HIRSCHSPRUNG-ASSOCIATED ENTEROCOLITIS IN FEMALE INFANT:

A CASE REPORT

I Wayan Aryanta Putra

Advisor: dr. Santi Rini, SpBA

BACKGROUND
Neonatal intestinal obstruction is one of the most common causes of neonatal surgical
emergencies. Hirschsprung’s disease (HD) is a common cause of bowel obstruction in
neonatal.1 HD is a congenital disorder defined by the absence of ganglion cells (GC) at the
Meissner's plexus of the submucosa and Auerbach's plexus of the muscularis in the terminal
rectum that extends in a variable distance proximally. 2 Its prevalence varies from 1 to 1.63
per 10,000 births and has an overall 4:1 male predominance worldide.2 The incidence of HD
has been reported to be higher in Asian infants as compared to infants from other ethnic
origin.3 Nevertheless, HD case in Indonesia remains under-examined. Such limited awareness
often causes late appropriate treatment on patients leading to the increased morbidity and
mortality including Hirschsprung-associated enterocolitis (HAEC). HAEC is the most
common and severe complication of HD. Even though the mortality rate of HAEC has begun
to decline, it is still considered as the main cause of mortality and morbidity in HD since
mortality happens in about 50% of patients. RSUD Aji Muhammad Parikesit as one of
general hospitals in Indonesia provides pediatric care in handling HD cases. The number of
HD cases at RSUD Aji Muhammad Parikesit in 2018, 2019, and 2020 were 19, 19, and 21
cases, respectively, where the average male: female ratio was 1.7:1.

CASE PRESENTATION
1. History
The clinical case consisted of a 51-day-old female infant who presented with chronic
constipation afflicting her since the age of 7 days. The mother referred that the patient could
only defecate 1 to 2 times per week. The patient’s defecation was spontaneous with liquid
yellow feces of about 5 cc per defecation accompanied explosive defecation. The defecation
did not involve rectal bleeding and mucus, but it had foul smell. The stomach kept bloating
since the age of 7 days. Vomiting of breast milk or formula milk that has just been drunk
occurred 1 to 3 times a day. The patient was restless and fussy with excessive thirst. There
were no intermittent lumps in the abdominal area and groin.
1
Pregnancy Record: The mother followed routine antenatal care (ANC) once per month from
5 weeks of gestation to 28 weeks of gestation at the Pusat Kesehatan Masyarakat
(Puskesmas) followed by ANC twice per month from 28 weeks of gestation until birth at the
hospital. Iron tablets were taken 1 tablet per day during the 2nd Trimester, while tablets of
folic acid were taken one to twice a week.
Birth record: The patient was a 5th child born through cesarean section due to her breech
position and oligohydramnion with a gestational age of 38 weeks and a birth weight of 2950
grams. The baby cried spontaneously after birth. Infants underwent phototherapy after birth
until the age of 3 days for the indication of neonatal jaundice. Meconium was passed at 2
days after birth. Formula feeding began at age of 7 days due to a little milk coming out. The
formula milk was changed three times since the age of 7 days until the present.
Family health history: Similar case was not presented by the siblings. There were no similar
complaints in siblings. Thyrotoxicosis was found both in the biological mother of the father
and mother.

2. Clinical Findings
The patient was sent from the Pediatric Surgery Polyclinic to the ER. Vital sign examination
resulted in composmentis consciousness (GCS: E4V5M6), temperature 36.2 degrees Celsius,
respiratory rate 30 times per minute, and pulse 128 times per minute, regular, strong lifting.
The patient's weight was 4.0 kg.
Examination on the general status of the head-neck region resulted in flat fontanel, anemic,
no tear production upon crying, and while the mucous membranes of the lips and tongue were
wet.
Chest examination showed that there was no chest wall retraction, breath sounds were within
normal limits, and the 1st and 2nd heart sounds were single and regular.
Upon abdominal inspection, the patient presented distension without darm contour and darm
steifung. On auscultation, bowel sounds were normal with tympanic sound over the entire
abdominal region on percussion. On palpation, the abdomen was soft, with an abdominal
circumference of 33 cm. No abnormalities were found on genital and anorectal inspection.
On examination of the extremities, capillary refill time (CRT) 1-2 seconds with warm toes
and fingertips.

