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By Prof. Youssri Gaweesh Professor of colorectal surgery Alexandria university Constipation in children

Constipation in children final

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Page 1: Constipation in children final

By Prof. Youssri Gaweesh

Professor of colorectal surgeryAlexandria university

Constipation in children

Page 2: Constipation in children final

Definition Constipation is decreased frequency of bowel

movements usually associated with a hard

stool consistency passed with difficulty every

3rd day.

Functional constipation implies that there is

no identifiable causative organic condition

Functional constipation typically starts after

the neonatal period

Page 3: Constipation in children final

Chronic constipation

3% of the visits to general pediatrics.25% of the visits to pediatrics G.I.Functional / non organic is most

commonOrganic is rare ( except in infancy)

Page 4: Constipation in children final

Idiopathic constipationIdiopathic constipation is a self-perpetuating and

self-aggravating disease. A patient that has a certain degree of constipation that is not adequately treated only partially empties the colon throughout the day, leaving larger and larger amounts of stool inside the rectosigmoid, which results in greater degrees of megasigmoid.

Most surgeons accept that the dilatation of a hollow viscus produces poor peristalsis which leads to dilatation. This explains why constipation means fecal retention, which produces megacolon that exacerbates the constipation.

Page 5: Constipation in children final

Idiopathic constipationIn addition, the passage of large, hard

pieces of stool may produce painful anal lacerations (fissures), which result in a reluctance by the patient to have bowel movements. Consequently, if the patient was born with a certain degree of constipation and does not receive proper treatment, the constipation worsens and becomes an increasingly serious problem.

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Idiopathic constipationThe condition is mostly incurable, which

means that these patients must be monitored for life.

Unfortunately, treatments are frequently administered on a temporary basis; they are then tapered or interrupted, followed by a subsequent recurrence.

This creates a great deal of frustration for patients and parents and may contribute to the well-known pattern of patients who seek a solution from many different doctors or clinics.

Page 7: Constipation in children final

Idiopathic constipationAnother controversy involves symptom onset. Many

doctors believe that this problem starts during toilet training. Although symptoms become more evident at that time, the motility disorder is present at birth.

Breastfed babies may not show symptoms, because of the well-known laxative effect of human breast milk.

When breastfeeding is discontinued and the patient receives formula and other foods, the symptoms become obvious. Babies who have constipation problems while breastfeeding are likely to have severe constipation that will only worsen over time. Some of these patients need to be checked for Hirschsprung disease.

Page 8: Constipation in children final

Functional constipation

Exclude red flag signsTriggering eventsThe withholding behaviorComplicationsManagementoutcome

Page 9: Constipation in children final

Red flag signs Symptoms appearing from birth or during

the first few weeks of lifeDelay in passing meconium for more than

48 hours after birth in a full term babyAbdominal distension with vomitingRibbon stool patternAbnormal appearance of anus (shape and

site) with absence of anal wink

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Red flag signsWeakness in legs or locomotor delayAbnormalities in the lumbosacral or gluteal

regionsEvidence of faltering growth and well being

(hypothyroidism)

Page 11: Constipation in children final

Functional constipation

Triggering eventsPainful defecation.Postponing.Toilet: unavailability, phobia, training

technique.Changes in diet, routine.Stress.Sexual abuse

Page 12: Constipation in children final

Functional constipationThe withholding behavior

Present in about 30% of the children with functional constipation.

Struggle against the urge to defecateContract anal sphincter and gluteal muscles,Stand on their toes, assume unusual

postures and cross their legs

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Complications

Enormous stools to the point of being “toilet-plugging-specials”

Significant pain and a prepassage stereotyped behavior of gluteal tightening and posturing

Early satiety, small meals all day, irritability, and unpredictable spasms of abdominal pain usually located in the lower abdomen.

Encopresis becomes increasingly frequent. Painless rectal bleeding after defecation. After the passage, symptoms generally resolve

for a few days, then recur.

