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Heteropagus twins: six cases with systematic review and embryological insights

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Abstract

Purpose

Heteropagus twinning (HT) is a rare anomaly. Six new cases along with a systematic review are described.

Methods

Six cases of HT managed at two tertiary care teaching hospitals over the last 26 years are described. A PubMed search with words: Heteropagus AND/ OR parasitic twins from 2001 to 2021 hit 183 articles. 36 were added from non-PubMed sources. Finally, 120 cases including 114 from 69 articles and 6 new cases were analysed.

Results

Of the new cases, 2/6 had an antenatal diagnosis. Five were males. 4 autosites had omphaloceles. Split notochord and 2 parasites attached to a single autosite were encountered. 5/6 autosites survived.

On systematic review, the most frequent variant seen was rachipagus (n = 50) followed by omphalopagus (n = 46). Limbs were reported in 75 cases. Congenital heart disease was seen in 17/120(14.2%) autosites. Omphalocele and meningomyelocele were the most common extracardiac anomalies in autosites. Weight along with the anatomy and position of heteropagus twins was a better determinant of the mode of delivery than weight alone. Mortality was reported in 12 cases.

Conclusion

Autosites in HT generally carry a good prognosis, however, final outcome depends mainly on associated major cardiac anomalies. Meticulous antenatal assessment and preoperative planning are of paramount importance.

Level of evidence

IV.

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Abbreviations

CECT:

Contrast enhanced computed tomography

CHD:

Congenital heart disease

DOL:

Day of life

DNA:

Deoxyribonucleic acid

HT:

Heteropagus twinning

MRI:

Magnetic resonance imaging

POD:

Post-operative day

USG:

Ultrasonography

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Acknowledgements

This research did not receive any grants from funding agencies in the public, commercial, or not-for-profit sectors.

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This research did not receive any grants from funding agencies in the public, commercial, or not-for-profit sectors.

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Authors and Affiliations

  1. Department of Pediatric Surgery, SMS Medical College, Jaipur, Rajasthan, 302004, India

    Praveen Mathur, Priyanka Mittal & Dinesh Barolia

  2. Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India

    Shilpa Sharma

  3. Department of Radiodiagnosis, SMS Medical College, Jaipur, India

    Raj Kumar Yadav

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  1. Praveen Mathur
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  2. Shilpa Sharma
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Contributions

CONCEPTUALISATION : Praveen Mathur; LITERATURE SEARCH AND DATA ANALYSIS : Priyanka Mittal; ASSISTANCE IN CLINICAL STUDIES: Dinesh Barolia, Raj Kumar Yadav; ORIGINAL DRAFT PREPARATION : Priyanka Mittal; REVIEW AND EDITING : Shilpa Sharma; CRITICAL REVISION : Praveen Mathur, Shilpa Sharma. All authors approved the final version of the manuscript.

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Correspondence to Priyanka Mittal.

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Ethical approval was waived in view of the retrospective nature of the study and all patients were managed and treated as per standard institutional protocol.

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Mathur, P., Sharma, S., Mittal, P. et al. Heteropagus twins: six cases with systematic review and embryological insights. Pediatr Surg Int 38, 963–983 (2022). https://doi.org/10.1007/s00383-022-05135-w

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