CASE PRESENTATION

Informant: Mother
Reliability: 90%

General data:
P.D.E, 21 day old neonate, male, filipino, Roman
Catholic, single born on March 20, 2014 currently
residing at Tagbilaran City admitted for the first
time last April 10, 2014 at 6:51 PM at Chonghua
Hospital.


Chief Complaint: Constipation and Fever

History of Present Illness:
19 days PTA, patient was two days old when
he was able to passed out small amounts of loose
stool, then the mother noticed that the patients
abdomen was distended, she then sought consult
to a private pediatrician and was advised for
admission at Gov. Celestino Gallares Memorial
Hospital.





He underwent an abdomen X-ray and was
diagnosed to have Hirschsprung Disease.
18days PTA, he was then submitted to
underwent bowel irrigation and rectal
stimulation, he was able to pass out stools 9x
that day (consistency and character not recalled).
Parents decided to bring the patient home since
he was able to pass out stools. For the next six
days patient didnt have any difficulty defecating.

12 days PTA, patient started to have
constipation again and had small hard stools.
Mother tried to stimulate his rectum and he was
able to defecate.
6 days PTA, patients condition persisted.
4 days PTA, patients parents brought him to
a private pediatrician at Tagbilaran, Bohol where
he was given Glycerine suppository half tab,
which provided no relief of constipation. He was
then referred to a Pediatric Surgeon and bowel
irrigation was done and was able to pass out large
amount of stools.

3 days PTA, patient had fever with a highest
document temperature of 39 degree Celsius per
axilla and was admitted to Ramiro Community
Hospital where barium enema was done. He was
started with Cefuroxime sodium 250mg.
Metronidazole 500mg/100ml and Paracetamol
300mg/2ml ampule.
1 day PTA, patients parents decided to bring
him to Cebu for second opinion and thus this
admission.

PRENATAL
Mothers age at birth was 23 years old, a
G2P1(1001), having her first pre-natal visit at 4
weeks AOG. During the course of pregnancy during
7
th
month, patients mother developed PIH
(pregnancy induce Hypertension) and was given
methyldopa (dosage was unrecalled), 9th month,
she developed UTI, Cefuroxime 500mg for 5 days 2x
a day was given, during the later weeks she went
into preterm labor and was told that she had
placenta previa and polyhydramnios .
Heredo-familial diseases; HPN, DM, Bronchial
Asthma and Stroke. Medications taken;
Multivitamins, Calcium, Iron, Folic acid. Mother
couldnt remember her total weight gain during
the course of pregnancy.
Mother does not drink alcoholic beverages,
neither does she smokes nor use addictive drugs.
Immunizations; Hep B unrecalled when, and
tetanus toxiod.

NATAL
Patient was delivered via Repeat Cesarean
section secondary to cephalopelvic
disproportion, no complications noted, having a
birth weight of 7 lbs, Apgar score unrecalled but
mother verbalized hearing her baby cry. Ballards
score unrecalled, head circumference, chest
circumference and birth length were unrecalled
as well.
POST NATAL
Developmental milestones, when at prone
position, patients legs are more extended,
holds chin up and turns head; at supine
position patients head lags when pulled to
sitting position. Visual; watches person and
follows moving object. Social: body
movements in cadence with voice of other in
social contact, begins to smile.
Immunization status, 1
st
dose of hepatitis B and
BCG were given. Feeding mixed with breast milk
and formula milk (S26, 1:1 dilution)

Family and Social
The patient is first baby of the family, currently
residing at Tagbilaran Bohol, Father works as a
government employee while the mother is a
nurse at a local hospital. Their house is situated
few kilometers away from the health center.
Water for drinking is supplied by a water refilling
station, while water for washing is from local
water supplier. During the course of pregnancy
the mother most of the time cooks their own
food and occasionally buys from a carenderia.
They have 1 toilet separated from the bath room.
2 bed room house.
General Survey
Awake, conscious, febrile, not cooperative with
moderate nutritional status, not in respiratory distress,
with the following vital signs:

