View clinical trials related to Hirschsprung Disease.
Filter by:Hirschsprung disease (HSCR) is a rare congenital intestinal disease characterized by the absence of ganglion cells in the distal rectum, extending for variable distances into the proximal intestine.The "pull-through" reconstruction procedure described in 1949 by Orvar Swenson involving the removal of the aganglionic bowel and creating an anastomosis between the normally innervated bowel and the anal canal, remains the standard surgical approach for HSCR today. However, as rectal dissection by laparotomy in infants is technically difficult and can result in high rates of complications, other pull-through techniques were developed and several techniques are still widely used today. In our institute, we developed the laparoscopic-assisted modified Soave with short muscular cuff anastomosis in July 2017, and achieved good therapeutic effects. However, there have some patients suffered soiling incidents in the short period post-surgery. Therefore, we developed the robot-assisted modified Soave with short muscular cuff anastomosis procedures to protect the vital nerve and blood vessels of the pelvis from injury, decrease the injury of the sphincter. this clinical trials was to compare the efficacy of robot-assisted and laparoscopic-assisted modified Soave with short muscular cuff anastomosis procedures for classical Hirschsprung disease (HSCR).
This study explores the use of an exclusive human milk diet versus standard feeding practices to compare the influence on feeding outcomes and the gut bacteria in infants with intestinal differences.
Bowel dysfunction has been proven as the most common complication after pull-through(PT) of Hirschsprung disease(HD) ，which may persist to adulthood and lead to social problems.The reason of bowel dysfunction is complicated and the risk factors were not defined.
Hirschsprung disease (HSCR) is characterized by the absence of ganglion cells (aganglionosis) in the distal bowel extending proximally for varying distances that results in persistent spasm in the affected bowel and functional intestinal obstruction. Patients can be classified as rectosigmoid HSCR when aganglionosis confined to the rectosigmoid and long-segment or total colonic HSCR when aganglionosis extends beyond the upper sigmoid. Aganglionosis of long-segment HSCR can extend to the descending colon, transverse colon, ascending colon, but not to the terminal ileum. To date, there is insufficient evidence to recommend a preferred or superior method for the surgical repair for long-segment HSCR. In general, a pull-through with standard of care for the intestine and mesentery, which avoids excessive resection of the colon and coloanal reconstruction, is performed for long-segment HSCR.There are reports that a significant percentage of long-segment HSCR patients continue to have difficulty with soiling and incontinence,however there were also reports long-segment HSCR patients have the same continece as rectosigmoid HSCR.The outcome of long-segment HSCR should be thoroughly evaluated. The present study was designed to evaluate the long-term outcomes of long-segment HSCR.
Approximately 340 babies are born with Hirschsprung's disease (HSCR) or an anorectal malformation (ARM) per year in the UK. Most require corrective surgery in the newborn or early infancy period. In both conditions, there is both variability in the severity of the condition and the type of operative technique used. Many children do well following surgery and have good continence in later life. However a significant proportion of children suffer from a lifetime of constipation or incontinence. This has a significant impact on their social and psychological welfare and is a significant burden on healthcare resources. Due to the variation in practice, there are limited data on long-term outcomes following surgery for children with HSCR or ARMs. Where studies have been performed, they often include small numbers, non-standardised outcome measures and short follow-up periods. It is therefore difficult to ascertain the effectiveness of different management strategies. However, a recent Delphi process has been carried out to establish 10 core measures in HSCR to improve outcome reporting. This research group has a long track record in this research area, specifically in the determination of long-term outcomes of children with HSCR, leading to well cited papers within the literature. In the last 2 decades there has been a significant change in the surgical techniques used in HSCR, however the underlying evidence base for this is still lacking. This study seeks to follow-on from previous studies looking specifically at the long-term outcomes in children with HSCR. Furthermore, the aim is to widen the study to encompass all children treated at Alder Hey and also to use the same methodology to assess long-term outcomes for children with ARMs, as both groups of patients experience similar long term morbidity. This will provide qualitative and quantitative data aiding counselling parents of children with HSCR/ARM.
