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Infantile Hypertrophic Pyloric Stenosis¹

¹ From the Department of Radiology and Radiological Sciences, Vanderbilt University Medical Center, MCN D-1120, 21st Ave and Garland St, Nashville, TN 37232. Received August 6, 2001; revision requested September 25; revision received February 22, 2002; accepted March 14. Address correspondence to the author (e-mail: marta.schulman@vanderbilt.edu).

View larger version (135K): [in a new window]   Figure 1. Harald Hirschsprung (1830-1916). Dr Hirschsprung’s postmortem description of two cases led to the recognition of IHPS by the scientific community and ushered in the interest and research leading to our modern understanding and treatment of the condition. (Reprinted, with permission, from reference 2.)

View larger version (48K): [in a new window]   Figure 2a. (a) Schematic of gastric segmental anatomy. (Reprinted, with permission, from reference 9.) (b) Endoscopic photograph of open pyloric sphincter. Arrow = open canal. (Reprinted, with permission, from reference 10.)

View larger version (153K): [in a new window]   Figure 2b. (a) Schematic of gastric segmental anatomy. (Reprinted, with permission, from reference 9.) (b) Endoscopic photograph of open pyloric sphincter. Arrow = open canal. (Reprinted, with permission, from reference 10.)

View larger version (108K): [in a new window]   Figure 3a. (a) Illustration of pyloric antrum in IHPS. Note circumferentially thickened muscle (arrows). The lumen is shown as a narrowed canal, but the mucosa, which fills and obstructs the lumen, is not illustrated. (Reprinted, with permission, from reference 11.) (b) Close-up view of hypertrophied pylorus from specimen of infant dying of IHPS. Abnormally thickened antropyloric muscle stops abruptly at the duodenal cap (D) and antrum (A). Note thickened mucosa within opened canal lumen curving from the antrum into the pyloric channel (curved arrows) deep to muscle (M). (Reprinted, with permission, from reference 14.) (c) Endoscopic photograph of pyloric sphincter in a patient with IHPS. Note mucosa (M) protruding into normal antrum, occluding and obstructing the pyloric orifice. Compare with Figure 2b. (Reprinted, with permission, from reference 16.)

View larger version (130K): [in a new window]   Figure 3b. (a) Illustration of pyloric antrum in IHPS. Note circumferentially thickened muscle (arrows). The lumen is shown as a narrowed canal, but the mucosa, which fills and obstructs the lumen, is not illustrated. (Reprinted, with permission, from reference 11.) (b) Close-up view of hypertrophied pylorus from specimen of infant dying of IHPS. Abnormally thickened antropyloric muscle stops abruptly at the duodenal cap (D) and antrum (A). Note thickened mucosa within opened canal lumen curving from the antrum into the pyloric channel (curved arrows) deep to muscle (M). (Reprinted, with permission, from reference 14.) (c) Endoscopic photograph of pyloric sphincter in a patient with IHPS. Note mucosa (M) protruding into normal antrum, occluding and obstructing the pyloric orifice. Compare with Figure 2b. (Reprinted, with permission, from reference 16.)

View larger version (110K): [in a new window]   Figure 3c. (a) Illustration of pyloric antrum in IHPS. Note circumferentially thickened muscle (arrows). The lumen is shown as a narrowed canal, but the mucosa, which fills and obstructs the lumen, is not illustrated. (Reprinted, with permission, from reference 11.) (b) Close-up view of hypertrophied pylorus from specimen of infant dying of IHPS. Abnormally thickened antropyloric muscle stops abruptly at the duodenal cap (D) and antrum (A). Note thickened mucosa within opened canal lumen curving from the antrum into the pyloric channel (curved arrows) deep to muscle (M). (Reprinted, with permission, from reference 14.) (c) Endoscopic photograph of pyloric sphincter in a patient with IHPS. Note mucosa (M) protruding into normal antrum, occluding and obstructing the pyloric orifice. Compare with Figure 2b. (Reprinted, with permission, from reference 16.)

View larger version (131K): [in a new window]   Figure 4. Infant with IHPS. Note protruding rib cage and scaphoid abdomen through which the distended stomach and prominent peristaltic activity can be seen. (Reprinted, with permission, from reference 18.)

