Materiały źródłowe

• #1 Hirschsprung’s disease – Symptoms & causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hirschsprungs-disease/symptoms-causes/syc-20351556

  Hirschsprung’s (HIRSH-sproongz) disease is a condition that affects the large intestine (colon) and causes problems with passing stool. The condition is present at birth (congenital) as a result of missing nerve cells in the muscles of the baby’s colon. Without these nerve cells stimulating gut muscles to help move contents through the colon, the contents can back up and cause blockages in the bowel. […] A newborn who has Hirschsprung’s disease usually can’t have a bowel movement in the days after birth. In mild cases, the condition might not be detected until later in childhood. Uncommonly, Hirschsprung’s disease is first diagnosed in adults. […] Signs and symptoms of Hirschsprung’s disease vary with the severity of the condition. Usually signs and symptoms appear shortly after birth, but sometimes they’re not apparent until later in life.

• #1 Hirschsprung’s disease – Symptoms & causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hirschsprungs-disease/symptoms-causes/syc-20351556

  Typically, the most obvious sign is a newborn’s failure to have a bowel movement within 48 hours after birth. […] Other signs and symptoms in newborns may include: Swollen belly, Vomiting, including vomiting a green or brown substance, Constipation or gas, which might make a newborn fussy, Diarrhea, Delayed passage of meconium a newborn’s first bowel movement. […] In older children, signs and symptoms can include: Swollen belly, Chronic constipation, Gas, Failure to thrive, Fatigue.

• #1 Hirschsprung’s Disease | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/hirschsprungs-disease

  Hirschsprung’s disease (also called congenital aganglionic megacolon) occurs when some of the intestinal nerve cells (ganglion cells) dont develop properly, delaying the progression of stool through the intestines. […] The intestine becomes blocked with stool, and your baby or child will be constipated (unable to have normal bowel movements). Often, a serious infection called enterocolitis can occur, which causes fever, pain, and diarrhea. […] Symptoms of Hirschsprung’s disease vary with age. Eighty percent of children with Hirschsprung’s disease have symptoms in the first six weeks of life. However, children who only have a short segment of intestine that lacks normal nerve cells may not exhibit symptoms for several months or years. Their primary symptom is constipation. […] Each child may experience symptoms differently, but common symptoms in infants include: Failure to have a bowel movement in the first 48 hours of life, Abdominal distention (stomach bloating), Gradual onset of vomiting, Fever, Constipation or failure to pass regular bowel movements.

• #1 Hirschsprung’s Disease | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/hirschsprungs-disease

  Children who dont have early symptoms may experience the following signs of Hirschsprungs disease as they get older: Constipation that becomes worse with time, Loss of appetite, Delayed growth, Passing small, watery stools, Abdominal distention. […] It is not uncommon for children with Hirschsprung’s disease to continue to have challenges after surgery. […] Possible problems include: Intractable constipation, Diarrhea, Stool accidents, Frequent episodes of infections (enterocolitis), Recurrent hospitalizations, Severe abdominal distention (a very bloated stomach), Pain, Inability to tolerate food, Vomiting. […] Children who have had a large section of intestine removed may also experience long-term digestive problems. Removing a large segment of the intestine can prevent a child from getting adequate nutrients and fluids, leading to problems with improper digestion, slow growth, and infection.

• #1 Hirschsprung Disease (for Parents) | Nemours KidsHealth

  https://kidshealth.org/en/parents/hirschsprung.html

  A newborn who can’t poop within the first 48 hours of life is often how doctors find Hirschsprung disease. This red flag can be very valuable in diagnosing the condition. […] Less severe cases might not be spotted until a child is a little older, or sometimes even later. Symptoms in these cases are usually milder but can be long-lasting (or chronic). They can include: a swollen belly, constipation, trouble gaining weight, vomiting, gas. […] Older kids with Hirschsprung disease might have a growth delay because the condition can affect the body’s ability to absorb nutrients.

• #1 Symptoms & Causes of Hirschsprung Disease – NIDDK

  https://www.niddk.nih.gov/health-information/digestive-diseases/hirschsprung-disease/symptoms-causes

  Symptoms of Hirschsprung disease vary. Signs or symptoms in newborns may be different than symptoms in older infants, children, and adults. […] Some infants with Hirschsprung disease have signs and symptoms of intestinal obstruction shortly after they are born, which include not having a first bowel movement within 48 hours after birth, green or brown vomit, swollen abdomen, or belly, problems feeding, explosive stools or diarrhea after a doctor inserts a finger into the newborns rectum. […] Children with Hirschsprung disease who dont have signs and symptoms of intestinal obstruction at birth may not be diagnosed and treated until they are older. Common symptoms in older infants and children include chronic constipation that doesnt get better after treatment with oral laxatives, swollen abdomen, malnutrition or failure to thrive, meaning that an infant or child weighs less or is gaining less weight than expected for his or her age, symptoms of Hirschsprung-associated enterocolitis, such as diarrhea. […] In rare cases, Hirschsprung isnt diagnosed and treated until adulthood. Adults with Hirschsprung disease typically have a history of abdominal swelling and chronic constipation that doesnt get better after taking oral laxatives.

• #1 Hirschsprung’s Disease: Diagnosis and Management | AAFP

  https://www.aafp.org/pubs/afp/issues/2006/1015/p1319.html

  Infrequent, explosive bowel movements caused by functional colonic obstruction are common in infants with Hirschsprungs disease. Rectal examination may demonstrate a tight anal sphincter and explosive discharge of stool and gas. Although most patients present in infancy and early childhood, some may not have symptoms until later in life. Common symptoms in older children include chronic progressive constipation, recurrent fecal impaction, failure to thrive, and malnutrition. One third of patients with Hirschsprungs disease present with enterocolitis-related diarrhea rather than constipation. […] Early symptoms of enterocolitis in patients with Hirschsprungs disease include abdominal distention; foul-smelling, watery diarrhea; lethargy; and poor feeding.