Temperature : 38.4 C right armpit
Blood Pressure = Unable to perform
Heart Rate : 149 beats per minute
Respiratory Rate : 38 cycles per minute
Head Circumference : 35.5 cm
Abdominal Girth: 32cm
Length : 54 cm
Weight : 3.4 kg

Physical exam
Skin: pale in color, texture is normal, good
turgor, no lesions noted
HEENT: head circumference 35.5 cm,
normocephalic, evenly distributed fine hair,
anterior and posterior fontanels are both
open and flat, anicteric sclera, ROR, no naso-
aural discharges, pale and dry lips, uninflamed
gums, pink and moist tongue, no
tonsillopharyngeal congestion
Chest and lungs: no scars, abrasions and rash
noted, equal chest expansion, no retractions,
breast is symmetrical, no lumps or masses
noted, no stridor, wheeze, rhonchi or rales
heard. Clear breath sounds, no crepitations
noted
Cardiovascular: apical impulse at 4
th
ICS, no
pericordial bulging, distinct S1 and S2, regular
rhythm and normal rate, holosystolic murmur
left parasternal area grade 2
Abdomen: globular with prominent bowel loops L
side, abdominal girth ( 32 cm (04/11/14)),
hypoactive bowel sounds (4 cpm) , tympanitic
DRE: no anal lesions, fistulas, smooth rectal
mucosa, tight sphincter tone, no palpable masses,
fecal maternal meconium
Genito-urinary: grossly male, testes descended, no
hydrocele noted, tanner stage PH1G1
Back: spine at midline, no dimpling, no hair tufts,
kidney punch not assessed
Extremities: strong peripheral pulses, CRT < 2
seconds
Neurologic: GCS of E4V2M3,
CNI not done
CN II pupils: reactive and equal direct and
consensual both eyes
CN III, IV, VI , EOM: full ROM by observation
CN V good suck, facial muscle: strong
CN VII no facial asymmetry
CN VIII turns head to sound
CN IX, X gag reflex (+), good swallow
CN XI not done
CN XII tongue midline at rest

CEREBELLAR: not done
SENSORY pain (+)
DTRs average/ normal, ankle clonus absent
Primitive reflexes: moro, rooting, sucking, grasp,
tonic neck, babinski



Musculoskeletal: no deformities noted
Progress notes
Subjective: good cry, good activity, irritable
Objective : examined pt on bed, awake, crying, not in
respiratory distress, afebrile
Skin: warm to touch, tinge of pale, no jaundice noted, no
unusual lesions noted
HEENT: anicteric sclerae, ROR (+), moist lips and oral mucosa,
no tonsilopharyngeal congestion
C/L: adynamic pericordium, holosystolic heart sound at the left
parasternal border Grade 2, normal rate and regular rhythm
Abdomen: soft, irregular shape (prominence on the left side)
hypoactive bowel sounds (4cpm), tight sphincter tone, smooth
rectal mucosa, fecal material on examining finger
Extremities: strong pulses, CRT <2 seconds
Assessment: Hirschprungs Disease
Plan:
04/10/14 (7:35 PM)
-monitor v/s q 4
monitor input and output very shift
-diet: per advice of surgery
Start venoclysis with D10 IMB as follows:
-D 5 IMB 115.7ML
-D50 WATER 14.5ML
==130 ml to run at 21 ml per hour refill every 6 hours
-laboratories
-repeat CBC
-URINALYSIS
-serum sodium and potassium
-HGT NOW (116 HGT)
-BLOOD CULTURE AND SENSITIVITY
-CHEST X RAY APL VIEW


