Juxtarenal aortic occlusion with situs inversus totalis: Surgical management of a rare association

Klippel-Feil syndrome (KFS) is a rare congenital anomaly in forming the cervical vertebrae resulting in the fusion of two or more of the vertebrae. KFS is associated with many congenital anomalies, some of which are common and well known. Here, we report a child with an extremely rare association of KFS with situs inversus totalis (SIT). Both KFS and SIT are genetically heterogeneous and their co-occurrence suggests a high possibility of sharing the same underlying causative agent. Here, we review the genetic background that is known for these two conditions in the literature.

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Stomach (Gastrointestinal Stromal Tumor)GIST in a Patient of Situs Inversus Totalis: A Rare Association and Brief Case Report

Journal of Clinical Epigenetics ◽

10.21767/2472-1158.100076 ◽

2017 ◽

Vol 03 (04) ◽

Cited By ~ 1

Author(s):

RA Hvastunov ◽

VV Ermilov ◽

Agarwal A ◽

Mouhammed HA ◽

YC Yang

Keyword(s):

Case Report ◽

Gastrointestinal Stromal Tumor ◽

Situs Inversus ◽

Situs Inversus Totalis ◽

Stromal Tumor ◽

Rare Association

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Malignant Duodenal GIST in a Patient with Situs Inversus Totalis—a Rare Association and Brief Review of Literature

Journal of Gastrointestinal Cancer ◽

10.1007/s12029-017-9974-1 ◽

2017 ◽

Vol 50 (1) ◽

pp. 109-112

Author(s):

Manoj Thillai ◽

Naveen Alexander ◽

Surendran Paramasivam ◽

Arun Ezhil ◽

Niranjan Raj ◽

...

Keyword(s):

Situs Inversus ◽

Situs Inversus Totalis ◽

Review Of Literature ◽

Rare Association ◽

Duodenal Gist

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Spinal dysraphism, club feet, and dextrocardia with situs inversus totalis in a neonate: a rare association and review

Case Reports in Perinatal Medicine ◽

10.1515/crpm-2020-0061 ◽

2020 ◽

Vol 9 (1) ◽

Author(s):

Surasak Puvabanditsin ◽

Akreeti Maskey ◽

Rannan Kased ◽

Sadia Haleem ◽

Rajeev Mehta

Keyword(s):

Situs Inversus ◽

Molecular Basis ◽

Spinal Dysraphism ◽

Situs Inversus Totalis ◽

Chromosome 6 ◽

Case Presentation ◽

Snp Microarray ◽

Rare Association ◽

Common Molecular Basis ◽

Field Defects

AbstractObjectivesThe spinal dysraphism and situs inversus are a rare association. Since 1909, reports on the coincidence of malformations of the spine and gastrointestinal tract have been published. So far there is no plausible explanation for the association.Case presentationWe report a term female infant with spinal dysraphism with club feet associated with dextrocardia and situs inversus totalis. Whole genome SNP microarray analysis was normal. However, there are extended contiguous regions of allele homozygosity [>8 Mb[megabase]) observed in chromosome 6 and 14.ConclusionsWe report a rare association of spinal dysraphism and situs inversus totalis in a neonate. We review the literature. There have recently been theorized by some to in fact represent nothing more than the presence of two or more polytopic field defects, with all the anomalies present sharing a common molecular basis.

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