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Functional constipation

ComplicationsFecal soiling ( encopresis).Acquired megacolonAcquired motility disorders of the distal colonDecreased awareness of the urge to

defecate.Decreased amplitude of rectal contractions.Lack of relaxation of the anal sphincter.Psychosocial

Page 15: Constipation in children final

Management of chronic constipation and encopresis

The three phases of management:

complete evacuation or disimpactionsustained evacuation to restore normal

colorectal toneweaning from intervention.

The success of each depends on the cooperation and understanding of the parent and, when possible, the child.

Page 16: Constipation in children final

Management of chronic constipation and encopresis The older child is encouraged to be

involved by keeping a sticker chart or calendar to document efforts, successes, and failures.

They are encouraged to establish a “habit” of toilet use independent of the rest of treatment.

Use a footstool for the child to maximize abdominal pressure during the Valsalva maneuver.

Page 17: Constipation in children final

Constipation due to intestinal diseaseOrganic diseases

Hirschsprung diseaseUltrashort segment HD Neural dysplasia Internal sphincter achalasia of neurogenic origin or

myogenic origin Functional diseases (no definite pathology was

elucidated)Slow transit constipation with normal calibered large

bowelSlow transit constipation with megalarge bowelFunctional internal sphincter achalasia due to

counteracting the reflex inhibition of the internal sphincter by voluntarily contracting the external sphincter

Page 18: Constipation in children final

Hirschsprung diseaseHirschsprung disease or aganglionosis occurs in 1

in 5,000 births. Male-to-female ratio is 4 to 1, and the incidence

increases with longer segments of disease. The diagnostic lack of ganglion cells in the

myenteric and submucosal plexus of the bowel wall extends proximally from the internal anal sphincter.

In among 80% of the involved children, the aganglionic segment does not extend above the sigmoid.

Page 19: Constipation in children final

Hirschsprung diseaseHirschsprung disease is a heterogeneous genetic

disorder with risk rates for siblings ranging from 3% with short segment disease to 25% with a female who has long segment disease.

An autosomal dominant form occurs with mutation in the RET gene.

Syndromes associated with Hirschsprung disease include trisomy 21, deletion of chromosome 13q, Smith-Lemli-Opitz, Waardenberg, Laurence-Moon-Biedl-Bardet, congenital deafness, and congenital central hypoventilation.

Page 20: Constipation in children final

Hirschsprung diseaseDifficulty with evacuation is present from birth;Meconium is not passed in the first 48 hours of

life in 40% of involved infants.Recurrent abdominal distension, emesis, failure

to thrive, and acute enterocolitis allow diagnosis of 60% of patients by 3 months of age.

The presence of early obstructive features, onset in infancy, and nearly complete absence of encopresis distinguish Hirschsprung disease from functional fecal retention.

Page 21: Constipation in children final

Hirschsprung diseaseOn rectal examination, the aganglionic

bowel is tight around the finger and the rectal ampulla is not dilated.

A barium enema usually allows visualization of the transition zone between the tonically contracted aganglionic segment and the dilated proximal bowel.

The enema should be performed without preparation, which distorts the distal anatomy.

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Hirschsprung diseaseThe entire colon and some small bowel

may be involved in 3%.The aganglionic segment of bowel fails to

relax because of the absence of inhibitory neurons containing nitric oxide and vasoactive intestinal peptide.

Page 23: Constipation in children final

Hirschsprung diseaseManometric studies of the rectum

demonstrate the failure of the internal anal sphincter to relax when distended (negative RAIR).

Confirmation of the diagnosis requires rectal biopsy (best is the suction capsule biopsy).

The aganglionic bowel has nerve fibers staining for acetyl cholinesterase (histochemical staining)

Page 24: Constipation in children final

VariableFunctional (Acquired)

Hirschsprung Disease

Onset of constipation after 2 yrs of ageAt birthAbdominal distensionUncommonCommonPoor weight gainRareUncommonAnal toneNormalNormalRectal examinationStool in ampullaAmpulla is emptyMalnutritionNonePossibleAnorectal manometryDistension of the

rectum causes relaxation of the internal sphincter

No sphincter relaxation or paradoxical increase of pressure

Rectal biopsyNormalNo ganglion cells Barium enemaMassive amounts of

stool, no transition zone

Transition zone, delayed evacuation

Page 25: Constipation in children final

Neuronal dysplasiaNeuronal dysplasia, in contrast to

aganglionic disease, is associated with increased numbers of ganglion cells (hyperganglionosis) in the lower colon.