Refer for poor
activity tachypnea, dyspnea,
and other uncasualties
-insert orogastric tube French
8
-measure abdominal girth
every shift and chart
04/10/14 (8.30pm)
-for colonic irrigation 2x a day
-NPO temporarily
4-11-14 (8:50am)
-Ranitidine (ulcin) 50mg/2ml
ampule give 5mg via slow IVTT
every 8 hours
(9:30 a.m.)
-start partial parenteral
nutrition (Aminosteril)
-for repeat serum sodium,
potassium tomorrow 4-12-14 to
include protime APTT

(10:00 A.M)
-REVISE 50 FLUID AS FOLLOWS
--0.5 IMB 9.17ML
--D50 WATER 11.3ML

=103ML REFILL every 6 hours to
run at 17ml per hour for 12 hours
while on Aminosteril

-piggyback aminosteril (6%)
100ml to run at 8ml per hour for
12 hours

(10:50a.m)
-for 2d echo today 4pm
(11am)
-colonic irrigation alone with 1liter PNSS
(11:40a.m.)
-clear liquids via dropper
-keep OGT open at all time
-schedule for rectal biopsy on Monday 4-14-14, IPM
once cleared with pedia service
-for colonic irrigation every HS at French 16 Robinsons
catheter
(12pm.)
-reschedule 2d echo tomorrow 4-12-14 10 am
(4pm)
-to include serum creatinine tomorrow
blood extraction



(5pm)
-after aminosteril is given for 12 hours
-IV TO follow with DI0 IMB as follows
D5 IMB 115.7ML
D50 water 14.3ml
==130 ml to run at 21 ml per hour refill every six
hours
S: afebrile, able to pass out small amounts of stool,
no changes in bladder habits, no other significant
subjective complaints
O : examined pt on bed, sleeping, not in
respiratory distress, afebrile
Skin: warm to touch, no jaundice noted, no unusual
lesions noted
HEENT: anicteric sclerae, ROR (+), moist lips and oral
mucosa, no tonsilopharyngeal congestion
C/L: adynamic pericordium,, normal rate and regular
rhythm
Abdomen: soft, irregular shape (prominence on the left
side) normoactive bowel sounds (15cpm)
Extremities: strong pulses, CRT <2 seconds


A: Hirschsprungs disease
P: 4-12-14 (12pm)
-next IVF refill
D5IMB 90.4
KCL 1.3 (2.5MGKCL)
D50W 11.3
=103ML to regulate to 177 cc/hr FOR 12 hours
-repeat serum potassium and HGT 8 am ( 112) Tomorrow 4-
13-14
(1.30pm)
-after 12 hours of refills with KCL and aminosteril
-IVF to follow with D10 IMB as follows
D5IMB 115.7ML
D50WATER 14.3ML
== 130 ML TO RUN at 21ml per hour refill every 6
hours for 12 hours
(4.45pm)
-get serum sodium and potassium tomorrow at 10 am
instead of 8 am
S: good cry, appears weak, was able to pass
out small amount of stool, no change in bowel
habits, abdominal girth 32 cm
O: Afebrile, not in respiratory distress
Skin: warm to touch, no jaundice noted, no
unusual lesions noted
HEENT: anicteric sclerae, ROR (+), moist lips and
oral mucosa, no tonsilopharyngeal congestion
C/L: adynamic pericordium,, normal rate and
regular rhythm
Abdomen: soft, irregular shape (prominence on
the left side) normoactive bowel sounds (15cpm),
Extremities: strong pulses, CRT <2 seconds
A: Hirschsprungs disease

P: 04/13/14(9.50am)
-may have clear liquids per OGT maximum at 15ml every
feeding
-NPO AT 6 A.M TOMORROW 4-14-14
-to include reticulocyte count next blood extraction and save
serum for possible mismatching
(11 a.m)
-secure 1 unit of packed rbc of patients blood type properly
screened and cross-matched, process into adequate, subdivide
1 into 40cc each
-once available transfuse 40ml of packed RBC for 4 hours
-give the following medication 30 minutes prior to transfusion
- paracetamol 100mg/ml drops give 0.5ml orally one
dose only
-closed mainline while on transfusion
-monitor vital signs every 15minutes for the first hour, every 30
minutes for the second hour, every hour thereafter

