THE CASE

ID: 44 day old term infant directly admitted to the floor from OSH ED.
CC: Vomiting
HPI: Mother reports that infant was feeding well until about 2 weeks
of life when he started vomiting. At first, this was intermittent, but now
infant vomits after every feed. NBNB. Emesis looks like formula and
seems to be the entire volume of feed, but non projectile. About 5 wet
diapers per day. Hard stools every other day. Wakes up to feed
easily and continues to want to feed after vomiting. No coughing or
sputtering. No increased fussiness.
At OSH, infant was noted to be cachectic, but alert and well hydrated.
Started on maintenance fluids of D5 1/2NS and transferred to PCH.
THE CASE
PMedHx: Term infant. No complications of pregnancy or birth.
Unclear history of newborn screening.
Immunizations: Hep B likely received.
Family Hx: No metabolic disorders or infants with FTT.
Social Hx: Lives with mother, father, and 3 healthy siblings.
Family works as migrant workers and infant was born in
Tennessee. Parents report that they had visited a pediatrician in
Salt Lake City 2-3 weeks prior to presentation and were told that
vomiting is normal.
THE CASE
VITALS: Temp 36.7, HR 120, RR 30, BP 84/55, O2 sat 99%
WEIGHT: 2.57 kg which is 89% of BW
General: Cachectic, NAD
HEAD: NC/AT, anterior fontanelle open, soft, depressed
EYES: Red reflex present, open spontaneously
NOSE: No discharge or obstruction.
OROPHARYNX: MMM. pallet intact.
CV: RRR. No murmur. Femoral pulses 2+. Cap refill 2 seconds
LUNGS: CTAB. No increased WOB.
ABDOMEN: Soft, NTND, no mass, sunken
EXTREMITIES: No clubbing, cyanosis, or edema. Thin and boney.
NEUROLOGICAL: Normal tone, movements, level of alertness.
SKIN: No rash. Loose.

DIFFERENTIAL DIAGNOSIS
44 day old term infant with vomiting and FTT.
DIFFERENTIAL DIAGNOSIS
VOMITING
NEONATE
Physiologic reflux/GERD
Pyloric stenosis
Necrotizing enterocolitis
Malrotation w/ midgut volvulus
Gastroenteritis
Hirschsprung disease
Congenital atresia
Congenital stenosis
Congenital webs
Metabolic disorders
Adrenal crisis
Dietary protein intolerance
Overfeeding

INFANT
Physiologic reflux/GERD
Gastroenteritis
Intussusception
Malrotation w/ midgut volvulus
Pyloric stenosis
Hirschsprung disease
UTI
Toxic ingestion
Adrenal crisis
Increased ICP
Pancreatitis
Dietary protein intolerance
Overfeeding
CHILD/ADOLESCENT
Gastroenteritis
UTI
Strep pharyngitis
Appendicitis
DKA
Toxic ingestion
Peptic ulcer
Eosinophilic esophagitis
Cyclic vomiting
Psychogenic
Increased ICP
Adrenal crisis
Obstruction
Constipation
Pancreatitis
Bezoar
Celiac disease
IBD



WORK UP
LABS:
135
2.2
78
44
31
0.47
91
Ca 11.1
iCal 1.19
Phos 4.7
Mag 2.0

Protein 6.5
Albumin 3.9
Tbili 1.6
AP 228
ALT 37
AST 45
CBG: pH 7.56 / Co2 45 / Bicarb 42
WORK UP
LABS:
135
2.2
78
44
31
0.47
91
Ca 11.1
iCal 1.19
Phos 4.7
Mag 2.0

Protein 6.5
Albumin 3.9
Tbili 1.6
AP 228
ALT 37
AST 45
CBG: pH 7.56 / Co2 45 / Bicarb 42
Hypochloremic
metabolic
alkalosis
WORK UP
LABS:
135
2.2
78
44
31
0.47
91
Ca 11.1
iCal 1.19
Phos 4.7
Mag 2.0

Protein 6.5
Albumin 3.9
Tbili 1.6
AP 228
ALT 37
AST 45
CBG: pH 7.56 / Co2 45 / Bicarb 42
Hypochloremic
metabolic
alkalosis
ABDOMINAL ULTRASOUND: The pylorus is enlarged. Pyloric channel
length 1.9 cm, with single layer muscular thickness 0.7 cm.



