate Line ◽  
Robotic Arms ◽  
Robotic Assisted ◽  
The Mean ◽  
Pull Through

AbstractRobotic surgery offers three-dimensional visualization and precision of movement that could be of great value to gastrointestinal surgeons. There were many previous reports on robotic technology in performing Soave colonic resection and pull-through for Hirschsprung’s disease in children. This study described the follow-up of the Robotic-assisted Soave procedure for Hirschsprung’s disease in children. Robotic-assisted endorectal pull-through was performed using three robotic arms and an additional 5-mm trocar. The ganglionic and aganglionic segments were initially identified by seromuscular biopsies. The rest of the procedure was carried out according to the Soave procedure. We left a short rectal seromuscular sleeve of 1.5–2 cm above the dentate line. From December 2014 to December 2017, 55 pediatric patients were operated on. Age ranged from 6 months to 10 years old (median = 24.5 months). The aganglionic segment was located in the rectum (n = 38), the sigmoid colon (n = 13), and the left colon (n = 4). The mean total operative time was 93.2 ± 35 min (ranging from 80 to 180 min). Minimal blood was lost during the surgery. During the follow-up period, 41 patients (74.6%) had 1–2 defecations per day, 12 patients (21.8%) had 3–4 defecations per day, and 2 patients (3.6%) had more than 4 defecations per day. Fecal incontinence, enterocolitis, and mild soiling occurred in three (5.4%), four (7.3%), and two pediatric patients, respectively. Robotic-assisted Soave procedure for Hirschsprung’s disease in children is a safe and effective technique. However, a skilled robotic surgical team and procedural modifications are needed.

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