(11: 20am)
-schedule patient for rectal biopsy with frozen section possible levelling
colostomy tomorrow 4-14-14 4pm
-frozen section care -for colonic irrigation until clear tonight
(6pm)
-may go ahead with contemplated procedure for rectal biopsy with frozen
section possible levelling colostomy tomorrow under general anaesthesia
Patient is at minimal rests ASA classification class 1
(7.30pm)
-diphenhydramine (Benadryl) 12.5mg/5ml give 1ml orally 30 minutes
before blood transfusion
(11.30pm)
-may have last feeding of formula milk before 7 am, may have water until
11 am then NPO thereafter


S: able to pass out small amount of stools after
irrigation, status post blood transfusion, was given
diphenhydramine due to allergic reactions to blood
transfusion, no other significant subjective
complaints
O: afebrile, vital signs stable HR: 120bpm, RR: 76
cpm, temp: 37.3 degrees Celsius
Skin: warm to touch, no jaundice noted, no unusual
lesions noted
HEENT: anicteric sclerae, ROR (+), moist lips and oral
mucosa, no tonsilopharyngeal congestion
C/L: adynamic pericordium,, normal rate and regular
rhythm
Abdomen: soft, irregular shape (prominence on the left
side) normoactive bowel sounds (10cpm)
Extremities: strong pulses, CRT <2 seconds


4-14-14 (6AM)
-IVF follow D5IMB 500ML at 10-15ml per hour

6AM till 6PM (OPERATING ROOM) rectal biopsy with frozen Leveling of sigmoid
colostomy

6.20pm
-post op only
-to PACU
-TPR every 4 hours
-1 to every shift, chart colostomy output every shift separately
-NPO
-NF D5IMB 500 ml at 10-15ml/hour
-save specimen for histopathology
-meds:
Cefuroxime (kelox) 250mg/10ml give 115mg IVTT EVERY 8 hours
Metronidazole (fLAgyl) 500 mg/100ml IV GIVE 50mg IV drop every
12 hours
Ranitidne (Ulcin) 50mg/amp 5mg IVTT every 8 hours
4-14-14
-to PACU
-Routine care and monitoring
-regulate DLR at 30cc per hour
-medication
1. Nalbuphine (Nubcin) 0.5mg slow IV to 5cc
PNSS every 6 hours for 2 doses. First dose 12
midnight
2. paracetamol (HEXAL) 40mg slow IV every 6
hours x4 doses first dose 12 midnight
(6.20pm )
(D5IMB 500 AT 20 CC/HR
(6.30PM)
HGT NOW

(7.20PM)
-SUMMARY OF orders
-NPO
-keep OGT open to admin
-keep thermoregulate between 36.5-37.5C
-oxygen 1L per minute o2 sat more than 95%
-maintain IVF d5imb EVERY 20 CC/HR
-LAB cbc at 8pm
-Meds
1. Cefuroxime(KEFUROX) 115mg IVTT EVERY8 HOURS
2. METRONIDAZOLE (FLAGYL) 50MG IVTT every 12 hours
3. Ranitidine (Ulcin) 5mg slow IVTT EVERY 8 hours
4. Nalbuphine (nabuin) 0.5mg slow IV diluted to 5cc
PNSS q 6hours for 2 doses. First dose 12 midnight
5. Paracetamol 40mg slow IV every 6 hours x4 doses first
dose 12 midnight

(7.25pm )
v/s hourly
-I AND O q 4hr absolute figures
(9:40pm)
-may discharge from PCU
(12AM)
-ivf to follow: D5IMB 500ml at 20cc/hr
-IVF to follow: D5IMB 500ML at 20cc/hr
4-15-14 (10am)
-unhook present fluid
Start d10 IMB prepared as follows
D5IMB 106.8ML
D5 W 13.2 ML
===120ML refill every 12 hours to run at
10ml per hour
-aminosteril (6%) piggyback via IV drip to run at 8ml per
hour for 12 hours