• #1 Hirschsprung’s Disease | Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/h/hirschsprung

  Most children with Hirschsprungs disease show symptoms in the first several weeks of life. Symptoms are most often seen during the first 24 to 48 hours of life. The most common symptoms in this time frame include: […] Sometimes symptoms are not noticed for many months or even years, but this is less common now than it was many years ago. […] Children with Hirschsprungs disease are at a higher risk for an infection called enterocolitis that can cause serious problems. Often called Hirschsprungs associated enterocolitis (HAEC), this condition is like a gastrointestinal flu. It is characterized by abdominal bloating, foul smelling diarrhea, fever, weakness, fatigue and not feeding well. It rarely occurs in older children and adults. It is most common in newborns and during the first five years of life.

• #1 Hirschsprung Disease in Children – Stanford Medicine Children’s Health

  https://www.stanfordchildrens.org/en/topic/default?id=hirschsprung-disease-in-children-90-P01999

  The blockage creates pressure on the inside of the intestine. This causes part of the intestinal wall to wear thin. Over time, a bacterial infection called enterocolitis can develop in the digestive tract. This is very serious. Symptoms of enterocolitis include: Fever, Swollen belly, Vomiting, Diarrhea, Bleeding from the rectum, Lack of energy. […] Your child’s bowel function may be affected after surgery. The most common long-term problems include bowel control and leaking stool, constipation, and infections. […] Problems that may occur after surgery depend on the amount of intestine that lacked nerve cells, and how much intestine was removed. […] Children who had a large section of intestine removed may have long-term problems. The digestive process can be affected. Nutrients and fluids are absorbed from food in the small intestine. Removing a large segment of the intestine can prevent a child from getting enough nutrients and fluids. Children can have problems with poor digestion, slow growth, and infection. Your child may need to eat and drink more in order to get enough nutrients and fluid.

• #1 Orphanet: Hirschsprung disease

  https://www.orpha.net/en/disease/detail/388

  Hirschsprung disease (HSCR) generally manifests shortly after birth with symptoms of lower intestinal obstruction such as failure to pass meconium within the first 48 hours of life, abdominal pain, constipation, progressive abdominal distention, vomiting, and occasionally diarrhea. […] Rarely, it presents later in childhood with symptoms of severe constipation and failure to thrive. […] Overall survival and functional prognosis are good in rectosigmoid or long-segment Hirschsprung, despite issues with constipation and continence even following surgical correction. […] The prognosis for children with small bowel or total intestinal aganglionosis leading to intestinal failure can be poor although intestinal transplantation is attempted to offer long term survival in complicated cases. Hirschsprung enterocolitis occurs in 30-50% of the cases but reacts well to treatment in most cases.

• #1 Hirschsprung’s Disease | Doctor

  https://patient.info/doctor/hirschsprungs-disease-pro

  If not recognised early, this may progress to sepsis, transmural intestinal necrosis and perforation. […] The mortality with this condition is around 30-35% and this accounts for most of the mortality associated with Hirschsprung’s disease. […] Hirschsprung patients who develop enterocolitis have a different mix of enteral organisms than those who do not. […] They may have abdominal distention (which is tympanic on percussion) and symptoms of intestinal obstruction. […] They may present with acute enterocolitis in this age group and (rarely) with neonatal meconium plug syndrome or appendicitis. […] They have chronic constipation. […] There may be marked abdominal distention with palpable dilated loops of colon. […] Rectal examination often reveals an empty rectum and may result in the forceful expulsion of faecal material as examination is completed.

• #1 Hirschsprung’s Disease: What It Is, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/9844-hirschsprung-disease

  Severe or untreated Hirschsprungs disease can lead to these potentially life-threatening problems: Large bowel obstruction: A large bowel obstruction stops food from passing through your babys large intestine. Toxic megacolon: This rare, life-threatening condition causes your babys colon to widen and expand. Gas and poop cant move through their swollen intestine. As pressure builds up, bacteria leak from the intestine into their bloodstream. This can cause enterocolitis and a deadly condition called sepsis. In extreme cases, your babys intestine could develop a hole or perforation. […] After surgery, some babies with Hirschsprungs disease may still have constipation, pooping accidents and colon infections. But with long-term follow-up care, most children can manage pooping without becoming severely constipated or developing fecal incontinence.

• #1 Hirschsprung Disease (HD): Symptoms, Diagnosis and Treatment Options | Nationwide Children’s Hospital

  https://www.nationwidechildrens.org/conditions/hirschsprung-disease

  Most children with Hirschsprung disease have symptoms within the first few days and weeks of life. Symptoms depend on how much of the intestine is affected. Children with a very short segment of involvement may not show symptoms for several months or even years, while those with longer lengths of abnormal intestine become sick very quickly. […] The most common symptoms in infants include: The absence of a bowel movement in the first 48 hours of life, Distension (bloating) of the abdomen, Vomiting (the color of bile, which can be dark green to yellow brown), Fever, Diarrhea. […] Older children may have these symptoms: Sepsis (a severe infection), Severe Constipation, Watery stools, Loss of appetite, Delayed growth (failure to thrive).

• #1 Hirschsprung Disease in Children – Stanford Medicine Children’s Health

  https://www.stanfordchildrens.org/en/topic/default?id=hirschsprung-disease-in-children-90-P01999

  Most babies with Hirschsprung disease have symptoms in the first few weeks of life. […] Each child’s symptoms may vary. Symptoms in newborns may include: Not having a bowel movement in the first 48 hours of life, Slow swelling or bloating of the belly, Vomiting green or brown fluid. […] Children who don’t show early symptoms may also have: Constipation that gets worse over time, Loss of appetite, Slow or delayed growth, Small, watery, bloody stools, Loss of energy. […] Symptoms of Hirschsprung disease may seem like other health problems. […] In Hirschsprung disease a part of the large intestine lacks normal nerve cells. This means that digested food and stool can’t move forward through that part of the digestive tract. The large intestine becomes blocked with stool. Your baby will be constipated, or unable to have normal bowel movements.