View larger version (124K): [in a new window]   Figure 5a. (a) Photomicrographs show immunocytochemical localization of D7, a marker for peripheral Schwann cells, in pyloric muscle of a healthy patient (left) and of a patient with IHPS (right). Staining of myenteric plexus (large arrow) is similar in both; however, there is a striking reduction in the intramuscular nerve fibers (small arrows) in the patient with IHPS. cm = circular muscle layer, lm = longitudinal muscle layer. (Peroxidase technique; original magnification, x100.) (Reprinted, with permission, from reference 21.) (b) Photomicrographs show immunohistochemical peroxidase staining for platelet-derived growth factor receptor in a healthy patient (left) and in a patient with IHPS (right). The muscle of the healthy patient shows no evidence of immunoreactivity, whereas there is abundant staining in the muscle of the patient with IHPS. (Original magnification, x400.) (Reprinted, with permission, from reference 1.)

View larger version (68K): [in a new window]   Figure 5b. (a) Photomicrographs show immunocytochemical localization of D7, a marker for peripheral Schwann cells, in pyloric muscle of a healthy patient (left) and of a patient with IHPS (right). Staining of myenteric plexus (large arrow) is similar in both; however, there is a striking reduction in the intramuscular nerve fibers (small arrows) in the patient with IHPS. cm = circular muscle layer, lm = longitudinal muscle layer. (Peroxidase technique; original magnification, x100.) (Reprinted, with permission, from reference 21.) (b) Photomicrographs show immunohistochemical peroxidase staining for platelet-derived growth factor receptor in a healthy patient (left) and in a patient with IHPS (right). The muscle of the healthy patient shows no evidence of immunoreactivity, whereas there is abundant staining in the muscle of the patient with IHPS. (Original magnification, x400.) (Reprinted, with permission, from reference 1.)

View larger version (132K): [in a new window]   Figure 6. Longitudinal sonogram of the normal stomach, pyloric ring (cursors), and duodenum outlining the open pyloric ring in an infant without IHPS. The distance between cursors is 3.1 mm.

View larger version (164K): [in a new window]   Figure 7. Imaging-histopathologic correlation of exaggerated mucosal thickening that occurs in some infants with IHPS. Top left: Image from upper gastrointestinal tract examination (UGI) shows a markedly widened pyloric channel with intervening mucosal filling defect (arrows). Top right: Sonogram in same patient shows hypertrophied mucosa (straight arrows) measuring approximately 8 mm protruding into the gastric antrum (curved arrow). Arrowheads = thickened pyloric muscle. (Reprinted, with permission, from reference 15.) Bottom: Histopathologic full-thickness biopsy specimen from another patient shows thickened, hypertrophied, and edematous mucosa (muc) and its relationship to the underlying hypertrophied musculature (MUS). (Reprinted, with permission, from reference 31.)

View larger version (156K): [in a new window]   Figure 8. Fluoroscopic image from UGI in a patient with IHPS. Contrast material courses through the mucosal interstices of the canal, forming the double-track sign (large arrowheads), with an additional central channel along the distal portion (small arrowhead). Mass impression on the gastric antrum (arrow), best seen during peristaltic activity, is termed the shoulder sign.

View larger version (135K): [in a new window]   Figure 9. Sonograms demonstrate transducer sweep from esophagus to pylorus in a patient with IHPS. Left: Transducer is placed transversely, below the xiphoid, allowing identification of the esophagus (E) anterior to the aorta (A) at the gastroesophageal junction. Middle: Transducer is swept in caudal direction to outline a distended, albeit gas-filled, stomach (S). Right: Gastroduodenal junction is shown bridged by the hypertrophied pylorus, with fluid-filled duodenal cap (arrow). In this case, the pyloric mucosa is of the same echogenicity as gastric contents, which could lead to a false impression of unimpeded gastric emptying. A = antrum.

View larger version (132K): [in a new window]   Figure 10. Sonograms demonstrate transducer sweep from esophagus to pylorus in a patient without IHPS. Left: The esophagus (E) is seen anterior to the aorta (A), allowing identification of the caudal gas-filled viscus to the left as the stomach (S). Right: Normal empty antrum (A) is seen adjacent to the duodenal cap (D).

View larger version (124K): [in a new window]   Figure 11a. Sonograms in a patient with IHPS. (a) Longitudinal sonogram shows anterior thickened muscle (cursors). Double layer of crowded and redundant mucosa fills the channel and protrudes into fluid-filled antrum (arrow). D = fluid-filled duodenal cap. (b) Cross-sectional sonogram shows circumferential muscular thickening (cursors) surrounding the central channel and filled with mucosa (M).

