Hirschsprung's disease and neonatal intestinal obstruction: Where does it lie in the spectrum?

2018 ◽  
Vol 7 (4) ◽  
pp. 231 ◽  
Author(s):  
Manish Pathak ◽  
Rahul Saxena ◽  
Arvind Sinha ◽  
Vijay Singh
Keyword(s):  
Intestinal Obstruction ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Neonatal Intestinal Obstruction

scholarly journals Patterns and Outcome of Neonatal Intestinal Obstruction in Kanti Children’s Hospital

2020 ◽  
Vol 40 (2) ◽  
pp. 120-124
Author(s):  
Bijay Thapa ◽  
Anupama Basnet
Keyword(s):  
Survival Rate ◽  
Intestinal Obstruction ◽  
Anorectal Malformation ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Intestinal Atresia ◽  
Meconium Ileus ◽  
Children's Hospital ◽  
Surgical Emergency ◽  
Neonatal Intestinal Obstruction

Introduction: Neonatal intestinal obstruction is the most common neonatal surgical emergency. More than 75% of neonatal surgeries are done for the correction of this problem. The outcome of this obstruction mainly depends upon the causes of obstruction, clinical condition of the patient, associated anomalies, expertise and centre where the patient is dealt. This study was undertaken to study the clinical features and outcome of neonatal intestinal obstruction presented in our centre. Methods: The medical records of all neonates admitted in the surgical NICU were selected and cases with the diagnosis of neonatal intestinal obstruction and managed surgically during two years period from September 1, 2015 to September 1, 2017 were retrospectively reviewed and analysed in regard to age at presentation, sex, weight, gestation, postoperative diagnosis and their outcome. Results: Out of 235 admitted neonates, 205 cases were treated surgically for intestinal obstruction. There were a total of 154 (75.2%) males and 51 (24.8%) females. Mean age at presentation was 4.5 days and average weight was 2.25 kg. Among them, anorectal malformation (ARM) 88 (42.92%), intestinal atresia 59 (28.78%), Hirschsprung’s disease 38 (18.53%), malrotation of gut 10 (4.87%) and meconium ileus 10 (4.87%) were the etiology. Among intestinal atresia, duodenal atresia 27 (45.76%) was the commonest one followed by ileal atresia 24 (40.67%), six jejunal atresia (10.16%) and two colonic atresia (3.38%). Overall, 160 neonates survived (78.04%). Among the survival, Hirschsprung’s disease was 34 (89.47%), ARM 78 (88.63%), seven malrotation of gut (70%), six meconium ileus (60%) and intestinal atresia 35 (59.32%). Conclusion: Neonatal intestinal obstruction is the most common neonatal surgical emergency in Kanti Children’s Hospital. Anorectal malformation was the commonest etiology followed by intestinal atresia and Hirschsprung’s disease. Overall survival rate was 78%. Cases with Hirschsprung’s disease and anorectal malformation had about 90% survival rate. Intestinal atresia was the major cause of mortality occupying 53.35% of total mortality of 21.95%. Mortality is mainly due to delayed diagnosis, referral and intervention which lead to higher complications of cases.

scholarly journals Neonatal intestinal obstruction : patterns, problems and outcome

2013 ◽  
Vol 45 (1-2) ◽  
pp. 6-10 ◽  
Author(s):  
Amar Kumar Saha ◽  
Md Barkot Ali ◽  
Sunil Kumar Biswas ◽  
HM Zafor Sharif ◽  
Anwerul Azim
Keyword(s):  
Surgical Treatment ◽  
Intestinal Obstruction ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Intestinal Atresia ◽  
Postoperative Management ◽  
Meconium Ileus ◽  
College Hospital ◽  
Female Ratio ◽  
Neonatal Intestinal Obstruction