• #1 Hirschsprung Disease: Background, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/178493-overview

  Total colonic aganglionosis is associated with a poorer outcome, with 33% of patients experiencing persistent incontinence and 14% requiring a permanent ileostomy. Patients with associated chromosomal abnormalities and syndromes also have poorer clinical outcomes. […] Hirschsprung disease is confined to the rectosigmoid region in about 75% of cases. Approximately 60% of infants with Hirschsprung disease have an associated condition, ranging from subtle to severe. […] Enterocolitis accounts for significant morbidity and mortality in patients with Hirschsprung disease and can progress into toxic megacolon. […] Approximately 10%-30% of patients with Hirschsprung disease develop enterocolitis. Long-segment disease is associated with an increased incidence of enterocolitis. The risk of enterocolitis does not decrease with surgical correction. […] Patients may present postoperatively with abdominal distention, emesis, or constipation indicative of ongoing obstruction. […] Persistent aganglionosis occurs rarely and may be due to pathologic error, inadequate resection, or loss of ganglion cells after the pull-through procedure.

• #1 Hirschsprung Disease in Children | Phoenix Children’s Hospital

  https://phoenixchildrens.org/specialties-conditions/hirschsprung-disease-children

  The blockage creates pressure on the inside of the intestine. This causes part of the intestinal wall to wear thin. Over time, a bacterial infection called enterocolitis can develop in the digestive tract. This is very serious. Symptoms of enterocolitis include: Fever, Swollen belly, Belly pain, Vomiting, Diarrhea, Bleeding from the rectum, Lack of energy. […] Your child’s bowel function may be affected after surgery. The most common long-term problems include bowel control and leaking stool, constipation, and infections. […] Problems that may occur after surgery depend on how much of the intestine lacked nerve cells and how much of it was removed. […] Children who are able to have their ostomy closed may have short-term problems after the closure, including: Stools may be frequent and loose at first.

• #1 Hirschsprung’s Disease – About Kids GI

  https://aboutkidsgi.org/lower-gi/hirschsprungs-disease/

  Infection or inflammation of the intestines continues to be the major cause of complications in Hirschsprungs disease. It generally appears suddenly with rapid progression and requires immediate doctor care. Symptoms include abdominal distention, explosive diarrhea, vomiting, fever, lethargy, and rectal bleeding. Non-operative management in a hospital is usually needed.

• #1 Hirschsprung Disease: What It Is and How It’s Treated

  https://www.healthline.com/health/hirschsprungs-disease

  For most people, Hirschsprung disease is caught very early in life due to identifying a failure to pass the first stool, or meconium, within 48 hours of birth. This is considered the most common symptom of the condition. […] Left untreated, Hirschsprung disease can lead to blockage, bacterial infections, and even death in extreme cases. But when the condition is treated, even though other digestive issues might persist, most people go on to live full lives.

• #1 Hirschsprung’s Disease – familydoctor.org

  https://familydoctor.org/condition/hirschsprungs-disease/

  Sometimes, symptoms are not present at birth. This may mean your child has a milder case. Symptoms in older children may include: Chronic constipation; Lack of weight gain or growth; Swollen abdomen; Diarrhea; Frequent bowel movements; Anemia (a low number of red blood cells). […] Hirschsprung’s disease cannot be cured on its own. It can be fatal for children who do not have surgery. After surgery, most children lead normal lives. They may have minor health problems because of the disease. These include diarrhea, constipation, or other problems passing stool. Some children develop an infection called enterocolitis (digestive tract inflammation). This can be serious, so call your doctor right away if your child has the following symptoms: Vomiting; Fever; Swollen stomach; Abnormal fatigue; Blood in the stool. […] Some problems can occur after pull-through, including: Narrowing of the anus; Constipation; Diarrhea; Leaking stool from the anus; Delayed toilet training; Enterocolitis (digestive tract inflammation).

• #1 Hirschsprung Disease (for Parents) – Humana – Ohio

  https://kidshealth.org/HumanaOhio/en/parents/hirschsprung.html

  A newborn who can’t poop within the first 48 hours of life is often how doctors find Hirschsprung disease. This red flag can be very valuable in diagnosing the condition. […] Less severe cases might not be spotted until a child is a little older, or sometimes even later. Symptoms in these cases are usually milder but can be long-lasting (or chronic). They can include: a swollen belly, constipation, trouble gaining weight, vomiting, gas. […] Older kids with Hirschsprung disease might have a growth delay because the condition can affect the body’s ability to absorb nutrients. […] Most children treated surgically for Hirschsprung disease have an excellent outcome. Most can pass stool normally and have no lasting problems. A few kids might continue to have symptoms, including constipation and bowel control problems.

• #1 Hirschsprung Disease: Background, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/178493-overview

  Most cases of Hirschsprung disease are diagnosed in the newborn period. Hirschsprung disease should be considered in any newborn who fails to pass meconium within 24-48 hours of birth. […] Once the diagnosis is confirmed, the definitive treatment is to remove the aganglionic bowel and to restore continuity of the healthy bowel with the distal rectum, with or without an initial intestinal diversion. […] Reports of long-term outcomes after definitive repair for Hirschsprung disease are conflicting. Some investigators report a high degree of satisfaction, whereas others report a significant incidence of constipation and incontinence. In general, more than 90% of patients with Hirschsprung disease report satisfactory outcomes; however, many patients experience disturbances of bowel function for several years before normal continence is established. Approximately 1% of patients with Hirschsprung disease have debilitating incontinence requiring a permanent colostomy.