View larger version (129K): [in a new window]   Figure 11b. Sonograms in a patient with IHPS. (a) Longitudinal sonogram shows anterior thickened muscle (cursors). Double layer of crowded and redundant mucosa fills the channel and protrudes into fluid-filled antrum (arrow). D = fluid-filled duodenal cap. (b) Cross-sectional sonogram shows circumferential muscular thickening (cursors) surrounding the central channel and filled with mucosa (M).

View larger version (175K): [in a new window]   Figure 12. Longitudinal sonograms obtained within a few minutes of each other show peristaltic activity and changes in pyloric anatomy in a patient with IHPS. Note the shorter canal in image on left and subsequent elongation coincident with peristaltic activity in image on right. There is failure of relaxation of the pyloric channel, and persistent obliteration of the lumen. Also note that on the left image, the gastric contents and pyloric mucosa have similar echogenicity, falsely suggestive of unimpeded passage of gastric contents through the canal. A = antrum, D = duodenal cap, arrows = outer muscular layer.

View larger version (87K): [in a new window]   Figure 13. Longitudinal sonograms of hypertrophied pylorus in a patient with IHPS. Left: On image obtained off center, the maximum width of the canal lumen and intervening mucosa (arrowheads) cannot be identified. Right: Image obtained through the central portion of the canal outlines full diameter of channel (arrowheads).

View larger version (71K): [in a new window]   Figure 14. Normal correlative anatomy. Sonogram (left), illustration (middle), and fluoroscopic image from UGI (right) show the normal pyloric ring (arrows) and the proximity of the duodenal cap (D) to the relaxed antropyloric portion of the stomach, bridged by the pyloric ring. See also Figure 2a. A = antrum. (Middle image adapted and reprinted, with permission, from reference 52.)

View larger version (134K): [in a new window]   Figure 15. Peristaltic changes in antropyloric anatomy on normal sonographic study. Left: Antropyloric channel is closed during peristaltic activity. Right: Distal antrum (A) is fully relaxed. Arrows = pyloric ring, D = duodenal cap.

View larger version (100K): [in a new window]   Figure 16. Sonogram of normal pylorus before (left) and after (right) ingestion of a small amount of fluid. Left: The stomach is empty. Note that the pyloric channel and stomach have a similar appearance and the anatomy of the pylorus is difficult to delineate. Right: The infant has been given a small amount of fluid to fill the stomach. Prepyloric antrum (A) distends normally and is immediately adjacent to the duodenal cap. D = duodenum.

View larger version (150K): [in a new window]   Figure 17a. Preampullary duodenal stenosis. (a) Sonogram in vomiting infant demonstrates a distensible antropyloric canal (A). However, there is a consistent gas shadow to the right of the pyloric ring, resembling a very dilated duodenal cap (D). This led to suspicion of preampullary duodenal stenosis and referral for UGI. (b) Initial UGI image of stomach shows gas-filled dilated duodenum (D). (c) UGI image obtained with patient prone shows normal pylorus (arrow) and dilated proximal duodenum (D).

View larger version (144K): [in a new window]   Figure 17b. Preampullary duodenal stenosis. (a) Sonogram in vomiting infant demonstrates a distensible antropyloric canal (A). However, there is a consistent gas shadow to the right of the pyloric ring, resembling a very dilated duodenal cap (D). This led to suspicion of preampullary duodenal stenosis and referral for UGI. (b) Initial UGI image of stomach shows gas-filled dilated duodenum (D). (c) UGI image obtained with patient prone shows normal pylorus (arrow) and dilated proximal duodenum (D).

View larger version (138K): [in a new window]   Figure 17c. Preampullary duodenal stenosis. (a) Sonogram in vomiting infant demonstrates a distensible antropyloric canal (A). However, there is a consistent gas shadow to the right of the pyloric ring, resembling a very dilated duodenal cap (D). This led to suspicion of preampullary duodenal stenosis and referral for UGI. (b) Initial UGI image of stomach shows gas-filled dilated duodenum (D). (c) UGI image obtained with patient prone shows normal pylorus (arrow) and dilated proximal duodenum (D).

View larger version (154K): [in a new window]   Figure 18. Surgical photograph of Ramstedt pyloromyotomy. Thickened pyloric muscle has been divided, and forceps outlines the internal bulging mucosal lining. (Photograph courtesy of R. Cywes, MD.)