Neonatal intestinal obstruction is the most common surgical emergency in newborn. Ideally neonatal surgery should be done in an organized neonatal surgical unit. This study was done mostly in general surgical setup in Khulna without any facility of NICU and TPN. The aim of this study was to detect the patterns of neonatal intestinal obstruction and to find out the problems and outcome of surgical treatment. This retrospective study was done between January 2008 and December 2010, in Khulna Medical College Hospital, Khulna Shishu Hospital and a private clinic in Khulna. A total of 205 neonates with intestinal obstruction were treated surgically. Babies of both sexes up to 28 days of age were included in this study. Common causes of neonatal intestinal obstruction were anorectal malformation (ARM), intestinal atresia, Hirschsprung's disease (HD), meconium ileus and malrotation of midgut. Male-female ratio was 1.6:1 and about 13% was premature. Out of 205 neonates, there were ARM-73, HD-47, meconium ileus-38, intestinal atresia-29, malrotation-13 and others-5. Total 172 (84%) survived. Those were ARM (94%), Hirschsprung's disease (91%), meconium ileus (79%), intestinal atresia (55%), malrotation of gut (85%) and others (40%). Overall mortality after initial surgical treatment was 16%. Prognosis of surgical treatment depends on early intervention, expert anaesthesia, associated anomaly and complication, gentle handling of delicate tissue and intensive postoperative management. Medical practitioners were the first attending physician in most instances. So both physicians and surgeons have a contributing role in reducing mortality. DOI: http://dx.doi.org/10.3329/bmjk.v45i1-2.13638 Bang Med J (Khulna) 2012; 45 : 6-10

scholarly journals Etiology and Treatment Outcome of Neona tal Intestinal Obstruction in a Tertiary Hospital

2009 ◽  
Vol 22 (2) ◽  
pp. 198-203
Author(s):  
SS Islam ◽  
MA Nowshad ◽  
A Jator ◽  
I Faisal ◽  
M Ahmed
Keyword(s):  
Intestinal Obstruction ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Intestinal Atresia ◽  
Paediatric Surgery ◽  
College Hospital ◽  
Female Ratio ◽  
Neonatal Intestinal Obstruction ◽  
Surgery Department

A prospective study was conducted on 54 cases of neonatal intestinal obstruction, admitted in Paediatric surgery department of Rajshahi Medical College Hospital during the period from January 2008 to December 2008, a period of 12 months. The objective of the study was to find out etiological aspect and outcome of admitted patient with neonatal intestinal obstruction. The patient constituted 33.7% of total neonatal admission in Paediatric surgery department during the study period. The male: female ratio was 1.4:1 with age ranging from 1 to 28 days. Birth weight of most of the patients was above 2 kg. Most of the patients came from poor and average family. Patients from poor family are slightly ahead. Plain x-ray abdomen was done in all cases in our study. Contrast radiography performed in selected cases. Ultrasound scan done to in selective case to rule out IHPS. No rectal biopsy was taken in case of suspected Hirschsprung's disease. The causes of neonatal intestinal obstruction were in order of frequency-Hirschsprung's disease (45.2%), Intestinal atresia (24.5%), malrotation (9.4%), bands & adhesion (5.6%), meconium ileus (5.6%), volvulus neonatarum (5.6%), meconium plug syndrome (1.8%), unknown (3.7%). 50 patients were treated operatively. 28.3% cases developed complications. Most of the complications were related to colostomy and sepsis. Overall mortality was 20.8%. Mortality was maximum in intestinal atresia. Every patient was advised to come for follow up at the time of discharge but most of the people did not report. In the study only 17 (32.1%) patient came for follow up as per advice.TAJ 2009; 22(1): 198-203

scholarly journals Early and long-term complications following transanal pull through Soave technique in infants with Hirschsprung's diseaseric infants with Hirschsprung's disease

2019 ◽  
Author(s):  
Manoochehr Ghorbanpour ◽  
Mohammad Ali Seyfrabie ◽  
Babak Yousefi
Keyword(s):  
Intestinal Obstruction ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Statistical Tests ◽  
Case Series ◽  
P Value ◽  
Short Term ◽  
Case Series Study ◽  
The Mean