(4-16-14) 2am
-may start oral feedings
-remove OGT
(9.40AM)
-MAY have milk feeding 30ml q 3 hours with strict
aspiration precaution
-consume open stock of aminosteril and D10IMB
(4-17-14) 10.30AM
-SHIFT TO ISA once aminosteril and D10 IMB
Consumed

(11am)
Give last dose of metronidazole at 2pm today shift
to metronidazole( flagly) 125mg/5ml suspension
give 2ml orally three times a day, to give first dose
tomorrow AM
- CEFUROXIME (ZINNAT) 125MG/ML
SUSPENSION GIVEN 1.5ML ORALLY TWICE A
DAY GIVE first dose now
- multivitamins (cherifer drops) given 1ml orally
once a day
- refer to PROD ONCE tolerated or not
- to consume opened stocked of ranitidine IV

Patient was discharge on April 19 2014, stable
v/s, colostomy care was instructed, no other
complications noted.






COMPLETE BLOOD COUNT RESULT

Blood Count 04/11/2014 REFERENCE 04/14/2014
WHITE BLOODCELLS
RED BLOOD CELLS
HEMOGLOBIN
HEMATOCRIT
PLATELET
14.95
3.42
10.5
30.9
452
4.8-10.8
4.7-6.1
14.0-18.0
42.0-52.0
130-400
23.63
5.12 N
15.3 N
45.3 N
410
Blood Indices
MCV
MCH
MCHC
RDW
PDW
MPV

90.4 N
30.7 N
34.0 N
15.5 N
11.2 N
10.0 N
80-94
27.0-31.0
33.0-37.0
11-16
9.0-14.0
7.2-11.1
88.5 N
29.9 N
33.8 N
16.2
11.3 N
10.2 N
Relative Differential Count
Neutrophil (%)
Lymphocytes (%)
Monocytes (%)
Eosinophils (%)
Basophils (%)
31.0
52.0
13.0
4.0 N
0.0 N
40-74
19-48
3.4-9.0
0.0-7.0
0.0-1.5
76.3
17.4
5.0 N
1.1 N
0.2 N
Absolute Differential Count
Neutrophil (#)
Lymphocytes (#)
Monocytes (#)
Eosinophils (#)
Basophils (#)
4.60 N
7.80
2.00
0.60 N
0.00 N
1.9-8.0
0.9-5.2
0.16-1.00
0.0-0.8
0.0-0.2
18.02
4.12 N
1.19
0.26 N
0.04 N
COMPLETE BLOOD COUNT Continuation

Reference 04/12/2014 04/13/2014
Reticulocyte count
12
Reticulocyte Count Infant
PARTIAL THROMBOPLASTIN TIME
(PTT/APTT)

Partial Thromboplastin Time
Patient
Control
26.4-36.7 33.7 N
30.0

Prothrombin time
Patient
Activity
INR
Control
Control Activity

>70%
1.21
14.3
86
1.09
13.3
100.0

FULLY AUTOMATED ROUTINE
URINALYSIS UNCENTRIFUGED
SPECIMEN
RESULT
Physical Characteristics 04/11/2014
(CHH)
REFERENCE 04/08/2014
(RAMIRO COMMUNITY
HOSPITAL)
Color LIGHT YELLOW YELLOW
Transparency CLEAR CLEAR
pH 8.0 4.8-8.0 6.0 N
Specific Gravity 1.010 N 1.002- 1.006 1.020 N
Chemical Characteristics
Protein NEGATIVE NEGATIVE TRACE
Glucose NEGATIVE NEGATIVE NEGATIVE
Ketone NEGATIVE NEGATIVE NEGATIVE
Urobilinogen NEGATIVE NEGATIVE -
Leukocytes NEGATIVE NEGATIVE -
Blood/Hb NEGATIVE NEGATIVE NEGATIVE
Bilirubin NEGATIVE NEGATIVE -
Nitrite NEGATIVE NEGATIVE -
04/11/2014
(CHH)
04/08/2014
(RAMIRO
COMMUNITY
HOSPITAL)

