When approaching a case with a situs abnormality, using the proper terminology, making a specific diagnosis, and understanding the other often associated abnormalities that need to be excluded are of great importance. We present a case of situs ambiguous in the presence of intestinal nonrotation and an obstructing duodenal web. Our patient initially presented at two days old with bilious emesis and failure to pass meconium after birth. An abdominal radiograph demonstrated an unusual bowel gas pattern, a reversed "double bubble" sign. A subsequent thorough imaging survey was crucial to further characterize our patient's unique anatomy. Overall, our case demonstrates many of the unusual plain radiographic and sonographic findings associated with our patient's situs abnormality and allows for review of situs abnormalities and their significance.
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http://dx.doi.org/10.3941/jrcr.v9i2.2358 | DOI Listing |
World J Gastrointest Surg
November 2024
Department of Gastroenterology, The Second Affiliated Hospital of Kunming Medical University, Kunming 650101, Yunnan Province, China.
Background: Asplenia-type heterotaxy syndrome (HS) is rare and refers to visceral malposition and dysmorphism. It is associated with a high infant mortality rate due to cardiac anomalies, and related digestive endoscopic interventions are poorly understood. With the improved long-term prognosis of these individuals after modern cardiac surgery, intra-abdominal anomalies have become increasingly significant.
View Article and Find Full Text PDFRadiol Case Rep
January 2025
Radiology Department, Pediatric Teaching Hospital, Mohammed V University, Rabat, Morocco.
The Polysplenia Syndrome (PSS) is a form of heterotaxy, a rare congenital anomaly with an estimated incidence of 1 in 250,000 live births, first described by Helwig in 1929. Most patients with polysplenia syndrome die during the neonatal period due to severe associated cardiac and biliary anomalies. Nevertheless, some individuals present with moderate cardiovascular malformations or abdominal anomalies, often discovered incidentally in adulthood.
View Article and Find Full Text PDFAust Vet J
November 2024
Centre Hospitalier Vétérinaire ADVETIA, 9 avenue Louis Bréguet 78140, Vélizy-Villacoublay, France.
A 3-year-old crossbreed dog (case 1) and a 3-month-old German Shorthaired Pointer (case 2) were presented for acute signs of encephalopathy. A portosystemic shunt (PSS) was suspected based on clinical context and laboratory exam results and was confirmed on computed tomography (CT) angiography in both cases. A left-sided azygos (case 1) and right-sided azygos (case 2) continuation of an interrupted caudal vena cava (CVC) and a situs ambiguous (SA) were also observed and considered as incidental findings.
View Article and Find Full Text PDFAdv Exp Med Biol
June 2024
Cardiovascular Genetics, Charité-Universitätsmedizin Berlin, Berlin, Germany.
Situs abnormalities may occur in many and most often more complex congenital cardiac malformations. These conditions are collectively referred to as heterotaxy syndromes, derived from the Greek words "heteros" meaning different and "taxos" meaning orientation or arrangement. Clinically, heterotaxy spectrum encompasses defects in the left-right laterality and arrangement of visceral organs.
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