Hirshprungs.pptx

8/12/2019 Hirshprungs.pptx

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CASE PRESENTATION


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Informant: Mother

Reliability: 90%

General data:

P.D.E, 21 day old neonate, male, filipino, RomanCatholic, 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.


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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 foradmission at Gov. Celestino Gallares Memorial

Hospital.


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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.


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12 days PTA, patient started to have

constipation again and had small hard stools.

Mother tried to stimulate his rectum and he wasable to defecate.

6 days PTA, patients condition persisted.

4 days PTA, patients parents brought him toa 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.


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3 days PTA, patient had fever with a highest

document temperature of 39 degree Celsius per

axilla and was admitted to Ramiro CommunityHospital where barium enema was done. He was

started with Cefuroxime sodium 250mg.

Metronidazole 500mg/100ml and Paracetamol300mg/2ml ampule.

1 day PTA, patients parents decided to bring

him to Cebu for second opinion and thus thisadmission.


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PRENATAL

Mothers age at birth was 23 years old, aG2P1(1001), having her first pre-natal visit at 4weeks AOG. During the course of pregnancy during

7th

month, patients mother developed PIH(pregnancy induce Hypertension) and was givenmethyldopa (dosage was unrecalled), 9th month,she developed UTI, Cefuroxime 500mg for 5 days 2x

a day was given, during the later weeks she wentinto preterm labor and was told that she hadplacenta previa and polyhydramnios .


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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.


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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 unrecalledas well.


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POST NATAL

Developmental milestones, when at proneposition, patients legs are more extended,

holds chin up and turns head; at supine

position patients head lags when pulled tositting position. Visual; watches person and

follows moving object. Social: body

movements in cadence with voice of other in

social contact, begins to smile.


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Immunization status, 1stdose of hepatitis B and

BCG were given. Feeding mixed with breast milk

and formula milk (S26, 1:1 dilution)


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Family and Social

The patient is first baby of the family, currentlyresiding at Tagbilaran Bohol, Father works as agovernment employee while the mother is anurse at a local hospital. Their house is situated

few kilometers away from the health center.Water for drinking is supplied by a water refillingstation, while water for washing is from localwater supplier. During the course of pregnancy

the mother most of the time cooks their ownfood and occasionally buys from a carenderia.They have 1 toilet separated from the bath room.2 bed room house.


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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


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Physical exam

Skin: pale in color, texture is normal, goodturgor, no lesions noted

HEENT: head circumference 35.5 cm,

normocephalic, evenly distributed fine hair,anterior and posterior fontanels are bothopen and flat, anicteric sclera, ROR, no naso-aural discharges, pale and dry lips, uninflamedgums, pink and moist tongue, notonsillopharyngeal congestion


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Chest and lungs: no scars, abrasions and rash

noted, equal chest expansion, no retractions,

breast is symmetrical, no lumps or massesnoted, no stridor, wheeze, rhonchi or rales

heard. Clear breath sounds, no crepitations

noted Cardiovascular: apical impulse at 4thICS, no

pericordial bulging, distinct S1 and S2, regular

rhythm and normal rate, holosystolic murmurleft parasternal area grade 2


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Abdomen: globular with prominent bowel loops Lside, 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, nohydrocele noted, tanner stage PH1G1

Back: spine at midline, no dimpling, no hair tufts,

kidney punch not assessed Extremities: strong peripheral pulses, CRT < 2

seconds


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Neurologic: GCS of E4V2M3,

CNInot done

CN IIpupils: reactive and equal direct andconsensual both eyes

CN III, IV, VI, EOM: full ROM by observation

CN Vgood suck, facial muscle: strong

CN VIIno facial asymmetry

CN VIIIturns head to sound

CN IX, X

gag reflex (+), good swallow CN XInot done

CN XIItongue midline at rest


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CEREBELLAR: not done

SENSORYpain (+)

DTRsaverage/ normal, ankle clonus absent

Primitive reflexes: moro, rooting, sucking, grasp,

tonic neck, babinski

Musculoskeletal: no deformities noted


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Progress notes

Subjective: good cry, good activity, irritable

Objective : examined pt on bed, awake, crying, not inrespiratory 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 leftparasternal border Grade 2, normal rate and regular rhythm

