Constipation is difficult to define and its meaning differs from person to person. For some, there may be a decrease in stool frequency, for some, very hard and difficult defecation, and for some, there may be pain when defecating. It can be defined simply as difficulty or delay in defecation. In the first weeks after birth, the frequency of defecation in children decreases from an average of four per day, to two by the age of two, and to one by the age of four.
Poop incontinence
various pictures
When constipation is defined by stool frequency, less than three bowel movements per week is considered. The definition of constipation according to the Roman criteria in infants and children differs from adults and includes:
1. Pebble-like hard stools with most stools for at least two weeks
2. Two or fewer hard stools per week for at least two weeks
3. Absence of a structural, endocrinological or metabolic disease.
Approximately 3% of preschool children and 1-2% of school-age children complain of constipation. The reported incidence varies between 0.3-8%. In addition, constipation patients constitute 3-5% of general pediatric outpatient clinics and up to 25% of pediatric gastroenterology clinics. Soiling is the involuntary leakage of stool and different terms are used in relation to it. Of these, incontinence is used when there is an underlying disease (anatomical, organic or inflammatory, such as meningomyelocele, mass pressing on the spinal cord, ulcerative colitis). This condition is responsible for less than 5% of children with incontinence. Encopresis is the involuntary leakage of shaped, semi-shaped, or liquid stools in children older than four years of age, secondary to constipation. More than 95% of fecal incontinence cases are caused by encopresis. It is more common in men. Primary encopresis describes children who have never developed stool control, and secondary encopresis defines children who start to have stool incontinence after developing stool control at some point in their life. Although these are the most commonly used definitions, some define encopresis as the incontinence of stools in a near-normal amount and consistency in inappropriate places, and blame more on psychological-developmental abnormalities. In recent years, the term “nonretentive fecal soiling” has been used for encopresis.
Dyschesia describes excessive straining during defecation. To describe children who strain for hours to pass small amounts of stool, “ineffective defecation”, “anismus”, “anal achalasia”, “emptying obstruction”, “pelvic floor dyssynergia”, “anal sphincter dysfunction”, pelvic-floor dysfunction, spastic pelvic-floor syndrome is used in terms such as “functional stool accumulation of children”. In this case, the child contracts during defecation when he should relax his external anal sphincter and perineal muscles. It is not known whether it develops as a conditioned response to painful defecation or whether it is a developmental event.
In developed countries, the frequency of fecal incontinence is 3% at the age of five, 1.5% at the age of seven, and 0.8% at the age of 12. In a study conducted in our country, the frequency of encopresis in children was found to be 0.43% (boy/girl: 2.28). It is 0.23% in children over the age of nine. . The fact that it is seen less often may be due to the fact that animal foods take up less place in our country’s diet, the use of Turkish-style toilets that provide more effective straining in many places, or the fact that families do not hide the situation.