The objective of this prospective study is to compare the diagnostic accuracy of rectal suction biopsy (RSB) compared to full thickness rectal biopsy (FTB) in the diagnosis of Hirschsprungs Disease (HD) in children. The secondary objective to compare cost-effectiveness of the two procedures
With an increasing body of evidence to support a causal link between drinking milk that contain cow's milk protein (CMP) and the development of gastrointestinal disturbance in infants, many clinicians avoid the use of CMP containing feed in high risk babies. Delivery of adequate nutritional intake is one of the great challenges in the care of newborn infants, particularly those born preterm or with gastrointestinal problems. Whilst there are recognised benefits of human milk, a diet of exclusive human milk may not meet the nutritional demands of the infant. To close this gap, breast milk fortifier (BMF) is typically added to human milk. However, addition of BMF may be associated with gastrointestinal disturbance, possibly due to the fact that it contains CMP. This research study is to test the tolerability and safety of a new human milk-based BMF in neonates with gastrointestinal problems. It is hoped that this may provide an opportunity for high risk infants, to receive the benefits of human milk whilst minimising the risks reported to be associated with CMP. Eligible infants will be those in whom nutritional supplementation of breast is deemed clinically necessary, a weight of greater than 1.0kg at the time of starting fortifier and at least one of: - previous gastrointestinal surgery - congenital gastrointestinal anomaly - medically treated gastrointestinal disease - previously suspected intolerance of CMP based breast milk fortifier in the absence of other gastrointestinal disease Infants will be started on human milk-based BMF once they are tolerating 100 mls per kilo per day of human breast milk. The human milk-based fortifier will be commenced at half the recommended dose for 48 hours then increase to full strength. This will be continued until the infant reaches 44 weeks corrected gestational age, or until such time as they are deemed to no longer require the additional nutrition.
Hirschsprung's Disease (HD) and anorectal malformations (ARM) are both paediatric diseases of the colon and rectum. Both of these conditions require surgery in order to correct them, frequently needing sections of the bowel to be removed. Some complications of removing parts of the bowel include forming a stricture (a narrowing of the place where the bowel is reattached), and leak from the join. These can be devastating complications, and can significantly impact a patient's quality of life. Indocyanine green (ICG) is a medication that binds to blood vessels and can be visualized using special cameras in the operating room. Once it binds, it "lights up" green on this equipment. This lends the advantage of being able to better see which parts of the bowel get good blood flow. The theory is that good blood flow can reduce the risks of previously mentioned complications. This has been shown to be true in a variety of bowel surgeries in adults. ICG has also been shown to be safe in children. Given that HD and ARM are both relatively rare conditions (~1/5000 live births), research in this area needs to be strategic. The investigators want to ensure that the investigators can recruit enough patients to the study using this protocol such that if this were to be scaled up to a multi-centre trial in the future, the investigators could demonstrate feasibility. The investigators will collect data both on the feasibility of recruitment as the primary outcome and secondary outcomes including stricture, leak, length of stay in hospital, and return to hospital.
The purpose of this study is to assess the efficacy of neuromodulation for treatment of chronic constipation and fecal incontinence in pediatric patients and to evaluate the differences between the invasive vs. non-invasive approach.
Registry for Hirschsprung disease of the BELAPS (Belgian Association of Pediatric Surgery) Protocol Short Title/Acronym / Sponsor name BELAPS Principal Investigator Prof. Dr. Marc Miserez Medical condition or disease under investigation Hirschsprung's disease Purpose of clinical study To register pre-, intra- and postoperative data in order to improve surgical care to paediatric patients with Hirschsprung's disease in Belgium. Primary objective The primary objective is to evaluate defecation habits at 3.5-5.5 years after surgery. Secondary objective (s) Evaluating the long-term results and evaluate risk factors for constipation / incontinence. Study Design Prospective registry Endpoints Krickenbeck score, urinary, sexual and QoL evaluation