Abdomen: soft, irregular shape (prominence on the left side)

hypoactive bowel sounds (4cpm), tight sphincter tone, smoothrectal mucosa, fecal material on examining finger

Extremities: strong pulses, CRT


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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


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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 hourswhile on Aminosteril

-piggyback aminosteril (6%)

100ml to run at 8ml per hour for

12 hours

(10 50 )


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(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, IPMonce cleared with pedia service

-for colonic irrigation every HS at French 16 Robinsonscatheter

(12pm.)

-reschedule 2d echo tomorrow 4-12-14 10 am

(4pm)

-to include serum creatinine tomorrow

blood extraction


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(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


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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)



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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 aminstead of 8 am


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S: good cry, appears weak, was able to passout 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, nounusual lesions noted

HEENT: anicteric sclerae, ROR (+), moist lips andoral mucosa, no tonsilopharyngeal congestion

C/L: adynamic pericordium,, normal rate andregular rhythm

Abdomen: soft, irregular shape (prominence onthe left side) normoactive bowel sounds (15cpm),Extremities: strong pulses, CRT


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A: Hirschsprungs disease

P: 04/13/14(9.50am)

-may have clear liquids per OGT maximum at 15ml everyfeeding-NPO AT 6 A.M TOMORROW 4-14-14

-to include reticulocyte count next blood extraction and saveserum for possible mismatching

(11 a.m)

-secure 1 unit of packed rbc of patients blood type properlyscreened and cross-matched, process into adequate, subdivide1 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 onedose only

-closed mainline while on transfusion

-monitor vital signs every 15minutes for the first hour, every 30minutes for the second hour, every hour thereafter


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(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


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S: able to pass out small amount of stools afterirrigation, status post blood transfusion, was givendiphenhydramine due to allergic reactions to blood

transfusion, no other significant subjectivecomplaints

O: afebrile, vital signs stable HR: 120bpm, RR: 76cpm, temp: 37.3 degrees Celsius

Skin: warm to touch, no jaundice noted, no unusuallesions noted

HEENT: anicteric sclerae, ROR (+), moist lips and oralmucosa, no tonsilopharyngeal congestion

C/L: adynamic pericordium,, normal rate and regularrhythm

Abdomen: soft, irregular shape (prominence on the leftside) normoactive bowel sounds (10cpm)



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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


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4-14-14

-to PACU

-Routine care and monitoring

-regulate DLR at 30cc per hour

-medication

1. Nalbuphine (Nubcin) 0.5mg slow IV to 5ccPNSS every 6 hours for 2 doses. First dose 12

midnight2. paracetamol (HEXAL) 40mg slow IV every 6hours x4 doses first dose 12 midnight

(6.20pm )

(D5IMB 500 AT 20 CC/HR(6.30PM)

HGT NOW


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(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 5ccPNSS q 6hours for 2 doses. First dose 12 midnight

5. Paracetamol 40mg slow IV every 6 hours x4 doses firstdose 12 midnight


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(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 at10ml per hour

-aminosteril (6%) piggyback via IV drip to run at 8ml perhour for 12 hours


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(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 IMBConsumed


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(11am)

Give last dose of metronidazole at 2pm today shiftto metronidazole( flagly) 125mg/5ml suspension

give 2ml orally three times a day, to give first dosetomorrow AM

- CEFUROXIME (ZINNAT) 125MG/ML

SUSPENSION GIVEN 1.5ML ORALLY TWICE ADAY 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


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Patient was discharge on April 19 2014, stable

v/s, colostomy care was instructed, no other

complications noted.