It may present throughout childhood with variable constipation or features of pseudo-obstruction.

It is more frequent among children who have neurofibromatosis and has been associated with MEN type IIb due to glioneuromas of the intestinal tract.

Page 26: Constipation in children final

Neuronal dysplasia (cont.)Surgical intervention is individualized and

based on the severity of symptoms and manometric demonstration of severely impaired rectal relaxation.

Reduced numbers of ganglion cells (hypoganglionosis) usually is an acquired disease of ganglion cell destruction seen in Chagas disease or paraneoplastic syndrome.

Sometimes fecal retention causes a sort of ganglionitis causing loss of ganglia

Page 27: Constipation in children final

Slow transit constipationPatients which can be called the inertia

constipation category (slow transit constipation prolonged STT) two types of patients can be easily distinguished. Type one includes patients who proved to have

normal large bowel diameter may be with some extra looping of the sigmoid or other regions of the colon (dolichocolon),

Type two that includes patients with mega large bowel, whether it is only mega rectum, rectosigmoid , or megacolon.

Page 28: Constipation in children final

Slow transit constipationThe differentiation between these two

categories is done by radiographic evaluation, barium or gastrographin enema and double contrast.

There is dilatation that involves the rectum and/ or the large bowel to the pelvic floor

The process involves or begins in the rectum. A rectal diameter of 6.5 cm at the pelvic rim on lateral view defines mega rectum

Page 29: Constipation in children final

Slow transit constipationColonic inertia is defined as the failure of the

colon to propel stool towards the rectum, including failure to produce mass movement of stool around the time of defecation.

Colonic motor activity is abnormal with reduced high amplitude propagating contractions, and transit time through the colon is prolonged

There is failure to enhance colonic phasic pressure activity by a meal or stimulant, and impaired propagated colonic contractile response to bisacodyl and cholinergic agents

Page 30: Constipation in children final

Slow transit constipationTransit-time studies are very helpful in the

confirmation of the presence of slow transit constipation (functional constipation: FC)

It also differentiate the slow transit constipation from the constipation predominant irritable bowel syndrome (C-IBS) where the pellets are propagated to the left side of the colon in normal time with slow transit at the rectosigmoid area

Page 31: Constipation in children final

plain x-ray of cases showing markers scattered all over the colon after 5 days. These patients required subtotal colectomy

Page 32: Constipation in children final

plain x-ray of case showing markers scattered all over the colon after 5 days. This patient responded to subtotal colectomy

plain x-ray of case showing markers scattered all over the colon after 7 days. This patient did not improve after anorectal myotomy and required subtotal colectomy

Page 33: Constipation in children final

Plain X ray showing radio-opaque markers in the rectum in obstructed defecation (excluded from the study)

Page 34: Constipation in children final

Chronic constipation• Symptom onset at least 6

months prior to diagnosis• Presence of symptoms for the

last 3 months • Loose stools are rarely present

without the use of laxatives.• Fewer than 3 bowel movements

per week• Symptoms include 2 or more of

the following during at least 25% of defecations:

– Straining – Lumpy or hard stools – Sensation of incomplete

evacuation – Sensation of anorectal

obstruction or blockade – Manual maneuvers to facilitate

evacuation

IBS-CSymptom onset at least 6 months

prior to diagnosis• Recurrent abdominal pain or

discomfort at least 3 days per month in the last 3 months associated with 2 or more of the following: – Improvement with defecation – Onset associated with a change in

stool frequency – Onset associated with a change in

stool form (appearance)

• 1 or more of the following symptoms on at least 25% of occasions for subgroup identification:– Abnormal stool frequency (<3/week) – Abnormal stool form (lumpy/hard) – Abnormal stool passage

(straining/incomplete evacuation) – Bloating or feeling of abdominal

distension– Passage of mucous

Rome III Criteria for Chronic (Functional) Constipation and Constipation-Predominant Irritable Bowel Syndrome (IBS-C)

Page 35: Constipation in children final

If subtotal colectomy is decided, an ileosigmoid side to side stapled anastomosis is recommended to minimize the diarrhea problem; however the occurrence of postoperative adhesive small bowel obstruction should always be kept in mind.

