SONOWORLD : Congenital hypertrophic pyloric stenosis

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Abdomen » Gastrointestinal

Congenital hypertrophic pyloric stenosis

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Author(s) :

P.K. Srivastava, MD

Presentation A one month old male child presents with projectile vomitting after feeds. An abdominal ultrasound was performed.

Caption: Sagittal image of the epigastric region

Description: The pyloric channel appears significantly elongated and the thickened, hypoechoic muscular layers are well appreciated. The pyloric lumen is reduced and the apposed echogenic mucosal layers of both sides are noted.

Caption: Transverse image of the pyloric region

Description: The wall of the pylorus is thickened, measuring 6.5 mm. This image demonstrates the typical target sign.

Differential Diagnosis Congenital hypertrophic pyloric stenosis, pylorospasm

Final Diagnosis Congenital hypertrophic pyloric stenosis

Discussion Infantile or congenital hypertrophic pyloric stenosis is one of the most common surgical causes of vomiting in infancy. The infant may present with failure to retain feeds, persistent non-bilious vomiting after feeds, a palpable epigastric mass [which is the thickened pylorus] and dehydration [hypochloremic metabolic alkalosis due to loss of acid in the vomitus].

The circular muscle hypertrophies and the thickened muscle reduces the lumen of the pyloric channel and also elongates it. The peristaltic wave fails to pass through the narrowed pyloric channel, resulting in gastric outlet obstruction, gastric distention and possibly retrograde peristalsis.

Imaging: Ultrasound is currently the imaging modality of choice that reliably establishes the diagnosis of hypertrophic pyloric stenosis. There are various sonographic parameters that can be used to arrive at the diagnosis and include pyloric length, pyloric diameter, muscle thickness and also pyloric volume.
Pyloric muscle thickness of 3 mm or greater, pyloric canal length of 17 mm or greater and the absence of the passage of a peristaltic wave through the pylorus, during the period of scanning are the diagnostic ultrasound criteria. Classically seen is:
1. A ring on transverse section, resembling a ‘doughnut’ or a ‘bulls-eye’ or a ‘target’ with the echogenic pyloric canal in the center and surrounded by the hypertrophied pyloric muscle.
2. An elongated and narrowed pyloric channel.
3. Prolapse of the redundant mucosa into the antrum creates an ‘antral nipple sign’.
4. In many cases, an elongated pylorus that lies adjacent to and just below the gallbladder provides the initial clue to the diagnosis.
5. Other features that may be seen are retrograde or hyper-peristaltic contractions, though none of them pass through the pylorus.

The only other imaging studies that may be useful for diagnosis of this condition are a plain radiograph and upper GI studies with barium. The latter demonstrates the narrowed pyloric channel as a thin stream of barium passes through it [the ‘string sign’]. CT and MRI are not performed to diagnosis this condition. Surgery [pyloromyotomy] is the treatment of choice in these patients.

Case References 1. Ito S, Tamura K, et al. Ultrasonographic diagnosis criteria using scoring for hypertrophic pyloric stenosis. J Pediatr Surg. 2000 Dec; 35(12):1714-8.
2. Levine D, Wilkes DC, Filly RA. Pylorus subjacent to the gallbladder: an additional finding in hypertrophic pyloric stenosis. J Clin Ultrasound. 1995 Sep; 23(7):425-8.
3. Reid Janet. Hypertrophic Pyloric Stenosis. http://www.emedicine.com/radio/topic358.htm#section~ultrasound

Follow Up This child was surgically treated and is doing well.