Causes and etiopathogenesis
When the cause of constipation is investigated, no cause is found in more than 95% of children and these are called functional constipation. The remaining 5% have different causes (Table 2). Here, the pathogenesis of functional constipation will be discussed. An acute cause (dietary change, environmental change, febrile illness, dehydration, bed-ridden, pain in defecation due to any reason) can be detected in the beginning when constipation starts in infancy, when a careful history is taken. More than 2/3 of these children have pain during defecation, and some have anal fissures. This pain causes the desire to delay/prevent defecation, causing contraction of the external anal sphincter and accumulation of stool in the rectum. Stool that accumulates in the rectum becomes harder as a result of continued fluid absorption and causes more pain at the next defecation. Thus, a vicious circle begins. Every stool passing through the rectum creates fear in the child, and the child consciously or unconsciously holds his stool to avoid this painful defecation. During this stool retention, the child may have abnormal postures. In these stages, the child tenses his gluteal muscles, blushes, crouches in a corner, or crosses his legs in order to hold his stool; may cry due to pain; shouting, blushing, and movements of hyperextension and flexion of the legs may be observed. These behaviors are often misinterpreted by the family as an effort to defecate. Over time, this behavior becomes automatic. As the event progresses, the rectum enlarges and the megarectum develops. It should be kept in mind that cow’s milk can cause constipation, especially in children whose constipation starts during the weaning period. Cow’s milk elimination should be attempted in treatment in children who do not respond to other treatment modalities. A problem that can be encountered when toilet training starts is refusal to toilet training. These children urinate into the toilet but do not accept to defecate into the toilet. When their glands are tied, they defecate to their standing diapers. This condition can rarely last for years. These children are prone to being constipated. The thing to do is to tie the diaper again, although some families are not willing. 89% of cases start using the toilet within three months. Children who tend to hide while defecating before completing toilet training are more likely to refuse toilet training, become constipated and hold their stools. When a careful history is taken in older children, more than 80% of them have a triggering stressful event (birth of a sibling, divorce of parents, death of grandparents, moving house, starting school). When the behaviors and attitudes within the family are examined, the fact that there is complete autonomy in eating and sleeping habits as well as not providing the child with autonomy is an increasing factor. Since it is usually the fathers who set the rules in our family structure, the absence of paternal authority can create a symbiotic relationship between the child and the mother. Depending on the age, the child may want to delay the need to defecate when he/she engages in activities he/she enjoys, such as playing with toys, playing computer games, or when he/she cannot use his/her own toilet. Especially at school age, the insufficient level of school toilets (such as cleanliness, the number of toilets, the lack of separation between boys and girls in some places) can lead to many problems, including constipation. After all, the vicious circle mentioned above can start at any age. The main organic causes of constipation are given in Table 2. Constipation is seen in 1/3 of children with neurological problems. The main reason for this is the inadequacy of neurological control systems, insufficient fiber intake in the diet, some medications (anticholinergics, opiates) and limitation of movement. Whatever the cause, illness and inactivity can be the onset of acute constipation in children. Constipation is also more common in autistic children. Under normal conditions, the rectum is empty and stool entering the rectum creates the need to defecate. In children with chronic constipation, the rectum is enlarged (megarectum) and stool is full. The rectum of these children is insensitive to incoming stool and the child does not need to defecate. When the accumulated stool reaches a pressure greater than the anal sphincter can handle, the stool will spontaneously escape, soiling the underwear. There may be abdominal pain and distention together. Loss of appetite, nausea and vomiting may occur due to delayed gastric emptying. Psychological changes can be detected in the child.
Evaluation
Story: The most important point is when the first defecation occurs after birth. Observation of the first meconium after 48 hours requires investigation. Even if they do not have Hirschsprung’s disease (HH), approximately 40% of constipated children have their first meconium exit after 24 hours. More than half of the parents have a family history of constipation. When constipation started, whether there was an acute onset, medications used (especially for enuresis), soiling of underwear, rectal bleeding, nausea, vomiting, abdominal pain, abdominal bloating, pain in the anal region (especially
during defecation), loss of appetite, insufficient weight gain and behavioral changes should be asked. Generally speaking, about 1/3 of the cases are chronic. Again, although 30-50% of the cases start before the age of two, the first thing that attracts parents’ attention or leads them to seek help is the delay in toilet training and incontinence of the child after the age of two. It is more common in men. Ongoing constipation and/or fecal incontinence affects the child physically, psychologically, educationally and socially and reduces self-confidence. Sometimes children are brought to the doctor with the complaint of diarrhea, not constipation. The reason for this is the leakage of the liquid part formed around the impacted stool in the rectum. The amount and consistency of stool varies depending on the cause of constipation. Those with slowed colonic transit time have more and softer stools, while those with functional rectal retention have fewer and harder stools. If there is normal-appearing stool incontinence, evidence of an error or neglect in toilet training (growth arrest, speech and motor delay, physical abuse findings) should be sought.