Objective. Patients undergoing Soave surgery for Hirschsprung's disease are at risk for some complications. The aim of this study was to investigate such short-term and long-term complications and evaluate the outcome of the operation in these patients. Methods. A case series study was carried out during the last 12 years, during 2007 to 2018 in Besat hospital of Hamadan. Data collection conducted using a checklist includes questions about demographic information, clinical features, and short-term and long-term complications, and consequences of post-operative surgery. The findings of the study were analyzed using SPSS software version 20 and appropriate statistical tests. P-value less than 0.05 was considered statistically significant. Results. A total of 55 children underwent Soave surgery during the last 12 years in Besat Hospital Hamadan, Iran. The mean age of the patients was 38±10 days during surgery, of which 56.4% were female. The mean hospital stay was 7.3 days. Also, the mean weight of children at birth was 2970±447 gr. Most of the patients were born as NVD (52.7%) and term (74.5%). The most common comorbidity was congenital heart disease. The most common short-term complication was intestinal obstruction in 14 patients (25.5%) and the most frequent long-term complication was intestinal obstruction and constipation (27.3% each cases). The mortality rate of patients in this study was 14.5% in total. Conclusions. One stage surgical procedure in Hirschsprung's disease is a safe and effective method, but care should be taken in choosing patients and patients should be monitored for possible complications, so that they can be considered and implemented for proper treatment.

scholarly journals Intestinal Duplication and Hirschsprung's Disease: An Extremely Rare and Misleading Combination

2018 ◽  
Vol 06 (01) ◽  
pp. e94-e96
Author(s):  
Fabrizio Vatta ◽  
Alessandro Raffaele ◽  
Noemi Pasqua ◽  
Marco Brunero ◽  
Gloria Pelizzo ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Intestinal Obstruction ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Incidental Finding ◽  
Simultaneous Occurrence ◽  
Unique Case ◽  
Intestinal Duplication ◽  
Intestinal Occlusion

AbstractHirschsprung's disease and, more rarely, intestinal duplication can both cause intestinal obstruction in neonates. The simultaneous occurrence of these two diseases is reported in only two studies, and in both cases, intestinal duplication was an incidental finding, as it had not determined clinical intestinal occlusion. This paper reports a unique case of coexistence of the two conditions, with both causing intestinal obstruction, delayed appropriate, and definitive surgical treatment.

scholarly journals Familial Near-Total Intestinal Aganglionosis

2017 ◽  
Vol 6 (3) ◽  
pp. 62 ◽  
Author(s):  
Saida Hidouri ◽  
Hayet Zitouni ◽  
Jamila Chahed ◽  
Sana Mosbahi ◽  
Samia Belhassen ◽  
...  
Keyword(s):  
Intestinal Obstruction ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Rare Form ◽  
Total Intestinal Aganglionosis ◽  
Intestinal Aganglionosis

Near total aganglionosis represents the most extreme and rare form of Hirschsprung's disease. It can affect more than one member of family. We report three cases of near total intestinal aganglionosis in a family presenting with intestinal obstruction at birth. All of them were operated and a jejunostomy was performed. Outcome was dismal.

scholarly journals HIRSCHSPRUNG’S DISEASE

2014 ◽  
Vol 21 (01) ◽  
pp. 020-026
Author(s):  
Muhammad Imran Ishfaq ◽  
Umar Farooq Ahmad ◽  
Saima Manzoor
Keyword(s):  
Intestinal Obstruction ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Results Of Treatment ◽  
Pull Through

Objective: To determine the frequency of Hirschsprung’s Disease as a cause ofneonatal intestinal obstruction, to compare the results of treatment with others and to suggestmeans and ways to improve the deficiencies in our circumstances. Place and duration of study:Nishtar hospital and Ibn-e-Siena Hospital, Multan from January, 2008 to June, 2012.Methodology: A total number of 115 patients from neonatal age to more than 5 years werediagnosed and treated for Hirschsprung’s Disease. Results: Hirschsprung’s Disease was acause of neonatal obstruction in 26 (22.6%) patients. Hirschsprung’s Disease was a cause ofrecurrent diarrhea in 37 (32.17%) patients. 76 (66.08%) patients presented with chronicconstipation. 27 patients underwent for pull-through operation. Conclusions: The overallcomplications rate and stooling pattern after Soave’s procedure are not significantly differentfrom others.

scholarly journals Sigmoid volvulus in Bardet-Biedl syndrome: serendipity or a new association?