• #2 Hirschsprung disease: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/hirschsprung-disease/

  Hirschsprung disease is an intestinal disorder characterized by the absence of nerves in parts of the intestine. This condition is usually identified in the first two months of life, although less severe cases may be diagnosed later in childhood. […] Without these nerves in parts of the intestine, the material cannot be pushed through, causing severe constipation or complete blockage of the intestine in people with Hirschsprung disease. Other signs and symptoms of this condition include vomiting, abdominal pain or swelling, diarrhea, poor feeding, malnutrition, and slow growth. People with this disorder are at risk of developing more serious conditions such as inflammation of the intestine (enterocolitis) or a hole in the wall of the intestine (intestinal perforation), which can cause serious infection and may be fatal. […] Hirschsprung disease occurs in approximately 1 in 5,000 newborns.

• #2 Hirschsprung’s disease

  https://johnsonmemorial.org/jmh-health/disease-conditions/con-20157228

  Hirschsprung’s (HIRSH-sproongz) disease is a condition that affects the large intestine (colon) and causes problems with passing stool. The condition is present at birth (congenital) as a result of missing nerve cells in the muscles of the baby’s colon. Without these nerve cells stimulating gut muscles to help move contents through the colon, the contents can back up and cause blockages in the bowel. […] A newborn who has Hirschsprung’s disease usually can’t have a bowel movement in the days after birth. In mild cases, the condition might not be detected until later in childhood. Uncommonly, Hirschsprung’s disease is first diagnosed in adults. […] Signs and symptoms of Hirschsprung’s disease vary with the severity of the condition. Usually signs and symptoms appear shortly after birth, but sometimes they’re not apparent until later in life.

• #2 Hirschsprung Disease (for Parents) | Nemours KidsHealth

  https://kidshealth.org/en/parents/hirschsprung.html

  A newborn who can’t poop within the first 48 hours of life is often how doctors find Hirschsprung disease. This red flag can be very valuable in diagnosing the condition. […] Less severe cases might not be spotted until a child is a little older, or sometimes even later. Symptoms in these cases are usually milder but can be long-lasting (or chronic). They can include: a swollen belly, constipation, trouble gaining weight, vomiting, gas. […] Older kids with Hirschsprung disease might have a growth delay because the condition can affect the body’s ability to absorb nutrients.

• #2 Hirschsprung’s Disease | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/hirschsprungs-disease

  Hirschsprung’s disease (also called congenital aganglionic megacolon) occurs when some of the intestinal nerve cells (ganglion cells) dont develop properly, delaying the progression of stool through the intestines. […] The intestine becomes blocked with stool, and your baby or child will be constipated (unable to have normal bowel movements). Often, a serious infection called enterocolitis can occur, which causes fever, pain, and diarrhea. […] Symptoms of Hirschsprung’s disease vary with age. Eighty percent of children with Hirschsprung’s disease have symptoms in the first six weeks of life. However, children who only have a short segment of intestine that lacks normal nerve cells may not exhibit symptoms for several months or years. Their primary symptom is constipation. […] Each child may experience symptoms differently, but common symptoms in infants include: Failure to have a bowel movement in the first 48 hours of life, Abdominal distention (stomach bloating), Gradual onset of vomiting, Fever, Constipation or failure to pass regular bowel movements.

• #2 Hirschsprung’s Disease: What It Is, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/9844-hirschsprung-disease

  Some babies with Hirschsprungs disease have blocked intestines at birth. Theres a chance your baby has Hirschsprungs disease if they dont poop within 48 hours after birth. […] Other symptoms of Hirschsprungs disease in babies may include: Swollen belly. Constipation. Vomiting. Diarrhea. Lack of appetite (refusing to eat) and poor weight gain. Delayed growth. […] Without treatment, Hirschsprungs disease can cause serious complications. […] Up to 40% of children with Hirschsprungs disease develop enterocolitis. This condition is the swelling of your childs small and large intestines. Some children have mild inflammation. Others have severe symptoms that can be life-threatening. If your child has severe enterocolitis from Hirschsprungs disease, they may have fever and explosive diarrhea.

• #2 Hirschsprung Disease (HD): Symptoms, Diagnosis and Treatment Options | Nationwide Children’s Hospital

  https://www.nationwidechildrens.org/conditions/hirschsprung-disease

  Most children with Hirschsprung disease have symptoms within the first few days and weeks of life. Symptoms depend on how much of the intestine is affected. Children with a very short segment of involvement may not show symptoms for several months or even years, while those with longer lengths of abnormal intestine become sick very quickly. […] The most common symptoms in infants include: The absence of a bowel movement in the first 48 hours of life, Distension (bloating) of the abdomen, Vomiting (the color of bile, which can be dark green to yellow brown), Fever, Diarrhea. […] Older children may have these symptoms: Sepsis (a severe infection), Severe Constipation, Watery stools, Loss of appetite, Delayed growth (failure to thrive).

• #2 Symptoms & Causes of Hirschsprung Disease – NIDDK

  https://www.niddk.nih.gov/health-information/digestive-diseases/hirschsprung-disease/symptoms-causes

  Symptoms of Hirschsprung disease vary. Signs or symptoms in newborns may be different than symptoms in older infants, children, and adults. […] Some infants with Hirschsprung disease have signs and symptoms of intestinal obstruction shortly after they are born, which include not having a first bowel movement within 48 hours after birth, green or brown vomit, swollen abdomen, or belly, problems feeding, explosive stools or diarrhea after a doctor inserts a finger into the newborns rectum. […] Children with Hirschsprung disease who dont have signs and symptoms of intestinal obstruction at birth may not be diagnosed and treated until they are older. Common symptoms in older infants and children include chronic constipation that doesnt get better after treatment with oral laxatives, swollen abdomen, malnutrition or failure to thrive, meaning that an infant or child weighs less or is gaining less weight than expected for his or her age, symptoms of Hirschsprung-associated enterocolitis, such as diarrhea. […] In rare cases, Hirschsprung isnt diagnosed and treated until adulthood. Adults with Hirschsprung disease typically have a history of abdominal swelling and chronic constipation that doesnt get better after taking oral laxatives.