2
3. Additional Examination
Based on the physical examination, it was decided to do a laboratory examination resulted as
follows.
Table 1. Laboratory Examination Result I
Data Result Normal Value
Complete Blood Count (CBC)
Hemoglobin 8,7 gr/dl 12 - 14 gr/dl
Hematocrit 26 % 37 - 43 %
Leukocyte 10.400 /mm3 5.000 - 10.000 /mm3
Granulocyte 23,8 % 50 - 70 %
Lymphocyte 67,3 % 20 - 40 %
Monocyte 8,9 % 2-8%
Platelet 617.000 /mm3 150.000 - 450.000 /mm3
PT 15,0 seconds 11,75 - 15,1 seconds
APTT 32,5 seconds 28,6 - 42,2 seconds
INR 1,06
Comprehensive Metabolic Panel
Glucose 97 mg/dl 60 - 150 mg/dl
Total Bilirubin 1,48 mg/dl 0,1 - 1,2 mg/dl
Direct Bilirubin 0,49 mg/dl ≤ 0,2 mg/dl
Indirect Bilirubin 0,99 mg/dl 0 - 0,75 mg/dl
Albumin 4,0 mg/dl 3,5 - 5,2 mg/dl
SGOT 29 U/L < 31 U/L
SGPT 17 U/L < 31 U/L
Sodium 137 mmol/L 135 - 155 mmol/L
Potassium 5,5 mmol/L 3,4 - 5,3 mmol/L
Chloride 102 mmol/L 98 – 106 mmol/L
Serology
HBsAg Negative Negative

4. Working Diagnosis
Suspect HAEC + Moderate dehydration + Electrolyte Imbalance + Hyperbilirubinemia

5. Planning
The following are the actions performed in the ER before the patient was transferred to the
inpatient room.
a. Diagnose Planning
- Chest X-Ray AP
- Abdominal X-Ray and Barium Enema / Colon in Loop
b. Therapy Planning
- OGT #8 for decompression

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 - IV D5% : ¼ NS 0,9% 400 cc per 24 hours
- IV Cefotaxime 200 mg per 12 hours
- IV Metronidazol 40 mg per 8 hours
- IV PRC 40 cc in 3 hours
- Clisma with warm NS 0,9% 20 cc per 12 hours
c. Monitor Planning
- 6-8 hours post-transfusion CBC examination
- Fluid balance monitoring per 3 hours
- Abdominal circumference monitoring per 8 hours
The following is a photo of the patient's colon in loop and thorax.
Figure 1. Chest X-Ray AP

Information:
- Coarse bronchovascular markings.
- Infiltrates in the right and left para-cardial fields
- Impressions of bilateral pulmonary pneumonia
Figure 2. Abdominal X-Ray and Colon in Loop

Information:

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Abdominal X-Ray
- Increasing amount of distribution and intestinal air
- Dilatation of the structure of the large bowel
- Visible fecal material
Colon in Loop
- Reductant of sigmoid colon
- Narrowing of the anal sphincter region to the rectum accompanied by widening of the
intestinal structure in the proximal part
- Visible prominent fecalite
- Image impression: Type Short HD
The patient was treated in the ER for 24 hours with the following post-transfusion CBC
examination result.
Table 2. Laboratory Examination Result II
Data Result Normal Value
CBC
Hemoglobin 10,2 gr/dl 12 - 14 gr/dl
Hematocrit 29 % 37 - 43 %
Leukocyte 12.200 /mm3 5.000 - 10.000 /mm3
Granulocyte 23,0 % 50 - 70 %
Lymphocyte 67,6 % 20 - 40 %
Monocyte 9,4 % 2-8%
Platelet 556.000 /mm3 150.000 - 450.000 /mm3

The following diagram presents the other monitoring results.

5
 Figure 3. Fluid Balance Monitoring Chart

120

100

80

60
Positive Balance
40

20

0
I II III IV V VI VII

Figure 4. Abdominal Circumference Monitoring Chart

33.5
33
32.5
32
31.5
31
Abdominal Circumference
30.5
30
29.5
29
28.5
I II III IV

The production of OGT was in the form of breast milk is about 5 cc.