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Patients with mega large bowelThe question is:Are all megalarge bowel are of organic

origin? as in the following cases Ultrashort segment Hirschsprung diseaseInternal sphincter achalazia ,neurogenic and /or

myogenicNeural dysplasia

Or some of the megalarge bowel are of functional origin

The answer is yes

Page 37: Constipation in children final

Patients with mega large bowelA contrast enema performed by using

hydrosoluble material (never barium) is the most valuable study to confirm the diagnosis of idiopathic constipation. The dilatation of the colon extends all the way down to the level of the levator mechanism, which is recognized because it coincides with the pubococcygeal line. The lack of dilatation of the rectum below the levator mechanism (pubococcygeal line) should not be interpreted as a transition zone or nondilated aganglionic bowel.

Page 38: Constipation in children final

Patients with mega large bowelUnder normal circumstances, the anal canal and the part

of the rectum below the levator mechanism are collapsed by the effect of the striated muscle tone from the sphincter mechanism. The rectum above the anal canal is extremely dilated, as is the sigmoid. The contrast enema in patients with idiopathic constipation reveals different degrees of dilatation of the rectosigmoid, as is expected in a spectrum-type condition. Most interestingly, a dramatic size discrepancy is noted between a normal transverse descending colon and an extremely dilated megarectosigmoid.

These changes are actually the reverse from what is observed in patients with Hirschsprung disease. The colon in a patient with Hirschsprung disease is dilated only proximal to the aganglionic segment, which remains non dilated.

Page 39: Constipation in children final

Anorectal and Colonic Manometry

Anorectal manometry is used by many practitioners. Traditionally, it is performed by placing a balloon in the rectum while measuring the pressure of the anal canal. Under normal circumstances, when the rectal balloon is inflated, the intra-anal canal pressure decreases; this is described as the anorectal reflex (RAIR). Pressure that does not decrease in the anal canal is considered abnormal and is considered a sign of “lack of relaxation of the internal sphincter.” This is also considered diagnostic for Hirschsprung disease.

Page 40: Constipation in children final

Anorectal and Colonic Manometry

If the patient’s rectum has no ganglion cells, the diagnosis of Hirschsprung disease is confirmed. Alternatively, if the rectal biopsy reveals ganglion cells, the patient has idiopathic constipation. In patients with Hirschsprung disease, the treatment is well established.

Page 41: Constipation in children final

Anorectal and Colonic Manometry

The whole issue becomes more controversial when the rectal manometry is critically scrutinized. The pressure recorded in the lumen of the anal canal, supposedly generated by the internal sphincter, is actually generated both by the internal sphincter (smooth muscle) and by the striated voluntary muscle mechanism (external sphincter and levator), which surrounds the lower rectum and the anal canal around the area of the internal sphincter. To date, no publication has clarified this serious flaw in the interpretation of manometric studies.

Page 42: Constipation in children final

Anorectal and Colonic Manometry

Pressure in the anal canal is not the primary result of the contraction of the voluntary sphincter mechanism. The original manometric studies in animals were performed by using muscle relaxants, which kept the voluntary muscle mechanism paralyzed. Any changes in the pressure of the anal canal, under those circumstances, could be attributed to the effect of the smooth muscle (internal sphincter). However, none of the clinical studies published have made use of muscle relaxants.

Page 43: Constipation in children final

Anorectal and Colonic Manometry

In addition, the inflation of a balloon in the rectum is assumed to produce tension on the rectal walls, which triggers some mechanism that produces a decrease in pressure in the lumen of the anal canal as a final result. However, patients who are constipated have widely varying degrees of megarectosigmoid, ranging from minimal to giant. The sizes of the balloons that are used in manometric studies are never large enough to stretch giant rectosigmoids. Thus, the inflation of a regular balloon may not be enough to stretch the rectal wall in patients with megarectum, and this underinflation may produce false results.