• #2 Hirschsprung’s disease – Symptoms & causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hirschsprungs-disease/symptoms-causes/syc-20351556

  Typically, the most obvious sign is a newborn’s failure to have a bowel movement within 48 hours after birth. […] Other signs and symptoms in newborns may include: Swollen belly, Vomiting, including vomiting a green or brown substance, Constipation or gas, which might make a newborn fussy, Diarrhea, Delayed passage of meconium a newborn’s first bowel movement. […] In older children, signs and symptoms can include: Swollen belly, Chronic constipation, Gas, Failure to thrive, Fatigue.

• #2 Hirschsprung’s Disease Symptoms and Treatments

  https://www.verywellhealth.com/hirschsprungs-disease-2632069

  Hirschsprung’s disease is a congenital cause of intestinal blockage or obstruction. It’s uncommon, occurring in about 1 in every 5,000 births, and occurs more often in males than in females. […] The life expectancy of someone with Hirschsprung’s disease is reduced if the condition goes untreated. Surgical treatment generally results in excellent outcomes. […] Most of the children with Hirschsprung’s disease will have a delay in passing meconium. Some others will develop chronic constipation later in the first month of life. Either way, it can lead to intestinal obstruction, with many associated signs and symptoms, including: Abdominal distension, Vomiting, which can be bilious, Poor feeding, Poor weight gain. […] Not all babies born with Hirschsprung’s have symptoms right away. Some may develop symptoms in later infancy or early childhood. About 50 percent of children with Hirschsprung’s disease are diagnosed by age 1 year, and about 80 percent are diagnosed by age 7 years.

• #2 Adult Hirschsprung’s disease presenting as chronic constipation: a case report | Journal of Medical Case Reports | Full Text

  https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-023-03986-y

  Hirschsprungs disease is a congenital disorder identified by the absence of ganglion cells at the Meissners plexus of the submucosa and Auerbachs plexus of the muscularis. It is usually characterized by nonspecific symptoms such as chronic constipation. […] In adult patients presenting with history of constipation since childhood, it is necessary to consider the possibility of Hirschsprungs disease that was not diagnosed in early childhood. Hirschsprungs disease in adults is usually a short or ultra-short aganglionic segment because it shows relatively mild symptoms. […] Adult Hirschsprungs disease is commonly misdiagnosed as refractory constipation. The absence of intramural ganglion cells in the affected segment of the colon is the underlying mechanism of refractory constipation in Hirschsprungs disease in both infants and adults. […] Mild Hirschsprungs disease in early life might go undetected due to the proximal colon compensating for the nonmobile colon segment, then presenting as chronic refractory constipation in adulthood. […] These findings thus directed our clinical judgement to the diagnosis of Hirschsprungs disease.

• #2 Hirschsprung’s Disease | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/hirschsprung-s-disease

  Hirschsprungs disease is most often diagnosed in the newborn period within the first six weeks of life. Symptoms may include: […] Newborns with Hirschsprungs disease will often have abdominal distension (an enlarged abdomen), while older children may suffer from chronic constipation. […] Although much less common, older children can be diagnosed with Hirschsprungs disease. Symptoms in older children with Hirschsprungs disease may include: […] Some infants and children present with Hirschsprung-associated enterocolitis, which is a life-threatening infection of the intestine seen in children with Hirschsprungs disease. Signs and symptoms of enterocolitis are diarrhea, fever, abdominal distension and decreased energy. These signs and symptoms are considered serious and life-threatening. If they occur, notify your child’s doctor immediately.

• #2 Hirschsprung Disease: What You Need to Know

  https://www.massgeneral.org/children/hirschsprung-disease

  After surgery, your childs care team can help with pain control and monitor for stool output (how much stool your child produces and passes). […] Enterocolitis, also known as Hirschsprung-associated enterocolitis (HAEC), is the most serious complication. Enterocolitis can occur before or after pull-through surgery. Symptoms of enterocolitis include: Severe diarrhea, Abdominal distension, Lethargy, Fever. […] Enterocolitis affects about 6 out every 10 children before pull-through surgery. It also affects about 4 out of every 10 children after pull-through surgery.

• #2 Hirschsprung’s disease – Pathophysiology – TeachMePaediatrics

  https://teachmepaediatrics.com/surgery/abdominal/hirschsprungs-disease/

  The aganglionic segment remains in a tonic state leading to failure in peristalsis and bowel movements. Faeces in the rectum fail to trigger relaxation of the internal anal sphincter, due to aganglionosis. The accumulation of faeces in the rectosigmoid region is responsible for the functional obstruction, which is the cause of many of the symptoms. It can lead to proximal bowel dilatation which can present as abdominal distension. Increased intraluminal pressure can lead to decreased blood flow and deterioration in the mucosal layer. This stasis can lead to bacterial proliferation and the subsequent complication of Hirschsprungs enterocolitis, which has a mortality rate of 25-30%. If not recognised early this can lead to sepsis and death. […] Long term prognosis is generally positive, with many patients acquiring faecal continence. Outcome is usually dependent on the extent of aganglionosis with longer sections correlating with increasing complications.

• #2 Hirschsprung’s Disease | Doctor

  https://patient.info/doctor/hirschsprungs-disease-pro

  If not recognised early, this may progress to sepsis, transmural intestinal necrosis and perforation. […] The mortality with this condition is around 30-35% and this accounts for most of the mortality associated with Hirschsprung’s disease. […] Hirschsprung patients who develop enterocolitis have a different mix of enteral organisms than those who do not. […] They may have abdominal distention (which is tympanic on percussion) and symptoms of intestinal obstruction. […] They may present with acute enterocolitis in this age group and (rarely) with neonatal meconium plug syndrome or appendicitis. […] They have chronic constipation. […] There may be marked abdominal distention with palpable dilated loops of colon. […] Rectal examination often reveals an empty rectum and may result in the forceful expulsion of faecal material as examination is completed.

