Infantile Hypertrophic Pyloric Stenosis

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A Sonographic walk-through: Infantile Hypertrophic Pyloric Stenosis Tara K. Cielma, RDMS, RDCS, RVT, RT(S) Anjum N. Bandarkar, MD, Adebunmi O. Adeyiga, MD, Diagnostic Imaging and Radiology, Children s National Health System, Washington, D.C.,

Objectives Review sonographic anatomy of normal and abnormal pylorus Illustrate technical approach to pyloric ultrasound Describe tips & tricks to minimize equivocal exams

Introduction Infantile hypertrophic pyloric stenosis (IHPS) is a common condition affecting young infants, in which the antropyloric portion of the stomach becomes abnormally thickened and manifests as obstruction to gastric emptying. Not present at birth, but mechanical obstruction typically develops in the first 2-12 weeks of life. Most common condition requiring surgery in infants. Treatment: Surgical pyloromyotomy. Drawing of pylorus specimen

Background 3 in 1000 incidence M>F 4:1 Familial Disposition, Unknown etiology

Clinical Presentation The infant presents with a recent onset of forceful nonbilious vomiting, typically described as projectile. Initially intermittent, the frequency of emesis increases with time. Vomitus may be stained with blood due to rupture of small capillaries in gastric mucosa. Dehydration and weight loss are often present. Occasionally, indirect hyperbilirubinemia may be seen. Hypochloremic metabolic alkalosis is the characteristic biochemical abnormality because vomiting of gastric contents leads to depletion of sodium, potassium and hydrochloric acid.

Normal Longitudinal view Muscularis mucosae Submucosa Mucosa

Abnormal Longitudinal view Submucosa Mucosa Muscularis mucosae

Abnormal Transverse view Mucosa lumen interface Muscularis mucosa Submucosa Muscularis externa Serosa

Zonal Anatomy of Pylorus Layers from innermost to outermost Mucosa- echogenic Muscularis mucosa- hypoechoic Submucosa- echogenic Muscularis externa- hypoechoic Serosa- echogenic Normal Pylorus Transverse image

Zonal Anatomy of Pylorus Muscularis externa Serosa Submucosa Lumen Mucosa Muscularis mucosa Normal Pylorus Longitudinal image

Zonal Anatomy of Pylorus Muscularis Externa Submucosa- echogenic Mucosa collapsed Abnormal Pylorus Transverse image

Zonal Anatomy of Pylorus Submucosa Lumen Stomach Duodenum Abnormal Pylorus Longitudinal image

Sonographic Technique for Pylorus US High frequency linear transducer must be used for optimal evaluation of pyloric anatomy.

- Supine position, or - Right posterior oblique

Diagnostic Criteria: Normal Normal pyloric muscle: - Measures <3mm thick Normal pyloric channel: - 11 to 14 mm in length

Normal Pylorus US Muscularis externa Submucosa Duodenum Pylorus open Antrum

Diagnostic Criteria: Abnormal Pyloric muscle thickness >3mm Pyloric canal length >15mm

How to measure pylorus muscle: Long axis Incorrect, overmeasured Correctly measured 0.31 cm 0.16 cm

How to measure pylorus muscle: Short axis 0.27 cm

Sonographic Signs of Pyloric Stenosis: Target sign Peripheral ring of hypertrophied hypoechoic muscle surrounding central echogenic mucosa, resembling a doughnut.

Sonographic Signs of Pyloric Stenosis: Antral nipple sign redundant pyloric mucosa protruding into the gastric antrum (orange arrow)

Sonographic Signs of Pyloric Stenosis: Cervix sign - Extension of hypertrophied pyloric muscle into the antrum and elongated pyloric channel form an image that resembles a cervix

Over measuring pyloric muscle thickness- Trans Long First Ultrasound: Pyloric muscle thickness read as 3 mm, and hence equivocal for pyloric stenosis.

Trans Long Repeat Ultrasound (next day): Pyloric muscle thickness read as 1.5 mm and normal. Correct measurement obtained using higher frequency(15mhz) transducer with MSK setting, and excluding the submucosa.

Correctly measured

Stomach gas can obscure the pylorus Patient should be turned to right posterior oblique position to displace stomach gas, and to better visualize the pylorus as fluid distends the gastric antrum.

Posterior displacement of pylorus It is important to know that when the stomach is over distended, the pylorus can flip posteriorly and may be hard to find

Beware of the GE junction! The gastro esophageal junction can be mistaken for pylorus, so remember to look carefully, by ensuring you see the stomach antrum proximally and duodenal bulb distally. GE junction GE junction In this infant, the enteric tube was seen passing through the gastro esophageal junction which was thus distinguished from the pylorus.

Look for hydronehrosis as a potential cause of emesis When the pylorus is normal, remember to scan the kidneys to evaluate for hydronephrosis or ureteropelvic junction obstruction, where the renal pelvis causes mass effect on the pylorus. Pylorus Right Renal pelvis

If pylorus is normal, evaluate the midline vessels When the pylorus is normal, and there is strong history of emesis, remember to evaluate the midline mesenteric vessels carefully to look for whirlpool sign. In this infant, there was abnormal swirling of midline mesenteric vessels concerning for malrotation & volvulus!

Take Home Points: 1. Recognize muscularis externa as the outermost hypoechoic layer that should be measured for pyloric muscle thickness. 2. Differentiate this from submucosa which is the thick echogenic layer deep to muscularis externa. Submucosa should be excluded while measuring muscle thickness. 3. Most textbooks recommend measuring the pylorus in transverse section, but in our experience, measurements are easier and more accurate in longitudinal sections, so try both.

Thank You Author info: tcielma@childrensnational.org anjumnb@gmail.com