6. Pre-Operative Care
The patient was transferred to the pediatric ward for treatment with an estimated length of
stay of 4 to 6 days with a target hemoglobin value of 12 g/dl with the following planning.

6
a. Diagnose and Therapy Planning
- OGT #8 for decompression
- IV D5% : ¼ NS 0,9% 340 cc per 24 hours
- IV Cefotaxime 200 mg per 12 hours
- IV Metronidazol 40 mg per 8 hours
- IV PRC 40 cc in 3 hours
- Clisma with warm NS 0,9% 20 cc per 12 hours
- Breast milk diet 8 x 10 to 12,5 cc until 1 day before the operative management
- Breast milk diet 6 x 5 to 7,5 cc 1 day before the operative management
- Echocardiography 1 day before the operative management
- Transanal pull-through procedure with frozen section biopsy
b. Monitor Planning
- Fluid balance monitoring per 8 hours
- Abdominal circumference monitoring per 8 hours
- Covid-19 antigen swab 1 day before the operative management
- CBC examination 1 day before the operative management

The following diagram presents the monitoring results.

Figure 5. Abdominal Circumference Monitoring Chart Rata-Rata Per Hari

36
35
34
33
32
31 Abdominal Circumference
30
29
28
27
I II III IV V VI

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The OGT treatment was not installed on day 2 and 3. The OGT was reinserted on the 4 th day
of treatment and the fluid that came out was bilious as much as about 5 cc per day. An
echocardiography, Covid-19 antigen SWAB, and laboratory examination were carried out on
the 5th day of treatment. The result showed normal echocardiography, non-reactive Covid-
19, and laboratory results as follows.
Table 3. Laboratory Examination Result III
Data Result Normal Value
CBC
Hemoglobin 14,3 gr/dl 12 - 14 gr/dl
Hematocrit 39 % 37 - 43 %
Leukocyte 10.900 /mm3 5.000 - 10.000 /mm3
Basophil 0,3 % 0-1%
Eosinophil 9,3 % 1-3%
Neutrophil 24,0 % 50 - 70 %
Lymphocyte 54,5 % 20 - 40 %
Monocyte 11,9 % 2-8%
Platelet 364.000 /mm3 150.000 - 450.000 /mm3
Comprehensive Metabolic Panel
Sodium 137 mmol/L 135 - 155 mmol/L
Potassium 5,0 mmol/L 3,4 - 5,3 mmol/L
Chloride 103 mmol/L 98 – 106 mmol/L

7. Operative
The operation was performed after 6 days of treatment with the patient's age at 58 days, body
weight 4.4 kg. The procedure was carried out under general anesthesia with transanal pull-
through procedure and frozen section biopsy. The procedures were as the following.
a. The patient was put in the lithotomy position before an antiseptic procedure was given
b. The perineal dissection began with the placement of circumferential 2-0 silk traction
sutures from the dentate line to the perineum 2 cm from the anus.
c. A needle-tipped electrocautery was used to incise the rectal mucosa approximately 0,5 - 1
cm circumferentially from proximal to the anal columns. Fine silk traction sutures were
then placed in the rectal mucosa to help retract the mucosa during circumferential
dissection.
d. The muscular cuff of the rectum had been divided, and the ganglionic colon had been
exteriorized through the anal canal.
e. A frozen section biopsy was performed with the following results
- Preparation I: adequate plexus, 1 immature ganglion
- Preparation II: adequate plexus, 1 immature ganglion

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 - Preparation III: adequate plexus, 2-3 mature ganglions
- Preparation IV: adequate plexus, > 3 mature ganglions
In preparations II (distal) and IV (proximal), pathological examination was carried out.
f. The anastomosis performed proximal to the biopsy site 11 cm from the anal column to
proximal. The pull-through colon was being completely transected above the biopsy site
and made ready for the coloanal anastomosis.
g. A rectal tube and tampon were inserted.
h. The patient was cleaned and the surgery was completed.
The procedures of the operation can be seen in the following figures.