2019 ◽  
Vol 6 (4) ◽  
pp. 1388
Author(s):  
Ashwath Narayan Ramji
Keyword(s):  
Intestinal Obstruction ◽  
Renal Dysfunction ◽  
Clinical Features ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Case Reports ◽  
Sigmoid Volvulus ◽  
Bardet Biedl Syndrome ◽  
Emergency Presentation ◽  
New Association

Bardet-Biedl syndrome is a rare ciliopathy with a wide array of clinical features, including congenital blindness, obesity, neuroendocrine disturbance, hypogonadism and renal dysfunction. The association of Hirschsprung’s disease with Bardet-Biedl syndrome has been published earlier, however, there is no literature regarding patients with Bardet-Biedl syndrome presenting later in life due to complications associated with Hirschsprung’s disease. Bardet-Biedl Syndrome is exceedingly rare, with only around 15 case reports published from India. Here we present the case of a 55-year-old male patient with Bardet-Biedl syndrome who presented with intestinal obstruction, raising the question as to whether his emergency presentation was a complication of his flawed genotype or serendipity.

scholarly journals COLON ATRESIA IN COMBINATION WITH HIRSCHSPRUNG’S DISEASE: A RARE CLINICAL CASE

2019 ◽  
Vol 25 (4) ◽  
pp. 75-80
Author(s):  
S. A. Karavaeva ◽  
A. V. Kagan ◽  
A. N. Kotin ◽  
T. V. Kesaeva
Keyword(s):  
Intestinal Obstruction ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Chromosomal Abnormalities ◽  
Ganglion Cells ◽  
Histological Study ◽  
Ileocecal Valve ◽  
Contrast Enema ◽  
Surgical Department ◽  
Colonic Atresia

Introduction. Hirschsprung’s disease is a congenital anomaly characterized by the absence of ganglion cells in submucosal and intramuscular layers of intestinal wall that leads to the intestinal obstruction. 70–80 % of cases are isolated malformation but it can be combined with chromosomal abnormalities and other malformations. Coexistence of Hirschsprung’s disease with intestinal atresia is extremely rare. It can cause significant difficulties in diagnostics and treatment.Material and methods.Patient A, a boy, was transferred to the surgical department at the age of two days with a history of intestinal obstruction. He had abdominal X-ray studies: intestinal obstruction. The contrast enema showed microcolon that was coiled in the pelvis. Patient was prepared to the surgical treatment. Intraoperatively colonic atresia was identified at 10 sm from the ileocecal valve. A double colostomy was performed to the child and biopsy on the level of mucous fistula was taken. Histological study showed the aganglionosis of the distal colon. At the age of 4 months, the patient underwent Soave-Swenson endorectal pullthrough procedure with intraoperative extended express-biopsy that confirmed the absence of ganglion cells in whole distal bowel. The aganglionic part was resected, the ileocecal valve with the part of the colon of 10 cm long was mobilized and the endorectal bringing the colon down to the perineum was performed by Soave – Swenson. Postoperative recovery was uneventful.Results.A high index of suspicion is required to promptly diagnose Hirschsprung’s disease in a child with colonic atresia despite the rare combination of these two anomalies. In this case the histological study allowed to recognize association of colonic atresia with Hirschsprung’s disease and helped to avoid complications after further surgery.Conclusions. Early detection of coexisting of these two anomalies helps to prevent the development of serious postoperative complications. 

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