• #2 Hirschsprung Disease | Texas Children’s

  https://www.texaschildrens.org/content/conditions/hirschsprung-disease

  Hirschsprung disease is a condition children are born with that stops or slows the movement of food and waste through the intestine. It affects about 1 in 5,000 children. It is caused by a problem with the nerve cells in the rectum or colon (the lower part of the intestine). Normally these nerves help the muscles in the wall of the bowel squeeze and relax, pushing food and waste along the way. When these nerves are missing, the bowel cannot push the digested food through, and children cannot have normal bowel movements or have fewer bowel movements than normal. […] Symptoms can vary depending on how much of the intestine is affected. Children with a very small portion of intestine involved may not have symptoms until they are several months or even years old. Children with a longer segment of intestine involved may have symptoms earlier in life or have more severe symptoms. Common symptoms include: No stool in the first 2 days of a baby’s life, Swelling or bloating of the abdomen, Constipation, Vomiting, Fever. […] Older children may have these symptoms: Chronic constipation, Poor growth (failure to thrive), Passage of small, watery stools, Poor appetite.

• #2 Hirschsprung Disease in Children | Phoenix Children’s Hospital

  https://phoenixchildrens.org/specialties-conditions/hirschsprung-disease-children

  Most babies with Hirschsprung disease have symptoms in the first few weeks of life. […] Each child’s symptoms may vary. Symptoms in newborns may include: Not having a bowel movement in the first 48 hours of life, Slow swelling or bloating of the belly, Vomiting green or brown fluid. […] Children who don’t show early symptoms may also have: Constipation that gets worse over time, Loss of appetite, Slow or delayed growth, Small, watery, bloody stools, Loss of energy. […] Symptoms of Hirschsprung disease may seem like other health problems. […] In Hirschsprung disease, a part of the large intestine lacks normal nerve cells. This means that digested food and stool can’t move forward through that part of the digestive tract. The large intestine becomes blocked with stool. Your baby will be constipated, or unable to have normal bowel movements.

• #2 Hirschsprung Disease in Children | Phoenix Children’s Hospital

  https://phoenixchildrens.org/specialties-conditions/hirschsprung-disease-children

  The blockage creates pressure on the inside of the intestine. This causes part of the intestinal wall to wear thin. Over time, a bacterial infection called enterocolitis can develop in the digestive tract. This is very serious. Symptoms of enterocolitis include: Fever, Swollen belly, Belly pain, Vomiting, Diarrhea, Bleeding from the rectum, Lack of energy. […] Your child’s bowel function may be affected after surgery. The most common long-term problems include bowel control and leaking stool, constipation, and infections. […] Problems that may occur after surgery depend on how much of the intestine lacked nerve cells and how much of it was removed. […] Children who are able to have their ostomy closed may have short-term problems after the closure, including: Stools may be frequent and loose at first.

• #2 Total Colonic Hirschsprung Disease | Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/t/total-colonic-hirschsprung-disease

  Infants usually begin having symptoms during the first 24 to 48 hours of life. While infants may experience a range of symptoms, the following are the most common: […] Not having a bowel movement in the first 48 hours of life […] Gradual swelling of the belly […] Gradual onset of vomiting […] Fever […] Sepsis (overwhelming infection) […] Constipation that worsens over time […] Small, watery stool.

• #2 Hirschsprung’s Disease; Signs, Symptoms, and Treatments | Worldwide Pediatrics Group

  https://www.toplinemd.com/worldwide-pediatrics/hirschsprungs-disease-signs-symptoms-and-treatments/

  In cases where symptoms do not manifest in the early weeks, older children may exhibit different signs, including: Worsening Constipation: Constipation becomes progressively severe. Loss of Appetite: Affected children may lose interest in eating, resulting in delayed growth. Passing Small, Watery Stools: Stools may become smaller and watery. Abdominal Distension: Bloating of the abdomen. […] The primary treatment for Hirschsprung’s disease is surgery. The surgical procedure aims to remove the diseased section of the intestine and pull the healthy portion down to the anus, effectively restoring bowel function. In most cases, these surgeries can be performed with minimally invasive techniques, and some can be done entirely through the anus, leaving no visible scars. […] However, it’s important to note that some children may continue to experience issues following surgery. The extent of the unhealthy intestine removed during surgery and the current functioning of the colon, rectum, and anus can influence the post-operative experience. Potential complications and long-term issues include intractable constipation, diarrhea, stool accidents, recurrent infections (enterocolitis), severe abdominal distension, difficulty tolerating food, vomiting, and growth-related problems.

• #2 Hirschsprung Disease | Children’s Hospital Pittsburgh

  https://www.chp.edu/our-services/surgery-pediatric/conditions-we-treat/hirschsprungs-disease

  Many children with Hirschsprung disease have a normal lifestyle after a pull-through procedure. Some children may need long-term follow up for problems with constipation or fecal incontinence (soiling). […] About a third of children who undergo pull-through surgery may at some point show symptoms of intestinal obstruction, called enterocolitis. Symptoms can include constipation, bloating, smelly loose stools, vomiting, and fever. These should be treated immediately.

• #2 Hirschsprung Disease in Children – Stanford Medicine Children’s Health

  https://www.stanfordchildrens.org/en/topic/default?id=hirschsprung-disease-in-children-90-P01999

  The blockage creates pressure on the inside of the intestine. This causes part of the intestinal wall to wear thin. Over time, a bacterial infection called enterocolitis can develop in the digestive tract. This is very serious. Symptoms of enterocolitis include: Fever, Swollen belly, Vomiting, Diarrhea, Bleeding from the rectum, Lack of energy. […] Your child’s bowel function may be affected after surgery. The most common long-term problems include bowel control and leaking stool, constipation, and infections. […] Problems that may occur after surgery depend on the amount of intestine that lacked nerve cells, and how much intestine was removed. […] Children who had a large section of intestine removed may have long-term problems. The digestive process can be affected. Nutrients and fluids are absorbed from food in the small intestine. Removing a large segment of the intestine can prevent a child from getting enough nutrients and fluids. Children can have problems with poor digestion, slow growth, and infection. Your child may need to eat and drink more in order to get enough nutrients and fluid.