04/08/2014
(RAMIRO
COMMUNITY
HOSPITAL)
Microscopic Findings S.I UNIT CONVENTION
AL UNIT
Red Blood Cells 1 N 4.7 N 0.8 N
White Blood Cells 3 N 18.3 3.3
Bacteria 13 N 74.7 N 13.4 N
Squamous Epithelial Cells 6 N 37.2 6.7
Cast 0 N 7.34 21.29
BLOOD CULTURE W/ARD
AND OTHER BODY FLUIDS
BLOOD (LEFT HAND)
CULTURE
No growth after 5 days
MICROBIOLOGY REPORT
04/11/2014

Date
Performed
EXAMINATION SPECIMEN RESULT
04/14/2014 Frozen Section w/
Biopsy (additional
Surgical Pathology)
Sero-Muscular
Layer Proximal
Sigmoid and
Midsigmoid
Many Ganglion
Cells Identified on
Both Specimens
04/14/2014 Frozen Section w/
Biopsy
Rectal Wall No ganglion Cells
Identified
FROZEN SECTION REPORT

COMPATIBILITY TEST OR CROSS MATCHING RESULT UNIT
Compatibilty Test or Cross Matching COMPATIBLE
RETICULOCYTE COUNT RESULT REFERENCE UNIT
Reticulocyte Count Infant 12 20-60 10^-3
4/13/14 1:05 pm
4/13/14 5:14 pm
REMARKS: 1UNIT PRBC, "B" POSITIVE
TEST RESULT
04/10/2014 04/12/2014 04/13/2014
CREATININE 0.6 N
SODIUM (SERUM) 136.0 N 131.0 137.0 N
POTASSIUM 2.7 2.9 4.8 N
CLINICAL CHEMISTRY REPORT

ECHOCARDIOGRAPHIC REPORT

04/12/2014
SUMMARY OF INTERPRETATION:

Normal abdominal situs
Levocardia
Patent Foramen Ovale measuring 0.18cm
Intact Interventricular Septum
Atrioventricular and ventriculoarterial concordance
Normal Chamber Size
Good Left Ventricular Systolic Function
Pulmonary Artery Pressure 40 mmHg by Pulmonary Acceleration Time
Good-sized confluent arteries
Left sided aortic arch
No coarctation


X Ray

04/10/2014
Examination: Chest X-ray- PA and Lateral/AP and Lateral

Reports:
There are faint linear, tiny nodular, hazy, and ill-defined densities in the
right paracardiac, left retrocardiac, and left parahilar areas. The rest of the lungs are
clear. Heart is not enlarged. The medial aspect of both hemidiaphragms are slightly ill
defined. Both costophrenic sulci are intact. The tracheal air column is at the midline.
No discrete adenopathy is demonstrated. The superior mediastinum is widened. The
visualized bony structures are unremarkable. A feeding tube is seen with its tip well
within the gastric fundus.

IMPRESSION:
Mild Inflammatory Process in the Right Paracardiac, Left Retrocardiac, and Left
Perihilar Areas.
Widened Superior Mediastinum Due To Prominent Thymic Shadow.
Presence of a Feeding Tube in Place.