COMPLETE BLOOD COUNT RESULT


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Blood Count 04/11/2014 REFERENCE 04/14/2014

WHITE BLOODCELLS

RED BLOOD CELLS

HEMOGLOBIN

HEMATOCRITPLATELET

14.95

3.42

10.5

30.9 452

4.8-10.8

4.7-6.1

14.0-18.0

42.0-52.0130-400

23.63

5.12 N

15.3 N

45.3 N410

Blood Indices

MCV

MCH

MCHC

RDW

PDWMPV

90.4 N

30.7 N

34.0 N

15.5 N

11.2 N10.0 N

80-94

27.0-31.0

33.0-37.0

11-16

9.0-14.07.2-11.1

88.5 N

29.9 N

33.8 N

16.2

11.3 N10.2 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 N0.00 N

1.9-8.0

0.9-5.2

0.16-1.00

0.0-0.80.0-0.2

18.02

4.12 N

1.19

0.26 N0.04 N


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COMPLETE BLOOD COUNT Continuation

Reference 04/12/2014 04/13/2014

Reticulocyte count 12Reticulocyte 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 RESULT


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URINALYSIS UNCENTRIFUGED

SPECIMEN

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 NChemical 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 -


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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


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BLOOD CULTURE W/ARD

AND OTHER BODY FLUIDS

BLOOD (LEFT HAND)

CULTURE

No growth after 5 days

MICROBIOLOGY REPORT

04/11/2014


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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


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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


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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 FunctionPulmonary Artery Pressure 40 mmHg by Pulmonary Acceleration Time

Good-sized confluent arteries

Left sided aortic arch

No coarctation


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X Ray04/10/2014

Examination: Chest X-ray- PA and Lateral/AP and Lateral

Reports:

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

IMPRESSION:

Mild Inflammatory Process in the Right Paracardiac, Left Retrocardiac, and LeftPerihilar Areas.

Widened Superior Mediastinum Due To Prominent Thymic Shadow.

Presence of a Feeding Tube in Place.


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Partial Thromboplastin Time

(PTT/APTT)

RESULT REFERENCE UNIT

Partial Thromboplastin TimePatient

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


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50/80

Clinical Formulation


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Primary Impression


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Hirschsprung Disease

Neonate

Abdominal distention

Small pellet stool or watery stool

Explosive discharge of feces after DRE

(+) Barium enema and biopsy


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Differential Diagnosis


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1. Intestinal atresia

Newborn

Bilious Emesis

Dilated Bowel

Polyhydramnios

Congential defect (Downs Syndrome, CHD, Annularpancreas, Esophagial and Anorectal atresia)

Prematurity and Low birth weight


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2. Meconium Ileus

Newborn

Cystic Fibrosis

Abdominal Distention

Bilious Vomiting

No passage of meconium

Poor Feeding

Septic Facie


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3. Meconium Plug Syndrome

Newborn

Delayed passage of meconium

Intestinal Dilatation

Contrast Enema

Transient


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Case Discussion


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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


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Pathophysiology

Normal Physiology


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Normal Physiology

Neuroblastmigration

Proximal Distal

(+) Ganglion cells in

bowel wall pexus

Cholinergic Fibers Adrenergic Fibers

Inhibition of contractionContraction

Normal Motility


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Neuroblast migration

Proximal Distal

Absence of neural innervation in the bowel wall

Inadequate relaxation of bowel wall and bowel wall hypertonicity

INTESTINAL OBSTRUCTION


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Histology

Absence of Meissnersand Auerbachsplexus

Hypertrophied nerve bundles high in

acetylcholinesterase between muscle layers

and submucosa


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Clinical Manifestations


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Distended abdomen

Failure to pass meconium

Bilious emesis or aspirates

Feeding intolerance

Failure to thrive with hypoproteinemia

Ultrashort segment Long segment


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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


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Diagnosis


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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


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Other diagnostics:

Anorectal Manometry

Normal Finding

- Relaxation of internal anal sphincter in response torectal distention

Abnormal Finding Internal anal sphincter fails to relax in response to

rectal distention


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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

S ti Di ti


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Supportive Diagnostics

Plain Abdominal Radiograph

CBC

UA

Serum Na and K

Coagulation studies

ECG


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Treatment


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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


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Supportive Management

Intravenous hydration

Nasogastric decompression

IV antibiotics if indicated

Cardiac evaluation and genetic testing


75/80

Prognosis


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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


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BARIUM ENEMA


78/80


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END

Hirshprungs.pptx

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