Physical examination: With palpation, stool accumulation in the colon can be detected. The perineum should be examined for fissure, skin disease, analectopia and sacral agenesis. If urinary incontinence is also present, spine and sacral region examination should be performed. The newborn who does not remove meconium in the first 48 hours should be evaluated and investigated for anal atresia, anal stenosis, HH and cystic fibrosis. After rectal examination in HH, spitting can be seen when the finger is pulled. Biliary vomiting, abdominal distention, sometimes bloody diarrhea due to enterocolitis may be detected. Older children may have abdominal distention and growth retardation. Passage of meconium plugs is suggestive of cystic fibrosis.
Laboratory: Laboratory examination is not required for the diagnosis of functional constipation. Most of the examinations performed serve to distinguish the severity of the condition, some subtypes and make a differential diagnosis. If an organic cause is considered by history and physical examination, tests for the diagnosis of the suspected disease should be performed (Table 2).
Radiology: In functional constipation, it is performed only to show the transit time or to observe the amount of accumulated stool. Eight radiopaque markers are drunk for three consecutive days for the transit time, and on the fifth day, a plain film is shot and according to the number of markers seen in the film; severe delay, moderate delay, mild delay and normal. Also, where the markers stay is important. Deposition in the rectosigmoid region is evaluated as “exit obstruction” and distribution throughout the entire colon as “pancolonic delay”. Showing the movies to the family can provide a better understanding of the problem. Normal transit time does not rule out constipation. This radiological study can also be used to evaluate response to treatment. The absence of a radiopaque marker in a child with ongoing abduction indicates an overdose of the drug, while the persistence of symptoms indicates inadequate treatment or unresponsiveness. Transit time can also be demonstrated by radionuclide scintigraphy.
Manometry: Ineffective straining can be demonstrated in older children. During straining, contraction of the external sphincter is observed instead of relaxation. HH is one of the diseases that should be kept in mind in the differential diagnosis. HH occurs in 1 in 5,000 live births (3% of children of sick parents). The male/female ratio is 4. While 94-98% of term babies and 76% of preterm babies remove meconium in the first 24 hours, 94% of H-Holans do not. Almost all normal infants and 98% of preterm infants excrete meconium in the first 48 hours. The neonatal infant’s failure to expel meconium in the first 48 hours, vomiting (which may be biliary), and presence of abdominal distension indicate HH. The main pathology is the absence of ganglion cells in the affected area. Aganglionosis is in the rectosigmoid region at a rate of 70-80%, with total colonic involvement in 15% of the patients. When these symptoms are absent or missed, the child may present with a septic picture (enterocolitis picture) with vomiting, distention and diarrhea. Less frequently, the sick child may present with growth retardation and constipation. In older children, the presence of severe constipation, growth retardation, abdominal distention, and often an empty rectum on rectal examination suggest the diagnosis. Fecal incontinence is usually absent. Delayed emptying of barium from the colon is also helpful in diagnosis. X-ray may be insufficient in recognizing short lesions, total colonic aganglionosis, and neonatal disease. Its treatment is removal of the aganglionic segment and retraction of the normal part into the anal canal.
Constipation may continue after the operation and stool incontinence may develop. The typical appearance on barium radiography is narrow segment (the diseased part) and dilatation in the proximal colon. Internal anal sphincter achalasia (IASA), formerly called very short segment HH, is characterized by the absence of a rectoanal inhibitory reflex on a motility study despite normal rectal biopsy. The innervation of the internal anal sphincter is defective. The general appearance is constipation, which may be accompanied by stool incontinence. Internal sphincter myectomy is successful in its treatment.
Treatment (Consult your doctor)
Although the time elapsed between the onset of symptoms and initiation of treatment has not been fully proven, it does affect success. The earlier the treatment is started, the better the prognosis. A concern for families is that the child has an underlying chronic illness. If these families and their age are appropriate, the child should be convinced that the event is functional. Although the view that behavioral problems are more common in children with constipation is controversial, it is known that those with behavior problems have worse response to treatment. For this reason, medical and psychological approaches should be given together in treatment. The basis of treatment is “empty the colon and keep it empty”. It is aimed to prevent the accumulation of feces for a long time after the colon is emptied, to return the anatomical structure to normal and to regain the sensitivity of the rectum.