Figure 6. Operation Procedures

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The operation lasted for 2 hours 15 minutes. The amount of bleeding during surgery was 15
cc, while 10 cc PRC transfusion was given during surgery. Laboratory examinations were
carried out on the patient after the procedure, with the following results.
Table 4. Laboratory Examination Result IV
Data Result Normal Value
CBC
Hemoglobin 12,1 gr/dl 12 - 14 gr/dl
Hematocrit 36 % 37 - 43 %
Leukocyte 10.300 /mm3 5.000 - 10.000 /mm3
Granulocyte 78,6 % 50 - 70 %
Lymphocyte 15,9 % 20 - 40 %
Monocyte 5,5 % 2-8%
Platelet 119.000 /mm3 150.000 - 450.000 /mm3
Comprehensive Metabolic Panel
Albumin 4,6 gr/dl 3,5 - 5,2 gr/dl
Sodium 140 mmol/L 135 - 155 mmol/L
Potassium 4,8 mmol/L 3,4 - 5,3 mmol/L
Chloride 104 mmol/L 98 – 106 mmol/L

8. Post-Operative Care
After the procedure, the patient was given post-operative care in Pediatric Intensive Care Unit
(PICU). Post-operative care was planned for 5 days after the operation under the following
plan.
a. Therapy Planning
- Once the patient was conscious, intestinal peristalsis was present, and there was stool
production in the rectal tube, the patient would be given a diet in the form of D5% 6 x
0.5 to 1.5 cc for 24 hours postoperatively, followed by a 6 x 5 to 7.5 breast milk diet
cc on day 2; 6 x 7.5 to 10 cc on day 3; 8 x 7.5 to 10 cc on day 4; and 8 x 20 to 25 cc
on day 5.

10
 - IV D5% : ¼ NS 0,9% 340 cc per 24 hours
- IV Aminosteril 66,6 cc per 24 hours until the day 5th post-operative day
- IV Cefotaxime 200 mg per 12 hours
- IV Metronidazol 40 mg per 8 hours
- IV Paracetamol 40 mg per 8 hours
- Gentamicin cream on anal skin per 12 hours
- Tampons would be removed after 24 hours post-surgery
- Open treatment and antiseptic sprayed into the anus every hour
b. Monitor Planning
- Fluid balance monitoring per hour in the first 24 hours, continued for every 6 hours
once the patient was stable
- Urinary catheter insertion
- NGT insertion and flow for evaluation
- Rectal tube was maintained until day 5 post operation
The patient was transferred back to the Pediatrics Room on the 5th postoperative day with a
weight of 4 kg. The NGT and urinary catheter were removed. Education about personal
hygiene and how to treat patients' surgical wounds was taught to the patient's parents.
Injectable drugs were replaced with oral drugs. Treatment of skin irritation in the gluteal
region due to antiseptic irritation was consulted to the dermatologist. Observations were
conducted for 2 days in the Pediatrics Room before the patient was discharged. The following
are the results of the patient's laboratory examination before being discharged.

Table 5. Laboratory Examination Result V

Data Result Normal Value
CBC
Hemoglobin 11,0 gr/dl 12 - 14 gr/dl
Hematocrit 31 % 37 - 43 %
Leukocyte 13.300 /mm3 5.000 - 10.000 /mm3
Basophil 0,3 % 0-1%
Eosinophil 9,0 % 1-3%
Neutrophil 14,0 % 50 - 70 %
Lymphocyte 66,1 % 20 - 40 %
Monocyte 10,1 % 2-8%
Platelet 344.000 /mm3 150.000 - 450.000 /mm3

11
The planning before the patient was discharged is as the following.
a. Therapy Planning
- Cephadroxyl syr 2x1/8 cth
- Sanmol drop 3x0,4 cc
- Gentamicin cream in gluteal region 2x1
b. Monitor Planning
- Complication monitoring
c. Education Planning
- Personal hygiene education towards the patient’s parents
- Education on the upcoming actions namely control to the Polyclinic on the 14th post-
operative day for preparation of first anal dilation procedure at OK
- Notification of the risk of complications and long-term monitoring activities, i.e.
patients are monitored every month for 3 years.

9. Follow Up
The patient went for control to the Pediatric Surgery Polyclinic for the first post-surgery anal
dilation at OK, then a laboratory examination was carried out with the following results.