Colonic manometry, which measures propagation of peristalsis through the colon, has not yet reached a point of accuracy to determine which specific part of the colon is working and which is not.

Page 44: Constipation in children final

Histologic Findings

Rectal biopsies are usually performed with the specific purpose of ruling outHirschsprung disease. The study is usually unnecessary when the clinical picture and the radiologic findings are characteristic of idiopathic constipation.

Rectal biopsies are performed if the contrast enema reveals findings that suggest aganglionosis or if the patient behaves in a way that is clinically similar to a patient with Hirschsprung disease. If the patient has episodes of enterocolitis and does not soil, Hirschsprung disease is suspected. If the rectal examination reveals an empty rectum and the patient is still impacted above the reach of the finger, consider Hirschsprung disease and perform a biopsy.

Page 45: Constipation in children final

Mega large bowel of organic originThe contrast study with an attempted

defecation (defecography) has been demonstrated to differentiate between normal, patients with ultra short segment HD, patients with intestinal neural dysplasia (IND), and different types of internal sphincter achalazia.

As an alternative a contrast enema with straining and lateral view of the rectum down to the pelvic floor can be used for differentiation

Page 46: Constipation in children final

Mega large bowel of organic originIn the normal defecography in the lateral

view there is stretching and reestablishment of the anorectal angle with internal sphincter relaxation thus the column of contrast is seen to continue through the anal canal which looks to be at the same straight line to the outside.

On the contrary, in cases of ultra short segment HD the internal sphincter shows no relaxation and the anal canal remains completely closed

Page 47: Constipation in children final

Mega large bowel of organic originIn cases where there is neurogenic internal

sphincter achalazia internal sphincter is showing non relaxation and is possibly associated with the presence of a fecolith at the end of the rectum.

Manometerically speaking, whereas the demonstration of internal sphincter relaxations excludes the presence of HD, the absence of the relaxation reflex is only pathognomonic for HD when the anorectal fluctuations typical of the smooth muscle cells of the internal anal sphincter are observed

Page 48: Constipation in children final

Mega large bowel of organic origin On the other hand in cases of myogenic

anal sphincter achalsia there is minimal opening of the internal sphincter giving a smooth cone shaped opening of the lower rectal end with tight end of the anal canal and possible presence of fecolith.

There is also smooth ballooning of the posterior wall of the rectum.

Page 49: Constipation in children final

Mega large bowel of organic originIn patients with IND no pathognomonic

morphology of the relaxation reflex exists. The reflex mechanism may be normal, rudimentary or absent.

The same is true for hypoganglionosis and immaturity of ganglion cells. The internal anal sphincter sometimes also has an elevated tone with an increased anorectal pressure profile. This can be true in HD as well as in hypoganglionosis and IND

Page 50: Constipation in children final

Mega large bowel of functional origin

In case of functional internal sphincter achalazia there is deficient opening of the anal canal due to counteracting the internal sphincter relaxation by voluntary contraction of the external sphincter with diverticulum like protrusion of the posterior rectal pole with partial evacuation.

In these cases the RAIR reflex is showing inhibition of the internal sphincter tone

Page 51: Constipation in children final

Mega large bowel of functional originIn these cases usually anorectal myectomy

alone is curative with the presence of ganglia in the myectomy specimen

If in these cases myectomy did not improve the condition, the diagnosis of neural dysplasia is the usual outcome. It responds to the pull through operation

Page 52: Constipation in children final

There is abrupt stoppage of dye at the pelvic floor with the presence of fecoli in the rectum as well as in the sigmoid. This is similar to what is described in cases of ultrashort segment HD or in cases with neurogenic internal sphincter achalazia. This patient was 6 years old and the rectoanal inhibitory reflex (RAIR) was absent.