WORK UP
LABS:
135
2.2
78
44
31
0.47
91
Ca 11.1
iCal 1.19
Phos 4.7
Mag 2.0

Protein 6.5
Albumin 3.9
Tbili 1.6
AP 228
ALT 37
AST 45
CBG: pH 7.56 / Co2 45 / Bicarb 42
Hypochloremic
metabolic
alkalosis
ABDOMINAL ULTRASOUND: The pylorus is enlarged. Pyloric channel
length 1.9 cm, with single layer muscular thickness 0.7 cm.

DIAGNOSIS: Pyloric stenosis

WORK UP
WORK UP
TREATMENT
HYDRATION AND RESOLUTION OF ALKALOSIS: 20 ml/kg NS then D5NS+20
meq/L KCl at 1.5 maintenance rate. IV replacement potassium. NPO.

PYLOROMYOTOMY: incision through the longitudinal and circular muscles of the
pylorus until mucosa bulges through incision.

OP NOTE: The duodenum adjacent to the pylorus was grasped providing
stabilization of the pylorus. The pylorus was then incised in a longitudinal fashion
with an arthrotomy knife. The muscular fibers were then spread. A complete
pyloromyotomy was performed as demonstrated by independent movements of
the 2 halves of the pylorus. Careful inspection showed no evidence of
perforation.



TREATMENT
NUTRITION: Feeds started at 15 ml q 3 hours. Increased by 6 ml per
feed every 6 hours to goal of 50 ml q 3 hrs.

TREATMENT
NUTRITION: Feeds started at 15 ml q 3 hours and held at this rate for 24
hours. Increased by 6 ml per feed every 6 hours to goal of 50 ml q 3
hrs.

MONITORING FOR REFEEDING: RFP and Mg levels q 6 hours during the
first 24 hours. Consider EKG monitoring. Watch for hypophosphatemia,
hypokalemia, hypomagnesemia, signs of Wernicke encephalopathy 2/2
thiamine deficiency.

PREVENTION OF REFEEDING: Slow introduction of feeds.
Recommendations vary: 10 kcal/kg/day or 25% of daily calorie goal on
day one. Pretreatment with thiamine.


PYLORIC STENOSIS
EPIDEMIOLOGY:
2-3/1000 live births
Males>females
Symptoms begin around 3-5 weeks of life, rare after 12 weeks
ETIOLOGY:
Unclear
Likely a combination of genetic and environmental factors
Macrolide antibiotics?
Formula feeding?



PYLORIC STENOSIS
CLINICAL PRESENTATION:
Vomiting in a 3-6 week old infant
Age at presentation has changed (mean 5.4 weeks in 1975 and 3.4 weeks in
1995)
Emesis is immediately post prandial and may be projectile
Infant typically desires to feed soon after vomiting, happy spitters
Emaciated, dehydrated, with palpable olive sized mass in the RUQ at lateral
aspect of rectus abdominus with possible peristaltic waves



PYLORIC STENOSIS
LABORTATORY FINDINGS:
Hypochloremic metabolic alkalosis
Hypokalemia
Elevation of BUN and creatinine
CBC normal
Elevation of unconjugated bilirubin possible, icteropyloric syndrome

ULTRASOUND FINDINGS:
Increased pyloric muscle length, diameter and thickness
95% sensitive and specific if done by experienced examiner

PYLORIC STENOSIS
TREATMENT:
Rehydration
Pyloromyotomy
Reimplementation of nutrition

PROGNOSIS:
A review of cases from the Royal Hospital for Sick Children in Glasgow, Scotland
showed a decrease in mortality of IHPS from 59 percent in 1925 to 0 percent in
1975, as a result of surgical intervention
Mucosal perforations occur in about 1 percent of patients and are readily
recognized intraoperatively
Wound complications occur in about 3 percent of patients treated with a
laparoscopic approach and 7 percent of those undergoing open pyloromyotomy.
The pyloromyotomy is incomplete in 2 to 6 percent of patients treated with a
laparoscopic approach and is rare in those undergoing the open procedure.

RESOURCES
Parashtte KR. Vomiting. Peds in Review 2013; 34307-319.
Olive, A, et al. Infantile hypertrophic pyloric stenosis. Up to date. Accessed
8/2014.