Table 5. Laboratory Examination Result V

Data Result Normal Value
Complete Blood Count (CBC)
Hemoglobin 9,9 gr/dl 12 - 14 gr/dl
Hematocrit 28 % 37 - 43 %
Leukocyte 10.800 /mm3 5.000 - 10.000 /mm3
Basophil 0,4 % 0-1%
Eosinophil 8,4 % 1-3%
Neutrophil 22,4 % 50 - 70 %
Lymphocyte 58,2 % 20 - 40 %
Monocyte 10,6 % 2-8%
Platelet 585.000 /mm3 150.000 - 450.000 /mm3
Bleeding Time 2,5 minutes 1 - 3 minutes

12
 Clotting Time 4 minutes 1 - 6 minutes
Comprehensive Metabolic Panel
Glucose 105 mg/dl 60 - 150 mg/dl
Serology
HBsAg Negative Negative

Improvement of the patient's condition before the post-surgical anal dilation was carried out
with the following planning.
a. Therapy Planning
- IV D5% : ¼ NS 0,9% 410 cc per 24 hours
- IV PRC 40 cc in 3 hours
- IV Lasix 2 mg before anal dilation
b. Monitor Planning
- 6-8 hours post-transfusion CBC examination
The result of laboratory evaluation can be seen as follows.
Table 6. Laboratory Examination Result VI
Data Result Normal Value
Complete Blood Count (CBC)
Hemoglobin 13,3 gr/dl 12 - 14 gr/dl
Hematocrit 38 % 37 - 43 %
Leukocyte 13.800 /mm3 5.000 - 10.000 /mm3
Basophil 0,2 % 0-1%
Eosinophil 4,2 % 1-3%
Neutrophil 41,1 % 50 - 70 %
Lymphocyte 41,6 % 20 - 40 %
Monocyte 12,9 % 2-8%
Platelet 531.000 /mm3 150.000 - 450.000 /mm3

The anal dilation was performed under general anesthesia with the following procedure.
a. The patient was in the lithotomy position, then an antiseptic procedure was performed.
b. Anal dilation was conducted using dilator number 6, 7, 8, 9, 10, 11, 12, dan 13 and gel
lubricant
c. The evaluation of the coloanal anastomosis was carried out for 10 minutes at the number
13 dilator.
d. The number 13 dilator was removed followed by bleeding evaluation.
e. The patient was cleaned and the anal dilation was finished.
The figures of the anal dilation are as follows.

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 Figure 7. The Anal Dilation Procedure

The anal dilation lasted for 30 minutes with 0.1 cc bleeding during the procedure. Twelve
hours after the anal dilation, the patient was discharged and assigned for control after 30
days.

DISCUSSION
Infants with HD will have corrective surgery depending on their overall health and degree of
colonic distension. The goal of preoperative management is to prevent enterocolitis because
the patients are at risk for HAEC that represents the leading cause of serious morbidity and
mortality. The etiology of HAEC is unknown and is probably multifactorial. Stasis caused by
functional obstruction permits bacterial overgrowth with secondary infection, such as
Clostridium difficile or rotavirus. The classic manifestations of HAEC include abdominal
distention, fever, and diarrhea. However, there is a HAEC score has been developed. A score
of 10 or higher was associated with a positive diagnosis of HAEC by an international panel of
experts. The working diagnosis of this case was suspect HAEC and the scoring can be seen in
the following table.5
Table 7. HAEC Score
Criteria Score Value Case Score
History
Diarrhea with explosive stool 2 2
Diarrhea with foul-smelling stool 2 2
Diarrhea with bloody stool 1 0
Previous history of enterocolitis 1 0
Clinical Findings
Explosive discharge of gas and stool on rectal exam 2 1
Distended abdomen 2 1
Decreased peripheral perfusion 1 1

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 Lethargy 1 0
Fever 1 0
Radiology Examination
Multiple air-fluid levels 1 0
Dilated loops of bowel 1 1
Sawtooth appearance with irregular mucosal lining 1 0
Cut-off sign in rectosigmoid with absence of distal air 1 1
Pneumatosis 1 0
Laboratory Examination
Leukocytosis 1 1
Shift to left 1 1
Total 20 14

The American Pediatric Surgical Association Board of Governors has categorized the clinical
suspicion and severity of HAEC into three grades based on history, physical examination,
and imaging studies. This category is not intended as a scoring system, but rather a decision-
support tool to ensure that all of the relevant history, examination and radiographic findings
are considered. In general, presence of higher grade findings should prompt providers to
assign the higher grade.6