·Disimpaction of the colon: There are many drugs used to provide evacuation. If necessary, manual evacuation can be performed under an enema and, in some cases, general anesthesia.
·Maintenance treatment: After the rectosigmoid region is emptied, the aim is to prevent stool from accumulating in the colon and to return the rectum to its normal size. The aim is to defecate at least once or twice a day with a normal consistency. The child should be seated on the toilet for five minutes after each meal. In the meantime, keeping a diary and marking the number of stools per day and incontinence is helpful in monitoring the response to treatment as well as encouraging the child. The main drugs used in the treatment, their doses and side effects
are given in Table 3, and the fiber contents of some nutrients are given in Table 4. In addition to drug therapy, toilet training, psychological therapy and, in some cases, biofeedback therapy can be applied. Biofeedback is teaching the child the normal defecation pattern (increasing intra-abdominal pressure by pushing and relaxing the external sphincter) by using manometry and/or electromyography (EMG). Biofeedback is useful in those with pelvic-floor dyssynergia. Thus, children are taught the normal way of defecation by showing that the external anal sphincter should be relaxed while straining.
GIS: Gastrointestinal system.
Anorectal functions and colonic transit time are normal in manometry. In these, it is seen that the stool that is missed in the history is in normal amount. Dabiofeedback is usually more successful in these children. Biofeedback is of no use unless there is a defect in the defecation mechanism. Since the resting pressure of the internal anal sphincter is high in children with fissures (whether cause or effect), lowering this pressure is recommended in treatment. Attention should be paid to the presence of perianal infection, as it will cause pain and avoid defecation. Recurrence rates as high as 50-60% have been reported in some studies. With medical treatment, psychological support and changing behavior, the chance of success rises up to 88%. However, relapses may occur in up to half of the children, and in this case, short-term medical treatment should be given. Success is directly dependent on the adaptation of the family and the child to the program. After the rectum is fully emptied, toilet training should be provided, daily emptying should be ensured by using stool softeners and accumulation should be prevented. This period takes at least six months. After this period, it is important to discontinue the drugs under supervision. When it is thought that there is not enough ejaculation, the drug should be started again.
·Surgical treatment: Less than 5% of patients may require surgical treatment.
Table 1. Frequency of normal stools in children
Age Weekly stool frequency Daily stool frequency
0-3 months
Breast milk 5-40 2.9
Formula 5-28 2.0
6-12 months 5-28 1.8
1-3 years 4-21 1.4
> 3 years 3-14 1.0
![]()
1. Functional: 95%
2. Organic
a. Anatomical
Imperforate anus
Anal stenosis
Anus anterior location
Pelvic mass (teratoma etc.)
b. Metabolic and gastrointestinal
Hypothyroidism
Hypercalcemia
Hypokalemia
Cystic fibrosis
Diabetes mellitus
Multiple endocrine neoplasia type 2B
Celiac disease
Renal tubular acidosis
c. Neuropathic diseases
Spinal cord abnormalities
Spinal cord trauma
Neurofibromatosis
Encephalopathy
Tethered cord
Cerebral palsy
d. Intestinal nerve and muscle disorders
Hirschsprung’s disease
Intestinal neuronal dysplasia
Intestinal pseudoobstruction
Visceral myopathies
Visceral neuropathies
e. Abnormal abdominal musculature
Prune belly syndrome
Gastroschisis
Down syndrome
f. Connective tissue diseases
Scleroderma
Systemic lupus erythematosus
Ehlers-Danlos syndrome
g. Drugs
Opiates
Phenobarbital
Sucralfate
Antacids
Antihypertensives
Anticholinergics
Antidepressants
Sympathomimetics
h. Other
Heavy metal poisoning (lead)
Vitamin D poisoning
Botulism
Cow’s milk protein intolerance
Poop incontinence
various pictures
When constipation is defined by stool frequency, less than three bowel movements per week is considered. The definition of constipation according to the Roman criteria in infants and children differs from adults and includes:
1. Pebble-like hard stools with most stools for at least two weeks
2. Two or fewer hard stools per week for at least two weeks
3. Absence of a structural, endocrinological or metabolic disease.
Approximately 3% of preschool children and 1-2% of school-age children complain of constipation. The reported incidence varies between 0.3-8%. In addition, constipation patients constitute 3-5% of general pediatric outpatient clinics and up to 25% of pediatric gastroenterology clinics. Soiling is the involuntary leakage of stool and different terms are used in relation to it. Of these, incontinence is used when there is an underlying disease (anatomical, organic or inflammatory, such as meningomyelocele, mass pressing on the spinal cord, ulcerative colitis). This condition is responsible for less than 5% of children with incontinence. Encopresis is the involuntary leakage of shaped, semi-shaped, or liquid stools in children older than four years of age, secondary to constipation. More than 95% of fecal incontinence cases are caused by encopresis. It is more common in men. Primary encopresis describes children who have never developed stool control, and secondary encopresis defines children who start to have stool incontinence after developing stool control at some point in their life. Although these are the most commonly used definitions, some define encopresis as the incontinence of stools in a near-normal amount and consistency in inappropriate places, and blame more on psychological-developmental abnormalities. In recent years, the term “nonretentive fecal soiling” has been used for encopresis.
Dyschesia describes excessive straining during defecation. To describe children who strain for hours to pass small amounts of stool, “ineffective defecation”, “anismus”, “anal achalasia”, “emptying obstruction”, “pelvic floor dyssynergia”, “anal sphincter dysfunction”, pelvic-floor dysfunction, spastic pelvic-floor syndrome is used in terms such as “functional stool accumulation of children”. In this case, the child contracts during defecation when he should relax his external anal sphincter and perineal muscles. It is not known whether it develops as a conditioned response to painful defecation or whether it is a developmental event.
In developed countries, the frequency of fecal incontinence is 3% at the age of five, 1.5% at the age of seven, and 0.8% at the age of 12. In a study conducted in our country, the frequency of encopresis in children was found to be 0.43% (boy/girl: 2.28). It is 0.23% in children over the age of nine. . The fact that it is seen less often may be due to the fact that animal foods take up less place in our country’s diet, the use of Turkish-style toilets that provide more effective straining in many places, or the fact that families do not hide the situation.
Causes and etiopathogenesis
When the cause of constipation is investigated, no cause is found in more than 95% of children and these are called functional constipation. The remaining 5% have different causes (Table 2). Here, the pathogenesis of functional constipation will be discussed. An acute cause (dietary change, environmental change, febrile illness, dehydration, bed-ridden, pain in defecation due to any reason) can be detected in the beginning when constipation starts in infancy, when a careful history is taken. More than 2/3 of these children have pain during defecation, and some have anal fissures. This pain causes the desire to delay/prevent defecation, causing contraction of the external anal sphincter and accumulation of stool in the rectum. Stool that accumulates in the rectum becomes harder as a result of continued fluid absorption and causes more pain at the next defecation. Thus, a vicious circle begins. Every stool passing through the rectum creates fear in the child, and the child consciously or unconsciously holds his stool to avoid this painful defecation. During this stool retention, the child may have abnormal postures. In these stages, the child tenses his gluteal muscles, blushes, crouches in a corner, or crosses his legs in order to hold his stool; may cry due to pain; shouting, blushing, and movements of hyperextension and flexion of the legs may be observed. These behaviors are often misinterpreted by the family as an effort to defecate. Over time, this behavior becomes automatic. As the event progresses, the rectum enlarges and the megarectum develops. It should be kept in mind that cow’s milk can cause constipation, especially in children whose constipation starts during the weaning period. Cow’s milk elimination should be attempted in treatment in children who do not respond to other treatment modalities. A problem that can be encountered when toilet training starts is refusal to toilet training. These children urinate into the toilet but do not accept to defecate into the toilet. When their glands are tied, they defecate to their standing diapers. This condition can rarely last for years. These children are prone to being constipated. The thing to do is to tie the diaper again, although some families are not willing. 