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Case 179

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Full-term male infant, 21 days old, appropriate for gestational age (AGA), previously healthy, exclusively breastfed. Six days ago, he began vomiting in jet, with lacteal content vomitus, just after feedings, which progressed in intensity and frequency, resulting in weight loss. Clinical examination revealed adequate suction, signs of moderate dehydration and distended abdomen in upper quadrants. Abdominal ultrasound was inconclusive, and radiographies were requested (see attachments).

Based on the clinical and radiological findings, which is the most likely diagnosis?

a) Gastroesophageal reflux disease

25%

b) Duodenal atresia

25%

c) Hypertrophic pyloric stenosis

25%

d) Pylorospasm

25%
   

Image Analysis

 

Image 4: Anteroposterior (AP) chest and abdomen radiography revealing gaseous distension of the gastric chamber (yellow circle); radiolucent threadlike elongated image in the topography of the pylorus (red circle), known as string sign; and paucity of gaseous material in the descending portion of the duodenum (blue circles) and remaining in the intestine.

 

Image 5: Anteroposterior (AP) contrast radiological examination of the esophagus, stomach and duodenum showing distended stomach, filled with contrast (red circle). There are limitations to the progression of contrasting material, with reflux of this for the distal esophagus (blue circle).

 

Image 6: In detail, contrast radiological examination of the esophagus, stomach and duodenum in oblique view showing the pyloric antrum region. It is observed thin radiopaque image corresponding to the pyloric canal, narrow and elongated, completed by contrasting substance (string signal, red arrow). Note also the presence of up shoulder sign (blue line), and it refers to the bulging of the hypertrophied pyloric muscle into the lumen of the antrum.

 

Image 7: Illustration showing hypertrophy of the muscle layers with consequent lengthening of the pyloric canal. Available in: Hernanz-Schulman M. Infantile Hypertrophic Pyloric Stenosis. Radiology 2003 May; 227: 319-331

 

Image 8: Ultrasound with pyloric measurements. On the left, there is increased muscle thickness (measured 3, RR

 

Image 9: Contrast radiological examination of the esophagus, stomach and duodenum showing retention over a column of material between the folds of the thickened and redundant mucosa (double-track sign, yellow arrows), convex indentations in the base of the duodenal bulb caused by the hypertrophied layer (mushroom sign, white arrows) and beak sign, produced by similar indentation, but in proximal portion of the muscles over material present in gastric antrum (red arrow). Adapted from: http://www.learningradiology.com/notes/ginotes/hypertrophicstenosispage.htm

 


Image 10: Perioperative photography showing post-pyloromyotomy pyloric region, with hypertrophied muscle layer split lengthwise to expose the mucosa internally. Dr. Attila Courtesy of Magellan, Pediatric Surgeon at the UFMG School of Medicine- Hospital das Clínicas.

Highlights

- The IHPS is presented mainly between 3-5 weeks of life, with non-bilious vomiting in jet, postprandial and progressive.
- Diagnostic confirmation with olive palpation or visualization of gastric peristalsis, predominant in the past, nowadays is less frequent.
- Sonographic measurements may have borderline values, especially at younger ages, and in case of fixed values, it is important to track and monitor the workup.
- Contrast radiological examination of the esophagus, stomach and duodenum diagnostic method is used primarily in cases in which Ultrasound is inconclusive.
- The definitive treatment is surgical, by Fredet-Ramsted pyloromyotomy and has had very low complications.

References

- Junqueira JCF, Tavares AP, Ferreira B. Abdome Agudo. In: Carvalho E, Silva LR, Ferreira CT. Gastroenterologia e nutrição em pediatria. Barueri: Manole; 2012. p. 574-591.
- Colégio Brasileiro de Radiologia, Painel de Especialistas em Imagem Pediátrica e American College of Radiology. Vômito em crianças de até 3 meses de idade.
- Hernanz-Schulman M. Infantile Hypertrophic Pyloric Stenosis. Radiology 2003 Mai; 227:319-331.
- Olivé AP, Endom EE. Infantile hypertrophic pyloric stenosis. UpToDate [Internet] 2014 [acesso em Set 2014]. Disponível em: http://www.uptodate.com/contents/infantile-hypertrophic-pyloric-stenosis
- Figueiredo SS, Junior CRA, Nóbrega BB, Jacob BM, Esteves E, Teixeira KISS. Estenose hipertrófica do piloro: caracterização clínica, radiológica e ecográfica. Radiol Bras 2003; 36(2):111-116.
- Nazer H. Pediatric Hypertrophic Pyloric Stenosis. Medscape [internet] 2014 [acesso em Set 2014]. Disponível em: http://emedicine.medscape.com/refarticle-srch/937263-overview

Authors

André Ribeiro Guimarães, 5th year medical student at UFMG School of Medicine.

Email: guimaraesandrer [at] Hotmail.com
Cassio de Almeida Dias, 5th year medical student at UFMG School of Medicine.
Email: cassio_adias [at] Hotmail.com

Supervisor

Paula Valladares Guerra Resende, Pediatrician, Professor of the Department of Pediatrics at UFMG School of Medicine.
Email: paulavpg [at] gmail.com
Jovita Lane Soares Santos Zanini, Professor of the Department of Anatomy and Image at UFMG School of Medicine.
Email: jovitalane1 [at] yahoo.com.br

Reviewers

Hercules Riani, Marina Bernardes Lion, Ana Luiza Tavares, Barbara Queiroz, Ana Júlia Bicalho and Professor Viviane Parisotto.

Acknowledgements

Marcela Ferreira Nicoliello, Radiology and Diagnostic Imaging Resident at UFMG School of Medicine Hospital.

Translated by

Ana Júlia Furbino Dias Bicalho, 6th year medical student at UFMG School of Medicine.
Email: anajuliabicalho[at]gmail.com

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