Table 8. Guideline for the Diagnosis of HAEC

Grade Description History Clinical Finding Radiology
I Possible - Anorexia - Mild abdominal - Normal
HAEC - Diarrhea distention - Mild ileus gas
pattern
II Definite - Past episode of - Fever - Ileus gas pattern
HAEC HAEC - Tachycardia - Air/fluid levels
- Explosive - Abdominal - Dilated loops of
diarrhea distention bowel
- Fever - Abdominal - Recto sigmoid
- Lethargy tenderness cutoff
- Explosive gas /
stool
III Severe - Obstipation - Decrease peripheral - Pneumatosis
HAEC - Obtunded perfusion - Pneumoperitoneum
- Hypotension
- Altered mentation
- Peritonitis

15
Treatment of HAEC is based on the severity of the clinical presentation based of The
American Pediatric Surgical Association Board of Governors Guidelines, as detailed in Table
9.6
Table 9. Guideline for the Management of HAEC
Grade Dispotition Diet Antibiotic Irrigation
I Outpatient - Oral hidration - Metronidazol PO Consider rectal
irrigation
II Outpatient - Clear liquid or - Metronidazol PO or Rectal irrigation
or inpatient NPO IV
- IV Hidration - Consider broad
spectrum coverage
IV
III Inpatient, - NPO - Metronidazol IV Rectal irrigation
possible - IV Hidration - Broad spectrum
ICU coverage IV

The risk of HAEC may be decreased by using preventive measures such as routine irrigations
or chronic administration of Metronidazole or probiotic agents. In the pre-operative
management, the patient in this case had Metronidazole 40 mg and Cefotaxime 200 mg, a
broad spectrum antibiotic, as a combination of antibiotics. Both was given 7 days before
operation and maintained until day 7 post-operation.

1. Pre-Operative Care
Most affected patients present during the neonatal period with abdominal distension, bilious
vomiting, and feeding intolerance. Delayed passage of meconium beyond the first 24 hours is
present in approximately 90%. It is also important to obtain a plain radiograph 24 hours later
using a water-soluble material and retention of the contrast is very suggestive for HD.
Patients presenting later in childhood have severe chronic constipation and it can be difficult
to differentiate HD from the more common causes. Clinical features pointing to the diagnosis
include delayed passage of meconium at birth, failure to thrive, abdominal distention, and
dependence on enemas without significant encopresis. In this case, retention of contrast is
seen on the post-evacuation film, which was obtained 24 hours after the contrast enema. Once
the diagnosis of HD is suspected, it must be confirmed by rectal biopsy, but the biopsy was
planned during the operation day in this case.5

16
In the pre-operative care, the patient should be appropriately resuscitated with intravenous
fluids and treated with broad-spectrum antibiotics, nasogastric drainage, and rectal
decompression using rectal stimulation and/or irrigation. Patients with associated
abnormalities such as cardiac disease must be thoroughly evaluated prior to operative
correction. Once the infant or child has been resuscitated and stabilized, the operation can be
done semi-electively.5

2. Operative
The goals of surgical management for HD are to remove the aganglionic bowel and
reconstruct the intestinal tract by bringing the normally innervated bowel down to the anus
while preserving normal sphincter function. The most commonly performed operations are
the Swenson, Duhamel, and Soave procedures, although the Rehbein and State procedures
are still performed in some centers.2,5
In the 1980s, a number of surgeons reported series of single-stage pull-through operations
and many reports suggested that a one-stage approach was safe, avoided the morbidity of
stomas in infants, and was more cost effective. However, a stoma may still be needed in
severe enterocolitis, perforation, malnutrition, or massively dilated proximal bowel, and in
situations when it is not possible to reliably identify the transition zone on frozen section. In
this case, the frozen section biopsy was performed during the surgery and diagnosis of HD is
made. The figures of algorithm for diagnosis of HD is as follows.5