Partial Thromboplastin Time
(PTT/APTT)
RESULT REFERENCE UNIT
Partial Thromboplastin Time

Patient
Control
33.7
30.0
26.4 - 36.7 Sec
Sec
Prothrombin Time

Patient
Activity
INR
Control
Control Activity
14.3
88
1.09
13.3
100.0

> 70%
1.21
Sec
%

Sec
%
HEMATOLOGY REPORT
4/12/14 7:29 am


Clinical Formulation
Primary Impression
Hirschsprung Disease
Neonate
Abdominal distention
Small pellet stool or watery stool
Explosive discharge of feces after DRE
(+) Barium enema and biopsy
Differential Diagnosis
1. Intestinal atresia
Newborn
Bilious Emesis
Dilated Bowel
Polyhydramnios
Congential defect (Downs Syndrome, CHD, Annular
pancreas, Esophagial and Anorectal atresia)
Prematurity and Low birth weight
2. Meconium Ileus
Newborn
Cystic Fibrosis
Abdominal Distention
Bilious Vomiting
No passage of meconium
Poor Feeding
Septic Facie
3. Meconium Plug Syndrome
Newborn
Delayed passage of meconium
Intestinal Dilatation
Contrast Enema
Transient
Case Discussion
Hirschsprung Disease
Congenital Aganglionic Megacolon
absence of ganglionic cells in submucosal and
myenteric plexus
1 in 5000 livebirths
4:1 in short segment disease
1:1 in long segment disease
associated with other congenital defects

Pathophysiology
Normal Physiology
Neuroblast
migration
Proximal
Distal
(+) Ganglion cells in
bowel wall pexus
Cholinergic Fibers
Adrenergic Fibers
Inhibition of contraction
Contraction
Normal Motility
Neuroblast migration
Proximal Distal
Absence of neural innervation in the bowel wall
Inadequate relaxation of bowel wall and bowel wall hypertonicity
INTESTINAL OBSTRUCTION
Histology
Absence of Meissners and Auerbachs plexus
Hypertrophied nerve bundles high in
acetylcholinesterase between muscle layers
and submucosa
Clinical Manifestations
Distended abdomen
Failure to pass meconium
Bilious emesis or aspirates
Feeding intolerance
Failure to thrive with hypoproteinemia
Ultrashort- segment Long- segment
Location Internal sphincter Entire colon and at times
part of small bowel
Symptoms Similar to functional
constipation
Distended abdomen
Failure to pass meconium
Bilious emesis or
aspirates
Feeding intolerance
Labs Ganglion cells present on
rectal suction biopsy,
abnormal anorectal
manometry
Anorectal Manometry
and
Rectal suction biopsy
demonstrates classic
findings but colonic
transition zone cannot be
identified
Treatment Anal botulism injection
Anorectal myomectomy
Ileal anal anastomosis
Diagnosis
Gold Standard: Rectal Suction Biopsy

Sample should be obtained no closer than 2 cm
above the dentate line
Stained for acetylcholinesterase
Positive finding:
Large number of hypertrophied nerve bundles postive
for acetylcholinesterase with absence of ganglion cells


Other diagnostics:

Anorectal Manometry

Normal Finding
- Relaxation of internal anal sphincter in response to
rectal distention

Abnormal Finding
Internal anal sphincter fails to relax in response to
rectal distention
Unprepared Contrast Enema

Classic finding
Abrupt narrow transition zone between normal dilated proximal colon
and the smaller caliber obstructed distal aganglionic segment

In the absence of this finding,
Rectal diameter is equal or smaller than the sigmoid

Supportive Diagnostics

Plain Abdominal Radiograph
CBC
UA
Serum Na and K
Coagulation studies
ECG
Treatment
Definitive: Operative intervention
3 Surgical Options:
1. Swenson Procedure
2. Duhamel Procedure
3. Soave Procedure

Treatment of Choice: Laparoscopic single-stage
endorectal pull- through procedure
Supportive Management

Intravenous hydration
Nasogastric decompression
IV antibiotics if indicated
Cardiac evaluation and genetic testing
Prognosis
Satisfactory for those who undergone surgery
Long term post-operative problems requiring
myectomy or re-do pull- through procedure:
1. Constipation
2. Recurrent enterocolitis
3. Stricture
4. Prolapse
5. Perianal abscess
6. Fecal soiling
BARIUM ENEMA



END