INFANTILE HYPERTROPHIC PYLORIC STENOSIS

 

Incidence  

2.2 in 1000 live births

Ratio  

4male: 1 female

Associations

Caucasians, born in autumn and spring, bottle fed

Aetiology:  unknown , lack of nitrous oxide secondary to decrease synthesis of nitrous oxide synthease =>failure of relaxation of pylorus =>sustained contraction of sphincter =>hypertrophy of muscle.

? Vasoactive intestinal polypeptide

Pathology  

macro = elongated thickened firm non compressible pylorus

Body and fundus dilated forceful+visible contractions

Micro  hypertrophy of circular muscle layer +attenuated longitudinal muscle

histo marked reduction in neural element in hypertrophied circular muscle

 

Clinical picture

    pmhx unremarkable

Usual presentation  

3-6 weeks   c/o vomiting progressive frequent severe ,Forceful projectile

Desperately Hungary crying for feed, Vomiting after feed consisting of ingested milk

Not bile stained

Dehydration electrolyte imbalance wt loss

smalldry greenous stools starvation stools, urine concentrated 

LUQ +epigastric distension and discomfort

On examination  

(feed +n/g aspirate continuously)

Mobile firm smooth nontender supraumblical right hypochondria mass

palpable mass => diagnosis

Investigation:        

Ultrasound confirms diagnosis

Contrast x-rays

Labs

          increased urea,  PCV

          Decreased  Na  K  Cl  

          Alkalosis and acidic urine

         (Hypochloremic hypokalemic alkalosis)

 Management

N/G asp,

Sham feed, 

Fluids & electrolytes corrections

 

Treatment

ramsted’s pyloromyotomy only acceptable treatment

Balloon dilatation inappropriate ineffective

 

Approach: 

  (midline, paramedin no longer acceptable )

1 transverse RT upper quadrant

2 circum umbilical supra umbilical incision

3 laproscopic pyloromyotomy (complication CO2 pneumoperitoneum)

 

Complications  

Mucosal perforation ,

Wound infection

 Post op

 1 feeding is rapidly started within few hours (as soon as baby starts to cry)

2 Perforation: feed delayed for 3-5 days

Follow-up

Rapid catch-up on growth

Gastro oesophageal reflex & urinary tract abnormality needs treatment

Recurrence does not occur

Symptomatic pyloric sphincter incompetence is uncommon

 

 

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