Advances in infantile hypertrophic pyloric stenosis

Abstract

Infantile hypertrophic pyloric stenosis (IHPS) is a common condition in infancy, characterized by an acquired narrowing of the pylorus, which requires surgery. These infants usually present with projectile, nonbilious vomiting, with a palpable ‘olive’ in the abdomen and sometimes a ‘peristaltic wave’ after being fed with formula or breast milk. Although IHPS is a common disorder, its etiology is largely unknown. Surgical intervention is the standard treatment, preoperative preparation, however is essential to optimal outcome. In this review, the latest advances in IHPS regarding epidemiology, etiology, diagnostics and treatment will be discussed.

Keywords::

• fluid resuscitation
• infant
• infantile hypertrophic pyloric stenosis
• laparoscopy
• pyloromyotomy

Financial & competing interest disclosure

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript.

No writing assistance was utilized in the production of this manuscript.

Key issues

• In infantile hypertrophic pyloric stenosis (IHPS), a progressive hypertrophy of the pyloric muscle results in a (near-) complete obstruction of the pylorus. IHPS typically occurs between the third and sixth week of life in a formerly healthy infant.

• IHPS is found more common in the Western world compared with other regions. IHPS has a male predominance (male:female ratio of 4–5:1). No clear explanation for this male predominance exists.

• Despite the fact that IHPS is a common disorder, its etiology is largely unknown. IHPS shows a strong familial aggregation, suggesting a genetic background. Next to this, environmental factors are likely to play an important role in its etiology.

• IHPS can be found as an isolated entity but may also be associated with clinical syndromes.

• Up till now, genes found to be associated with IHPS in genetic studies could usually not be confirmed in other study populations. This suggests that IHPS is a heterogenetic disorder.

• Applying next-generation techniques in well-defined homogeneous study populations may be helpful in the future.

• In diagnosing IHPS, imaging by means of ultrasound has the highest sensitivity and specificity.

• The management of pyloric stenosis is surgical but preoperative preparation is essential to optimal outcome.

• Postoperative complications do occur in the surgical treatment of IHPS; major complications as incomplete pyloromyotomy, missed mucosal perforation and severe wound infections require a reoperation.

• There is an initial learning curve in laparoscopic pyloromyotomy.

• There is a tail in the learning curve in laparoscopic pyloromyotomy.

• Because of the paucity in randomized controlled trials regarding which procedure is better, open or laparoscopic pyloromyotomy, and the need for structured surgical training, collaboration between centers is necessary.

Notes