89% of cases start using the toilet within three months. Children who tend to hide while defecating before completing toilet training are more likely to refuse toilet training, become constipated and hold their stools. When a careful history is taken in older children, more than 80% of them have a triggering stressful event (birth of a sibling, divorce of parents, death of grandparents, moving house, starting school). When the behaviors and attitudes within the family are examined, the fact that there is complete autonomy in eating and sleeping habits as well as not providing the child with autonomy is an increasing factor. Since it is usually the fathers who set the rules in our family structure, the absence of paternal authority can create a symbiotic relationship between the child and the mother. Depending on the age, the child may want to delay the need to defecate when he/she engages in activities he/she enjoys, such as playing with toys, playing computer games, or when he/she cannot use his/her own toilet. Especially at school age, the insufficient level of school toilets (such as cleanliness, the number of toilets, the lack of separation between boys and girls in some places) can lead to many problems, including constipation. After all, the vicious circle mentioned above can start at any age. The main organic causes of constipation are given in Table 2. Constipation is seen in 1/3 of children with neurological problems. The main reason for this is the inadequacy of neurological control systems, insufficient fiber intake in the diet, some medications (anticholinergics, opiates) and limitation of movement. Whatever the cause, illness and inactivity can be the onset of acute constipation in children. Constipation is also more common in autistic children. Under normal conditions, the rectum is empty and stool entering the rectum creates the need to defecate. In children with chronic constipation, the rectum is enlarged (megarectum) and stool is full. The rectum of these children is insensitive to incoming stool and the child does not need to defecate. When the accumulated stool reaches a pressure greater than the anal sphincter can handle, the stool will spontaneously escape, soiling the underwear. There may be abdominal pain and distention together. Loss of appetite, nausea and vomiting may occur due to delayed gastric emptying. Psychological changes can be detected in the child.
Evaluation
Story: The most important point is when the first defecation occurs after birth. Observation of the first meconium after 48 hours requires investigation. Even if they do not have Hirschsprung’s disease (HH), approximately 40% of constipated children have their first meconium exit after 24 hours. More than half of the parents have a family history of constipation. When constipation started, whether there was an acute onset, medications used (especially for enuresis), soiling of underwear, rectal bleeding, nausea, vomiting, abdominal pain, abdominal bloating, pain in the anal region (especially
during defecation), loss of appetite, insufficient weight gain and behavioral changes should be asked. Generally speaking, about 1/3 of the cases are chronic. Again, although 30-50% of the cases start before the age of two, the first thing that attracts parents’ attention or leads them to seek help is the delay in toilet training and incontinence of the child after the age of two. It is more common in men. Ongoing constipation and/or fecal incontinence affects the child physically, psychologically, educationally and socially and reduces self-confidence. Sometimes children are brought to the doctor with the complaint of diarrhea, not constipation. The reason for this is the leakage of the liquid part formed around the impacted stool in the rectum. The amount and consistency of stool varies depending on the cause of constipation. Those with slowed colonic transit time have more and softer stools, while those with functional rectal retention have fewer and harder stools. If there is normal-appearing stool incontinence, evidence of an error or neglect in toilet training (growth arrest, speech and motor delay, physical abuse findings) should be sought.
