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In Vivo Visualization of Pyloric Mucosal Hypertrophy in Infants with Hypertrophic Pyloric Stenosis

Is There an Etiologic Role?

Marta Hernanz-Schulman1, Lisa H. Lowe1, Joyce Johnson2, Wallace W. Neblett3, D. Brent Polk4, Ramiro Perez, Jr.1, Luis E. Scheker1, Sharon M. Stein1, Richard M. Heller1 and Robert Cywes3

1 Department of Pediatric Radiology, Vanderbilt Children's Hospital, Vanderbilt University Medical Center-D-1120, Medical Center North, 21st Ave. S., Nashville, TN 37232-2675.
2 Department of Pathology, Vanderbilt Children's Hospital, Nashville, TN 37232-2675.
3 Department of Surgery, Vanderbilt Children's Hospital, Nashville, TN 37232-2675.
4 Department of Gastroenterology, Vanderbilt Children's Hospital, Nashville, TN 37232-2675.



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Fig. 1. Schematic representation of transverse diameter of hypertrophied pylorus. Pyloric diameter is defined as diameter of entire mass; it represents additive components of muscle thickness on each side and mucosal thickness in center.

 


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Fig. 2A. Sonographic images of typical hypertrophied pylorus in male infant with hypotrophic pyloric stenosis. Longitudinal sonogram shows two-layered, thickened mucosa (solid arrows) surrounded by muscular components (open arrows). Mucosa protrudes into, and is outlined by, fluid within gastric antrum (A).

 


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Fig. 2B. Sonographic images of typical hypertrophied pylorus in male infant with hypertrophic pyloric stenosis. Transverse sonogram shows redundant, infolded mucosa (solid arrows) between muscular components (open arrows).

 


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Fig. 3A. Histopathologic specimens in infant with hypertrophic pyloric stenosis. Photomicrograph shows mucosal hyperplasia, characterized by elongated, branched, and mildly distorted pits (solid arrow) and abundant lamina propria that is somewhat edematous (open arrow). (H and E, x25)

 


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Fig. 3B. Histopathologic specimens in infant with hypertrophic pyloric stenosis. Photomicrograph shows hyperplastic mucosa with crypt distortion and pit epithelial cell hypertrophy with abundant supranuclear cytoplasm (arrow). (H and E, x62.5)

 


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Fig. 4A. 4-month-old female infant with surgically confirmed infantile hypertrophic pyloric stenosis. Longitudinal sonographic image shows markedly hypertrophied mucosa (solid arrows) measuring 8 mm in thickness. Muscle thickness was 4.5 mm (open arrows). A = antrum.

 


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Fig. 4B. 4-month-old female infant with surgically confirmed infantile hypertrophic pyloric stenosis. Radiograph from upper gastrointestinal fluoroscopy shows increased peristaltic activity of the contrastfilled stomach, and double-track sign (arrows), denoting contrast material coursing around redundant, hypertrophied mucosa. A = antrum.

 


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Fig. 5. Endoscopic image of infant illustrated in Figure 3A,3B shows pyloric mucosa (M) protruding into gastric antrum (A). (Reprinted with permission from [12])

 


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Fig. 6. Photograph of full-thickness biopsy specimen of pyloric muscle (MUS) in previously reported infant with infantile hypertrophic pyloric stenosis. Marked enlargement of mucosa (muc) resembles that in our patient, illustrated in Figure 4A,4B. (Reprinted with permission from [22])

 

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