Page 53: Constipation in children final

A male 11 years old the lateral view showed mega large bowel with no coning at the lower rectal end. The RAIR was absent and the myectomy showed no ganglia and was inefficient in relieving the condition. Ultra short HD or less probably neurogenic internal sphincter achalsia were speculated as the most probable diagnoses.

Page 54: Constipation in children final

A female 23 years old the lateral view of the barium showed megarectum only with moderate coning at the lower end of the rectum. The anorectal myectomy proved beneficial and curative to the case and showed the presence of ganglia and present anorectal inhibitory reflex. This case can be an example of either functional or myogenic internal sphincter achalazia.

Page 55: Constipation in children final

First the case is a female almost 30 years old with perfect physical and mental development, second the rectum is the only part of the large bowel affected , thirdly there was no fecoli , and the posterior wall of the rectum showed no diverticular like projection but only smooth posterior ballooning with no coning at the anorectal angle. Her RAIR was absent, and no ganglia were detected in the myectomy specimen. Ultra short segment Hirschsprung megarectum was the most probable diagnosis.

Page 56: Constipation in children final

A classical ultrashort segment HD in the barium lateral view demonstrating no relaxing lower rectal segment ( since dilatation did not reach the pelvic floor) . The patient was one year with retarded growth that improved dramatically after the final operation. The RAIR was absent, and the ganglia were not detected.

Page 57: Constipation in children final

There is large bowel dilatation with very mild lower rectal coning and multiple fecoli and smooth posterior rectal ballooning. The presence of positive RAIR and the also the presence of ganglia makes the diagnosis of intestinal neural dysplasia a more probable diagnosis than ultra short segment HD or internal sphincter neural achalazia.

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Relation of the ganglia status to the RAIR reflexIn most cases if the RAIR is negative, the

ganglia are absent denoting either ultrashort segment Hirschsprung or neurogenic internal sphincter achalazia with superadded ganglionitis

On the contrary , if the ganglia are present and the RIAR is positive , the above lesions are excluded , and the most probable diagnosis is functional achalazia ( megarectum alone, with coning of the anal canal on barium study). This responds to myectomy alone.

It also can exist with neural dysplasia, that only respond to pull through operation

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Relation of the ganglia status to the RAIR reflexIf however , there were ganglia with a

negative RAIR , possibilities of neurogenic internal sphincter achalazia associated with neural dysplasia or other allied disorders is possible.

Myectomy alone in these cases usually fail and a pull through operation is needed

Page 60: Constipation in children final

CONCLUSIONS

In conclusion we advocate the following steps in managing patients with chronic constipation after exclusion of any possibility of obstructed defecation syndrome:

1.A contrast enema is mandatory as the initial step of evaluation

2.The patients are then categorized into either patients with normal caliber of the rectum and colon or patients with mega rectum with without mega colon

3.For patients with normal caliber of the colon a colon transit time is mandatory before any decision of surgery is taken. This test is cheap, can be repeated and of course the conventionally described precautions are taken in its interpretations.

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CONCLUSIONS If subtotal colectomy is decided, an

ileosigmoid side to side stapled anastomosis is recommended to minimize the diarrhea problem; however the occurrence of postoperative adhesive small bowel obstruction should always be kept in mind.

The anorectal manometric study is required for investigational purposes.

If mega large bowel is depicted, an ano-rectal myectomy is done either posteriorly or laterally for a minimum distance of 4 cm with biopsy taken for investigational purposes.

Page 62: Constipation in children final

CONCLUSIONSA minimum of a month should be left before a

decision is taken to proceed for the endorectal, abdominally assisted pull through operation.

A stoma is advised in case of the pull through operation until further investigations can provide evidence that the non use of stoma is safe.

Almost all patients needs after the colure of stoma some sort of stool softeners and regulation of daily eating and bowel habits to avoid encopresis which is very common in patients below 10 years of age.

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Anorectal myotomy and biopsy

2 stay sutures are taken at 4, 8 o'clock 1.5cm above the dentate line to the skin including only the mucosa and submucosa.