Physical examination: With palpation, stool accumulation in the colon can be detected. The perineum should be examined for fissure, skin disease, analectopia and sacral agenesis. If urinary incontinence is also present, spine and sacral region examination should be performed. The newborn who does not remove meconium in the first 48 hours should be evaluated and investigated for anal atresia, anal stenosis, HH and cystic fibrosis. After rectal examination in HH, spitting can be seen when the finger is pulled. Biliary vomiting, abdominal distention, sometimes bloody diarrhea due to enterocolitis may be detected. Older children may have abdominal distention and growth retardation. Passage of meconium plugs is suggestive of cystic fibrosis.
Laboratory: Laboratory examination is not required for the diagnosis of functional constipation. Most of the examinations performed serve to distinguish the severity of the condition, some subtypes and make a differential diagnosis. If an organic cause is considered by history and physical examination, tests for the diagnosis of the suspected disease should be performed (Table 2).
Radiology: In functional constipation, it is performed only to show the transit time or to observe the amount of accumulated stool. Eight radiopaque markers are drunk for three consecutive days for the transit time, and on the fifth day, a plain film is shot and according to the number of markers seen in the film; severe delay, moderate delay, mild delay and normal. Also, where the markers stay is important. Deposition in the rectosigmoid region is evaluated as “exit obstruction” and distribution throughout the entire colon as “pancolonic delay”. Showing the movies to the family can provide a better understanding of the problem. Normal transit time does not rule out constipation. This radiological study can also be used to evaluate response to treatment. The absence of a radiopaque marker in a child with ongoing abduction indicates an overdose of the drug, while the persistence of symptoms indicates inadequate treatment or unresponsiveness. Transit time can also be demonstrated by radionuclide scintigraphy.
Manometry: Ineffective straining can be demonstrated in older children. During straining, contraction of the external sphincter is observed instead of relaxation. HH is one of the diseases that should be kept in mind in the differential diagnosis. HH occurs in 1 in 5,000 live births (3% of children of sick parents). The male/female ratio is 4. While 94-98% of term babies and 76% of preterm babies remove meconium in the first 24 hours, 94% of H-Holans do not. Almost all normal infants and 98% of preterm infants excrete meconium in the first 48 hours. The neonatal infant’s failure to expel meconium in the first 48 hours, vomiting (which may be biliary), and presence of abdominal distension indicate HH. The main pathology is the absence of ganglion cells in the affected area. Aganglionosis is in the rectosigmoid region at a rate of 70-80%, with total colonic involvement in 15% of the patients. When these symptoms are absent or missed, the child may present with a septic picture (enterocolitis picture) with vomiting, distention and diarrhea. Less frequently, the sick child may present with growth retardation and constipation. In older children, the presence of severe constipation, growth retardation, abdominal distention, and often an empty rectum on rectal examination suggest the diagnosis. Fecal incontinence is usually absent. Delayed emptying of barium from the colon is also helpful in diagnosis. X-ray may be insufficient in recognizing short lesions, total colonic aganglionosis, and neonatal disease. Its treatment is removal of the aganglionic segment and retraction of the normal part into the anal canal.
Constipation may continue after the operation and stool incontinence may develop. The typical appearance on barium radiography is narrow segment (the diseased part) and dilatation in the proximal colon. Internal anal sphincter achalasia (IASA), formerly called very short segment HH, is characterized by the absence of a rectoanal inhibitory reflex on a motility study despite normal rectal biopsy. The innervation of the internal anal sphincter is defective. The general appearance is constipation, which may be accompanied by stool incontinence. Internal sphincter myectomy is successful in its treatment.
Treatment (Consult your doctor)
Although the time elapsed between the onset of symptoms and initiation of treatment has not been fully proven, it does affect success. The earlier the treatment is started, the better the prognosis. A concern for families is that the child has an underlying chronic illness. If these families and their age are appropriate, the child should be convinced that the event is functional. Although the view that behavioral problems are more common in children with constipation is controversial, it is known that those with behavior problems have worse response to treatment. For this reason, medical and psychological approaches should be given together in treatment. The basis of treatment is “empty the colon and keep it empty”. It is aimed to prevent the accumulation of feces for a long time after the colon is emptied, to return the anatomical structure to normal and to regain the sensitivity of the rectum.