• #2 Hirschsprung Disease in Children | Valley Children’s Healthcare

  https://www.valleychildrens.org/services/pediatric-surgery/conditions-we-treat/hirschsprung-disease

  The blockage creates pressure on the inside of the intestine. This causes part of the intestinal wall to wear thin. Over time, a bacterial infection called enterocolitis can develop in the digestive tract. This is very serious. Symptoms of enterocolitis include: Fever, Swollen belly, Belly pain, Vomiting, Diarrhea, Bleeding from the rectum, Lack of energy. […] Your child’s bowel function may be affected after surgery. The most common long-term problems include bowel control and leaking stool, constipation, and infections. […] Problems that may occur after surgery depend on how much of the intestine lacked nerve cells and how much of it was removed. […] Children who are able to have their ostomy closed may have short-term problems after the closure, including: Stools may be frequent and loose at first.

• #3 Hirschsprung’s Disease: What It Is, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/9844-hirschsprung-disease

  Some babies with Hirschsprungs disease have blocked intestines at birth. Theres a chance your baby has Hirschsprungs disease if they dont poop within 48 hours after birth. […] Other symptoms of Hirschsprungs disease in babies may include: Swollen belly. Constipation. Vomiting. Diarrhea. Lack of appetite (refusing to eat) and poor weight gain. Delayed growth. […] Without treatment, Hirschsprungs disease can cause serious complications. […] Up to 40% of children with Hirschsprungs disease develop enterocolitis. This condition is the swelling of your childs small and large intestines. Some children have mild inflammation. Others have severe symptoms that can be life-threatening. If your child has severe enterocolitis from Hirschsprungs disease, they may have fever and explosive diarrhea.

• #3 Hirschsprung’s Disease: Diagnosis and Management | AAFP

  https://www.aafp.org/pubs/afp/issues/2006/1015/p1319.html

  Hirschsprungs disease most commonly involves the rectosigmoid region of the colon but can affect the entire colon and, rarely, the small intestine. The disease usually presents in infancy, although some patients present with persistent, severe constipation later in life. Symptoms in infants include difficult bowel movements, poor feeding, poor weight gain, and progressive abdominal distention. […] Symptoms range from neonatal intestinal obstruction to chronic progressive constipation in older children. Approximately 80 percent of patients present in the first few months of life with difficult bowel movements, poor feeding, and progressive abdominal distention. Up to 90 percent of infants with Hirschsprungs disease fail to pass meconium in the first 24 hours of life; however, other causes of this delay also should be considered.

• #3 Hirschsprung’s Disease | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/hirschsprungs-disease

  Hirschsprung’s disease (also called congenital aganglionic megacolon) occurs when some of the intestinal nerve cells (ganglion cells) dont develop properly, delaying the progression of stool through the intestines. […] The intestine becomes blocked with stool, and your baby or child will be constipated (unable to have normal bowel movements). Often, a serious infection called enterocolitis can occur, which causes fever, pain, and diarrhea. […] Symptoms of Hirschsprung’s disease vary with age. Eighty percent of children with Hirschsprung’s disease have symptoms in the first six weeks of life. However, children who only have a short segment of intestine that lacks normal nerve cells may not exhibit symptoms for several months or years. Their primary symptom is constipation. […] Each child may experience symptoms differently, but common symptoms in infants include: Failure to have a bowel movement in the first 48 hours of life, Abdominal distention (stomach bloating), Gradual onset of vomiting, Fever, Constipation or failure to pass regular bowel movements.

• #3 About hirschsprungs disease | Children’s Wisconsin

  https://childrenswi.org/medical-care/gastroenterology-liver-and-nutrition-program/conditions/hirschsprungs-disease

  Eighty percent of children with Hirschsprung’s disease show symptoms in the first 6 weeks of life. […] Children who only have a short segment of intestine that lacks normal nerve cells may not show symptoms for several months or years. The following are the most common symptoms of Hirschsprung’s disease. However, each individual may experience symptoms differently. Symptoms may include: Not having a bowel movement in the first 48 hours of life. Gradual bloating of the abdomen. Gradual onset of vomiting. Fever. […] Children who do not have early symptoms may also present the following: Constipation that becomes worse with time. Loss of appetite. Delayed growth. Passing small, watery stools. […] Symptoms of Hirschsprung’s disease may resemble other conditions or medical problems. Please consult your child’s physician for a diagnosis.

• #3 Symptoms & Causes of Hirschsprung Disease – NIDDK

  https://www.niddk.nih.gov/health-information/digestive-diseases/hirschsprung-disease/symptoms-causes

  Symptoms of Hirschsprung disease vary. Signs or symptoms in newborns may be different than symptoms in older infants, children, and adults. […] Some infants with Hirschsprung disease have signs and symptoms of intestinal obstruction shortly after they are born, which include not having a first bowel movement within 48 hours after birth, green or brown vomit, swollen abdomen, or belly, problems feeding, explosive stools or diarrhea after a doctor inserts a finger into the newborns rectum. […] Children with Hirschsprung disease who dont have signs and symptoms of intestinal obstruction at birth may not be diagnosed and treated until they are older. Common symptoms in older infants and children include chronic constipation that doesnt get better after treatment with oral laxatives, swollen abdomen, malnutrition or failure to thrive, meaning that an infant or child weighs less or is gaining less weight than expected for his or her age, symptoms of Hirschsprung-associated enterocolitis, such as diarrhea. […] In rare cases, Hirschsprung isnt diagnosed and treated until adulthood. Adults with Hirschsprung disease typically have a history of abdominal swelling and chronic constipation that doesnt get better after taking oral laxatives.

