Congenital isolated asplenia accidentally discovered during acute peritonitis

Elmarouni Abdelouhab; Karam Azız; Et-Tayeb Ouzzanı; Ahmed Zerhounı; Tarik Souıkı; Karim Ibn Majdoub El Hassanı; Khalid Mazaz; Imane Toughraı

doi:10.28982/josam.588198

Authors

Elmarouni Abdelouhab https://orcid.org/0000-0003-2035-7982
Karam Azız https://orcid.org/0000-0002-3330-7311
Et-Tayeb Ouzzanı https://orcid.org/0000-0003-2977-2904
Ahmed Zerhounı https://orcid.org/0000-0001-8441-016X
Tarik Souıkı https://orcid.org/0000-0001-5657-7253
Karim Ibn Majdoub El Hassanı https://orcid.org/0000-0002-0421-7296
Khalid Mazaz https://orcid.org/0000-0001-7779-7802
Imane Toughraı https://orcid.org/0000-0003-0401-3012

DOI:

https://doi.org/10.28982/josam.588198

Keywords:

Congenital isolated asplenia, Adult peritonitis

Abstract

An extremely rare condition, congenital asplenia has 2 distinct types: heterotaxy syndromes and isolated congenital asplenia (ICA). Ivemark syndrome is one of the heterotaxy syndromes characterized by asplenia, malformations of the heart, and malposition of internal organs in the chest and abdomen. ICA cases are also fatal in childhood, but there are reported living adult cases. Those affected are typically at increased risk for fulminant sepsis and carry a higher risk of noninfectious complications, such as thrombocytosis and mesenteric thrombosis. We herein report the unusual case of a patient with congenital asplenia, which was discovered fortuitously during an emergency laparotomy for peritonitis due to ulcer perforation.

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