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Anorectal myotomy and biopsy

A shelf of mucosa is created posteriorly under which 10ml 1: 300,000 adrenaline in saline is injected submucosly.

Incision with raising a mucosal flap is done

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Anorectal myotomy and biopsy

Raising a mucosal flap is done as far the finger can reach (across to the 3rd sacral piece) to demonstrate the internal sphincter and circular muscle layer of the rectum.

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Anorectal myotomy and biopsy

A myotomy is done in the circular muscle layer using scissor or low voltage diathermy with a length of about 8-10 cm

Biopsies are taken from extreme upper edges of the myotomy

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Anorectal myectomy and biopsy

a strip 0.5cm width of the muscular rectal wall starting 1cm above the dentate line

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Anorectal myectomy and biopsy

a length of 8-10cm and this is taken all as a biopsy after demarcating its upper end with a stitch.

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Anorectal myectomy and biopsy

Closure of the transverse mucosal wound is done after reassuring hemostasis and a small pack is inserted for 12 hours

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Trans-rectal abdominally assisted pull through operation

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Another case of trans-rectal abdominally assisted pull through operation

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Is there other surgical options for mega large bowel?The idea of subtotal colectomy with ileorectal

anastomosis is not logic in the presence of mega rectum

The operation entailing total proctocolectomy with ileal pouch anal anastomosis is too big for a benign lesion, also exposing the patient to temporary diversion, deep pelvic dissection with autonomic nerve jeopardy.

The operation suggested in the literature is known as vertical reduction rectoplasty (VRR). It entails dissection in the pelvis to mobilize the rectum, vertical excision of part at the anti-mesenteric border ( anteriorly), and end to end or to side anastomosis of what is left from the sigmoid (partial sigmoidectomy is done)to the rectal remnant.

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Line of a linear stapler applied anteriorly after mobilizing the rectum

VRR first step is to mobilize the rectum and reduce its capacity by a linear stapler 10cm

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VRR the second step is to anastomose what is left from the sigmoid to the reduced rectal stump using a circular end to end anastomosis device.

The site of the anterior staples It is better to do the anastomosis more posterior away from the anterior stapler line.

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Sigmoid resectionSigmoid resection may be indicated in a small subset

of patients who are continent but have a huge laxative requirement.

In this group of patients, if a megasigmoid is revealed by using a contrast enema, a sigmoid resection can be performed to reduce the laxative requirement and improve quality of life.

The most dilated part of the colon is resected because that part of the colon is believed to be most seriously affected. The nondilated part of the colon is assumed to have normal motility, which is why the surgeons use it to anastomose to the rectum. The very distal rectum is preserved. This can best be done laparoscopically.

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Sigmoid resectionSigmoid resection is best accompanied by

VRR , otherwise, anastomosing the colon to a dilated rectum can eliminate the benefits of sigmoidectomy.

Performing a sigmoid resection in a patient who is incontinent with constipation is a major error. When the sigmoid is removed, the patient becomes incontinent with loose stools, a state that is much more difficult to manage.

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Appendecostomy or cecostomy In patients who are fecally incontinent, a

bowel management program with a daily enema is the correct treatment. The rectal route may be problematic in older children who require enemas: they tend to seek independence and do not want their parents to give them enemas. In these patients, a continent appendicostomy (Malone procedure) or cecostomy for antegrade rectal washout can be performed.

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Appendecostomy or cecostomyThe operation involves connecting the

appendix to the abdominal wall and fashioning a valve mechanism that allows catheterization of the appendix but avoids leakage of stool through it.

Some authors insert a cecostomy tube, which requires a synthetic tubing material that enters the cecum for the same goal of performing enemas. Both procedures have been laparoscopically performed. Occasionally, if the patient has had the appendix removed, a neoappendix can be created with a cecal flap.

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Appendecostomy or cecostomyRegular follow-up and reassessment is

necessary. Often, the volume of enema needs adjustment. Rectal examination and abdominal radiography help the surgeon assess the actual cleanliness of the colon.

Complications of appendicostomy include stricture and leakage, which usually require a revision of the stoma.