• #3 Hirschsprung’s Disease Treatment & Care | Dayton Children’s Hospital

  https://www.childrensdayton.org/patients-visitors/services/pediatric-surgery/conditions-we-treat/hirschsprung-disease

  Because Hirschsprung disease can affect the body’s ability to absorb nutrients, growth may be affected. […] Children with Hirschsprung disease also continue to be at risk of developing bowel infection (enterocolitis) after surgery, especially in the first year. Signs and symptoms of enterocolitis can include: bleeding from the rectum, diarrhea, fever, swollen abdomen, and vomiting. You should call your child’s doctor immediately if any of these signs and symptoms occur.

• #3 Hirschsprung Disease: Background, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/178493-overview

  Total colonic aganglionosis is associated with a poorer outcome, with 33% of patients experiencing persistent incontinence and 14% requiring a permanent ileostomy. Patients with associated chromosomal abnormalities and syndromes also have poorer clinical outcomes. […] Hirschsprung disease is confined to the rectosigmoid region in about 75% of cases. Approximately 60% of infants with Hirschsprung disease have an associated condition, ranging from subtle to severe. […] Enterocolitis accounts for significant morbidity and mortality in patients with Hirschsprung disease and can progress into toxic megacolon. […] Approximately 10%-30% of patients with Hirschsprung disease develop enterocolitis. Long-segment disease is associated with an increased incidence of enterocolitis. The risk of enterocolitis does not decrease with surgical correction. […] Patients may present postoperatively with abdominal distention, emesis, or constipation indicative of ongoing obstruction. […] Persistent aganglionosis occurs rarely and may be due to pathologic error, inadequate resection, or loss of ganglion cells after the pull-through procedure.

• #3 Hirschsprung’s Disease | Symptoms, Diagnosis, Treatment & FAQs

  https://www.londonchildrensurgery.co.uk/hirschsprungs-disease.php

  Many babies with HD experience long-term problems such as diarrhoea, constipation, soiling and abdominal pain and you may find that your child takes longer to toilet train. Sometimes medicines are needed to help with these problems. You will have specialist advice and support to manage and improve any symptoms in the longer term. Following discharge from hospital, your baby will be seen in the outpatients department by members of the surgical team, where help and advice will be available to you. Follow up appointments will usually occur on a regular basis for some years.

• #3 Hirschsprung Disease in Children | Valley Children’s Healthcare

  https://www.valleychildrens.org/services/pediatric-surgery/conditions-we-treat/hirschsprung-disease

  Children may have trouble sensing the need to have a bowel movement. […] Some children have problems with bowel movements because the anal opening is tight. […] Children who had a large part of the intestine removed may have long-term problems. The digestive process can be affected. Nutrients and fluids are absorbed from food in the small intestine. Removing a large segment of the intestine can prevent a child from getting enough nutrients and fluids. Children can have problems with poor digestion, slow growth, and infection. Your child may need to eat and drink more to get enough nutrients and fluid.

• #4 Hirschsprung Disease in Children – Stanford Medicine Children’s Health

  https://www.stanfordchildrens.org/en/topic/default?id=hirschsprung-disease-in-children-90-P01999

  Most babies with Hirschsprung disease have symptoms in the first few weeks of life. […] Each child’s symptoms may vary. Symptoms in newborns may include: Not having a bowel movement in the first 48 hours of life, Slow swelling or bloating of the belly, Vomiting green or brown fluid. […] Children who don’t show early symptoms may also have: Constipation that gets worse over time, Loss of appetite, Slow or delayed growth, Small, watery, bloody stools, Loss of energy. […] Symptoms of Hirschsprung disease may seem like other health problems. […] In Hirschsprung disease a part of the large intestine lacks normal nerve cells. This means that digested food and stool can’t move forward through that part of the digestive tract. The large intestine becomes blocked with stool. Your baby will be constipated, or unable to have normal bowel movements.

• #4 Hirschsprung’s disease | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/hirschsprungs-disease/

  Signs and symptoms of Hirschsprung’s disease vary with the severity of the condition. Usually signs and symptoms appear shortly after birth, but sometimes they’re not apparent until later in life. […] Typically, the most obvious sign is a newborn’s failure to have a bowel movement within 48 hours after birth. […] Other signs and symptoms in newborns may include: Swollen belly, Vomiting, including vomiting a green or brown substance, Constipation or gas, which might make a newborn fussy, Diarrhea, Delayed passage of meconium a newborn’s first bowel movement. […] In older children, signs and symptoms can include: Swollen belly, Chronic constipation, Gas, Failure to thrive, Fatigue.

• #4 Persistent Symptoms After Surgery for Hirschsprung’s Disease | Children’s National Hospital

  https://www.childrensnational.org/get-care/departments/colorectal/provider-resources/persistent-symptoms-of-hirschsprung-disease

  Unfortunately, sphincter weakness and abnormal bowel sensation cannot usually be repaired with surgery, which is why symptoms may persist afterwards. […] Bowel obstruction symptoms include: Abdominal distention, Bloating, Borborygmi (stomach growling), Vomiting, Ongoing severe constipation, Difficulty passing stools without the assistance of rectal stimulation or irrigations. […] There can be many reasons children continue to experience bowel obstruction symptoms after surgery. […] Symptoms of HAEC include: Bloating, Severe diarrhea, Vomiting, Fever, Lethargy, Poor feeding, Rectal bleeding. […] Although surgical intervention can improve their quality of life, children with Hirschsprung’s disease may still experience soiling, obstructive symptoms or enterocolitis following a pull-through surgery. However, the prognosis for children with persistent symptoms is very good. By applying an organized and effective therapeutic approach to treatment and care, most children respond very well and many of these problems improve as the child gets older.

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