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John H. Renwick first coined the term “synteny” in 1971, which refers to the genes present on the same chromosomes, even if they are not genetically linked. The species with common ancestry tend to show conserved syntenic regions. Therefore, the concept of synteny is nowadays used to describe the evolutionary relationship between species.
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Related Experiment Video

Updated: Apr 3, 2026

Midface Hypoplasia and Cranial Base Morphology in Syndromic Craniosynostosis: A Comparative Analysis Study Using a Predictive Regression Model
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[Scimitar syndrome. Correlation anatomo-embryological].

Luis Muñoz-Castellanos1, Magdalena Kuri-Nivon2

  • 1Departamento de Embriología, Instituto Nacional de Cardiología Ignacio Chávez, México, D. F., México.

Archivos De Cardiologia De Mexico
|September 16, 2015
PubMed
Summary

Scimitar syndrome involves abnormal right lung development and pulmonary venous connection to the inferior vena cava. This case suggests a developmental persistence of an embryonic venous pathway explains the anomaly.

Keywords:
Anomalous pulmonary venous connection of the right lungCardiopatía congénitaConexión anómala de las venas pulmonares derechasCongenital heart diseaseHipoplasia y displasia del pulmón derechoHypoplasia and dysplasia of the right lungMexicoMéxicoScimitar syndromeSíndrome de la cimitarra

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Area of Science:

  • Cardiovascular Anatomy
  • Thoracic Surgery
  • Developmental Biology

Background:

  • Scimitar syndrome is a rare congenital anomaly characterized by anomalous pulmonary venous return.
  • Understanding its complex morphology and embryological basis is crucial for diagnosis and management.

Purpose of the Study:

  • To provide a detailed morphological description of a toracoabdominal visceral block in a scimitar syndrome case.
  • To propose a pathogenetic theory for the pulmonary venous connection in this syndrome.

Main Methods:

  • Anatomical specimen analysis using the segmental sequential system.
  • Detailed description of cardiac and pulmonary anatomy, including venous connections.
  • Formulation of a pathogenetic hypothesis based on embryological development.

Main Results:

  • The case presented with situs solitus and normal cardiac chamber connections.
  • Right lung hypoplasia, dysplasia, and inferior lobe sequestration were observed.
  • Right pulmonary veins connected via a collector to the suprahepatic inferior vena cava; left veins connected to the left atrium.
  • An atrial septal defect and a collateral aortopulmonary vessel to the sequestered lobe were present.

Conclusions:

  • The proposed pathogenetic theory suggests the pulmonary venous connection in scimitar syndrome results from the persistence of Streeter's horizon XIV.
  • This embryonic period (28-30 days) involves a dual connection of the pulmonary venous sinus.
  • The anomaly involves a primitive collector vein, forming the suprahepatic inferior vena cava segment.