Figure 8. Algorithm for Diagnosis of HD

17
The transanal approach has a low complication rate, requires minimal postoperative
analgesia, and is associated.5

3. Post-Operative Care
Most patients undergoing transanal pull-through can be fed immediately and discharged
within 24-48 hours. The anastomosis is calibrated with an appropriately sized dilator or finger
1–2 weeks after the procedure. Although most surgeons instruct the parents to perform daily
dilatations, a program of weekly calibration by the surgeon is less traumatic and is associated
with similar outcomes. In this case, the combination of both was performed. Long-term
problems in children with HD include ongoing obstructive symptoms, soiling, and
enterocolitis. It is important for the surgeon to follow these children closely, at least until they
are through the toilet training process.5
a. Obstructive symptoms
Obstructive symptoms may take the form of abdominal distension, bloating, vomiting, or
ongoing severe constipation. There are five major reasons for these symptoms following a
pull-through: mechanical obstruction, recurrent or acquired aganglionosis, disordered
motility in the residual colon or small bowel, internal sphincter achalasia, or functional
megacolon caused by stool-holding behavior. The clinician will have much greater
success in managing these difficult patients if an organized approach is taken and the
algorithm for the investigation and management of the child with obstructive symptoms
following a pull-through is as follows.5
Figure 9. Algorithm for the Investigation and Management of the Child with
Obstructive Symptoms Following a Pull-Through

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b. Fecal soiling
There are three broad causes for soiling after a pull-through: abnormal sphincter function,
abnormal sensation, or pseudo-incontinence. There are two forms of abnormal sensation.
The first is lack of sensation of a full rectum, which can also be identified using anorectal
manometry, and the other is an inability to detect the difference between gas and stool.
This problem is usually due to loss of the transitional epithelium because the anastomosis
was performed below the dentate line. Neither sphincter weakness nor abnormal sensation
is amenable to a surgical solution. Most of these children are best managed using a bowel
routine, which may include a constipating diet, stimulant laxatives, and rectal or
antegrade enemas. Biofeedback training has been advocated, especially for those children
with sphincter weakness. In some cases, the child is best served by a colostomy.5
c. Enterocolitis

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 Enterocolitis may be present both before and after operative correction. The treatment of
postoperative HAEC involves nasogastric drainage, intravenous fluids, broad-spectrum
antibiotics, and decompression of the rectum and colon using rectal stimulation or
irrigations. Despite the relatively common occurrence of postoperative obstructive
symptoms, soiling, and enterocolitis, most resolve after the first 5 years of life. As
enterocolitis is the most common cause of death in HD and can occur postoperatively, it
is very important that the surgeon educates the family.5,6
d. HD-associated inflammatory bowel disease
There is a small group of children with HD who develop a condition resembling Crohn
disease when they get older. This consists of chronic inflammation, and in some cases,
fistula formation. Risk factors for this condition include long-segment disease and
trisomy 21. It is unclear whether this condition is a chronic form of HAEC or has a
separate etiology.5

CONCLUSION
The diagnosis and patient management had been done appropriately following the theories
and guidelines.

REFERENCES
1. Langer JC. Hirschsprung disease. In: Holcomb III GW, Murphy PJ, Ostlie DJ, editors.
Ashcraft's Pediatric Surgery. 6th ed. New York: Elsevier Saunders; 2014. p. 484-98.
2. Lotfollahzadeh S, Taherian M, Anand S. Hirschsprung Disease. [Updated 2021 May 14].
In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan-. Available
from: https://www.ncbi.nlm.nih.gov/books/NBK562142
3. Andro PW , Alpha FA, Adria H , Gusti MR. Risk factors influencing enterocolitis
development in pediatric patients with hirschsprung’s disease. Jurnal Berkala
Epidemiologi. 2020;8(3)218–227 DOI: 10.20473/jbe.v8i32020.
4. Keputusan Menteri Kesehatan Republik Indonesia Nomor HK.01.07/Menkes/474/2017
Tentang Pedoman Nasional Pelayanan Kedokteran Tata Laksana Penyakit Hirschprung
5. Langer JC. Hirschsprung disease. In: Holcomb III GW, Murphy PJ, Ostlie DJ, editors.
Ashcraft's Pediatric Surgery. 7th ed. New York: Elsevier Saunders; 2020. p. 557-73
6. Gosain A, Frykman PK, Cowles RA, et al. Guidelines for the diagnosis and management
of Hirschsprung-associated enterocolitis. Pediatr Surg Int. 2017;33(5):517-521.
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