Is Two0Always

Better Than One

Department of Pediatrics

Brokenshire Integrated Health Ministries, Inc.

October 29, 2020

 Z.E.

18 days old

male

NOT ACTUAL PHOTO

 HISTORY OF PRESENT ILLNESS

10 hrs PTA
• sudden decreased milk intake
• 1 episode of postprandial vomiting
ADMISSION

5 hrs PTA
• poor suck
• vomiting - projectile, bilious
• fever 37.8°C
BIRTH HISTORY

Delivered full term to a 36 yo G2P2 mother via

Repeat CS

Birthweight: 2.99 kg (AGA)

Ballard score: 38 weeks
APGAR score: 8,9

Able to void and pass out meconium

Discharged as well baby at 48 hrs old
FEEDING HISTORY

Exclusively breastfed for 1 week

Shifted to mixed formula feeding

PRENATAL HISTORY

Unremarkable

FAMILY HISTORY

Unremarkable
 REVIEW OF SYSTEMS

No change in activity and sensorium

5-6 diaper changes

(daily to every other day bowel movement)

No passage of stools 2 days PTA

PHYSICAL EXAMINATION

irritable

36.5 °C (afebrile)

134 bpm

57 cpm; 98% O2 saturation

3 kg; 54 cm
ANTHROPOMETRIC MEASUREMENTS

Length and Weight appropriate

for age
PHYSICAL EXAMINATION

HEENT: Head: non-tensed fontanelles

Eyes: anicteric sclera, pinkish palpebral conjunctiva,
briskly reactive pupils, non-sunken eyeballs
Lips and Oral mucosa: pale, moist

HEART: adynamic precordium, no murmurs, full pulses, capillary

refill time of <2 seconds

CHEST: equal chest rise, no retractions, no grunting

 PHYSICAL EXAMINATION

distended, abdominal girth: 34 cm, not shiny,

ABDOMEN normoactive bowel sounds, soft, tympanitic, with
: no palpable mass

EXTREMITIES: full range of motion

ANUS: Patent
 18-day-old male
Irritable

Full term
Pale lips

Decreased milk intake

PROMINENT
FEATURES Poor suck distention
Abdominal
Fever
Projectile, bilious vomiting
No passage of stool for 2 days
HD 1
Leukocyte 2.2 LOW
Neutrophils 46
Lymphocyte 43
Septic
Platelets 729 HIGH
Ileus
CRP 17.54 ELEVATE
D
U/A 4014 BACTEURIA
APPROACH TO DIAGNOSIS

MALE NEONATE with ONSET OF

FUNCTIONAL ABDOMINAL
MECHANICAL
DISTENTION AND PROJECTILE VOMITING

Necrotizing Ileus Malrotation Aganglionosis

Enterocolitis
Differentials

Necrotizing Enterocolitis
• Born term
Most common with weight appropriate
life-threatening for age in the newborn
surgical emergency
Dysbiosis
• Feeding history was unremarkable

• No history of birth asphyxia or hypoxia

3
FACTORS
Injury to the Proinflammatory
intestinal lining immune response
 MALE NEONATE with SUDDEN ONSET OF FEVER,
ABDOMINAL DISTENTION, AND PROJECTILE
VOMITING

FUNCTIONAL MECHANICAL

L E D
Necrotizing Ileus Malrotation Aganglionosis

U
Enterocolitis
R
U T
O
 Differentials

Ileus
Cessation of peristalsis

May be due to infection or side effect

of medication

May become obstructive

CANNOT TOTALLY RULE OUT

MALE NEONATE with SUDDEN ONSET OF FEVER,
ABDOMINAL DISTENTION, AND PROJECTILE
VOMITING

FUNCTIONAL MECHANICAL

Septic Ileus Malrotation Hirschsprung

Disease
Cannot Totally Rule
Out
Differentials

Malrotation
A congenital variation in the rotation and fixation of the GI tract
during development

Bilious and Non-bilious vomiting

Abdominal distension

Pallor
MALE NEONATE with SUDDEN ONSET OF FEVER,
ABDOMINAL DISTENTION, AND PROJECTILE
VOMITING

FUNCTIONAL MECHANICAL

Septic Ileus Malrotation Aganglionosis

Cannot Totally Rule Cannot Totally Rule

Out Out
 Defecated at 15 Hirschprung
Irritability Fever

Disease
hours old

No passage of stool x 2
Bilious vomiting Abdominal distention
days
Most common congenital neurointestinal disease, and 90%
of cases present in the neonatal period
HD 1
HD 2
•Abdominal girth of 36cm (34cm)
•Decreased bowel sounds of 2-3 clicks
Repeatground
•Coffee Abdominal
bilious OGT output
X-ray
01
02
• Ileum was dilated and dusky red
color
Intraoperative Finding • Biopsy
• Cecum wasofdisplaced
Appendix:
ExploratoryTOTAL COLONIC• AGANGLIONOSIS
laparotomy Redundant sigmoid
Absent adherent cells
ganglionic to the
cecal area with fibrous bands
enveloping the cecum
7 TH
POST OP

Trial of
feeding
DISCHARGED
 
 DIAGNOSIS

TOTAL COLONIC AGANGLIONOSIS

Intraoperativewith
Biopsy Finding
Displacement of the cecum and absence of ganglion cells
MALROTATION
and
INTESTINAL BANDS
 TOTAL COLONIC
AGANGLIONOSIS
1 in 500,000 cases

-Catangui (2012)
6,374
2 cases of TCA in Manila Nationwide

1 case of TCA in Mindanao

Hirschsprung Disease and
Malrotation concurrence
is RARE

X
HIRSCHSPRUNG
DISEASE
MANAGEMENT

Goal: to remove the aganglionic segment

Dilemma: gradual removal or one-step surgical

procedure (Hajar and Puri, 2017)
PROGNOSIS

Underdeveloped and Developing Countries:

Very poor prognosis with 50% mortality

Enterostomy alone: 60% mortality

X
Maximum deaths attributed by:
Early surgical
Ileostomyintervention
complications with good
Poor97%
compliance: compliance to follow
survival rateup
Poor familial support
 FINAL DIAGNOSIS
Total Colonic Aganglionosis with
Malrotation and Intestinal Bands