·Disimpaction of the colon: There are many drugs used to provide evacuation. If necessary, manual evacuation can be performed under an enema and, in some cases, general anesthesia.
·Maintenance treatment: After the rectosigmoid region is emptied, the aim is to prevent stool from accumulating in the colon and to return the rectum to its normal size. The aim is to defecate at least once or twice a day with a normal consistency. The child should be seated on the toilet for five minutes after each meal. In the meantime, keeping a diary and marking the number of stools per day and incontinence is helpful in monitoring the response to treatment as well as encouraging the child. The main drugs used in the treatment, their doses and side effects
are given in Table 3, and the fiber contents of some nutrients are given in Table 4. In addition to drug therapy, toilet training, psychological therapy and, in some cases, biofeedback therapy can be applied. Biofeedback is teaching the child the normal defecation pattern (increasing intra-abdominal pressure by pushing and relaxing the external sphincter) by using manometry and/or electromyography (EMG). Biofeedback is useful in those with pelvic-floor dyssynergia. Thus, children are taught the normal way of defecation by showing that the external anal sphincter should be relaxed while straining.
GIS: Gastrointestinal system.
Anorectal functions and colonic transit time are normal in manometry. In these, it is seen that the stool that is missed in the history is in normal amount. Dabiofeedback is usually more successful in these children. Biofeedback is of no use unless there is a defect in the defecation mechanism. Since the resting pressure of the internal anal sphincter is high in children with fissures (whether cause or effect), lowering this pressure is recommended in treatment. Attention should be paid to the presence of perianal infection, as it will cause pain and avoid defecation. Recurrence rates as high as 50-60% have been reported in some studies. With medical treatment, psychological support and changing behavior, the chance of success rises up to 88%. However, relapses may occur in up to half of the children, and in this case, short-term medical treatment should be given. Success is directly dependent on the adaptation of the family and the child to the program. After the rectum is fully emptied, toilet training should be provided, daily emptying should be ensured by using stool softeners and accumulation should be prevented. This period takes at least six months. After this period, it is important to discontinue the drugs under supervision. When it is thought that there is not enough ejaculation, the drug should be started again.
·Surgical treatment: Less than 5% of patients may require surgical treatment.
Table 1. Frequency of normal stools in children
Age Weekly stool frequency Daily stool frequency
0-3 months
Breast milk 5-40 2.9
Formula 5-28 2.0
6-12 months 5-28 1.8
1-3 years 4-21 1.4
> 3 years 3-14 1.0
1. Functional: 95%
2. Organic
a. Anatomical
Imperforate anus
Anal stenosis
Anus anterior location
Pelvic mass (teratoma etc.)
b. Metabolic and gastrointestinal
Hypothyroidism
Hypercalcemia
Hypokalemia
Cystic fibrosis
Diabetes mellitus
Multiple endocrine neoplasia type 2B
Celiac disease
Renal tubular acidosis
c. Neuropathic diseases
Spinal cord abnormalities
Spinal cord trauma
Neurofibromatosis
Encephalopathy
Tethered cord
Cerebral palsy
d. Intestinal nerve and muscle disorders
Hirschsprung’s disease
Intestinal neuronal dysplasia
Intestinal pseudoobstruction
Visceral myopathies
Visceral neuropathies
e. Abnormal abdominal musculature
Prune belly syndrome
Gastroschisis
Down syndrome
f. Connective tissue diseases
Scleroderma
Systemic lupus erythematosus
Ehlers-Danlos syndrome
g. Drugs
Opiates
Phenobarbital
Sucralfate
Antacids
Antihypertensives
Anticholinergics
Antidepressants
Sympathomimetics
h. Other
Heavy metal poisoning (lead)
Vitamin D poisoning
Botulism
Cow’s milk protein intolerance
