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    Case of the Day
    Hypertrophic pylorus stenosis

    Modality: Ultrasound Modality: Ultrasound

    View here all images of this case in different sizes !



    Go to the top of the page ID: 20021003170616
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     Hypertrophic pylorus stenosis   
     Available images: There are Ultrasound images available for this case. [ Ultrasound ]   
     Author/s:

    Carsten Bock, C. Kunze (Halle)  

     
     Email Address:

    Viewable for logged on visitors (Log on)  

     
     Age:

    8 Weeks  

     
     Gender:

    Female  

     
     History:

    8 week old girl, vomiting, starting in the 6th week of life. Spewing and exhausted vomiting (not bilious) almost after every meal.  

     
     Pathomorphology or Pathophysiology of this disease :

    Non-innate hypertrophy of the sphincter muscle (longitudinal muscle is practically not afflicted), which bulges into the antrum. Pathologically, if the diameter of the pylorus is over 14 mm as well as a length of 16mm (where the length is not the only thing that is important), a pyloric stenosis is noted. The thickness of the muscularis propria must be surpass 4mm in an 8 week old baby; in younger children (4 weeks), the limit of 3 mm is set.

    The illness afflicts, according to literature, one in 500 babies (in our observation, this number is high) and is often familial (6.9% postive family histories, concordance in identical twins), the ratio of boys to girls is 4:1.

    The pathogenesis is unclear. An underdeveloped plexus myentericus, hypoganglionoses as well as a problem in NO-synthetase have been discussed as causes.

    The diagnosis is made, aside from the clinical symptoms, by ultrasound. Depiction of the pylorus horizontally and longitudinally is obligatory (using at least a 7.5 MHz transponder). Next to the above-mentioned criteria, a longer examination time is important, so that one can document the insufficient passage of fluid and air through the pylorus. Noted as indirect signs are: full stomach for more than 2 hours after meals with increased peristalsis (in restless children, hard to differentiate).

    An x-ray examination is seen as obsolete today.  

     
     Radiological findings:


    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Pylorus transversal, thickened muscularis (5-6 mm). The ring of muscularis does not frequently appear (like also in this picture) circular and of low echo density.


    Ultrasound 2 <- view Ultrasound 2

    Ultrasound 2: Pylorus longitudinal. Next to the thickened ring of musculature, the well recognizable "Cervix sign" (protrusion into the antrum) as well as the wall stratification (from within going outward) : hyperechoic layer: initial echo of the mucosal membrane.
    hypoechoic layer: Lamina propria and Muscularis mucosae.
    hyperechoic layer: boundary to the Muscularis propria
    hypoechoic layer: Muscularis propria.

     

     
     Diagnosis confirmation:

    Surgery / Histo  

     
     Which DD would be also possible with the radiological findings:

    N/A  

     
     Course / Prognosis / Frequency / Other :

    Since 1912, the pyloromyotomy (Weber-Ramstedt, "extramucosal splitting"): Longitudinal splitting of the muscle with preservation of the mucosal lining, is still done today. The complication rate is small when correctly operated on and the prognosis is good. Symptoms disappear usually right away. Conservative treatment methods are only indicated in light cases without weight loss. Treatment includes smaller meals with concurring spasmolytics.  

     
     Comments of the author about the case:

    N/A  

     
     First description / History:

    Hypertrophic pyloric stenosis was first described in 1887 by Hirschprung and was operted on in 1907 for the first time. Since 1912, the pyloromyotomy (Weber-Ramstedt, "extramucosal splitting"): Longitudinal splitting of the muscle with preservation of the mucosal lining, is still done today.  

     
     Literature:

    N/A  

     
     Region-Organ:

    GI-Stomach  

     
     Most likely etiology:

    unknown  

     
     Available images: There are Ultrasound images available for this case. [ Ultrasound ]   
    More cases from these authors: Search Carsten Bock in Medline Carsten Bock (9)   Search Carsten Bock in Medline C. Kunze (3)     




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