A case report describing the successful separation of ischiopagus tetrapus conjoined twins in Vietnam

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Abstract

Ischiopagus conjoined twinning is a rare congenital defect. The surgical separation of conjoined twins is difficult because of the complex anatomy and physiology. Careful preoperative assessment, planning, and effective surgical teams are critically important for achieving a good outcome. We describe the successful separation of ischiopagus tetrapus conjoined twins as a representative case demonstrating the growth of pediatric surgery in southern Vietnam.

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Most cited references 12

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Surgical experience with thirteen conjoined twins.

J A O'Neill, G W Holcomb, L Schnaufer … (1988)

Conjoined twins occur in approximately one in 50,000 or so births, and most do not survive. The authors report herein their experience with 13 conjoined twins over the last 30 years, involving those of the following forms: thoracopagus (4 cases), omphalopagus (1 case), ischiopagus (4 cases), pygopagus (1 case), craniopagus (1 case), and incomplete or parasitic varieties (2 cases). The various diagnostic and imaging studies used are described in detail for each form of twinning. Separation is best delayed until such infants are relatively mature (i.e., 6-12 months of age). Operative survival was 50% in those operated on in the neonatal period, but 90% in those over 4 months of age. Ten separations were attempted in 13 sets of twins, with 16 operative survivors. Significantly, up to 10 years after surgery, there were six late deaths due to serious associated congenital anomalies, predominantly cardiac. Improved recent survival is probably the result of the availability of more accurate imaging studies and better anesthetic and operative techniques, with great emphasis on performing immediate reconstruction whenever possible. Use of skin expanders and prosthetic mesh has facilitated wound closure. In the future, ex vivo cardiac reconstruction and autotransplantation may permit separation of twins with complicated conjoined hearts.

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Conjoined twins: From conception to separation, a review.

Nitsa Topale, R. Tubbs, Tanuj Sharma … (2017)

Conjoined twins are suggested to result from aberrant embryogenesis. The two main theories proposed to explain the phenomena are fission and fusion. The incidence rate is 1 in 50,000 births; however, since about 60% of the cases are stillborn, the true incidence is approximated at 1 in 200,000. There is a higher predisposition towards female than male gender with a ratio of 3:1. Conjoined twins are classified based upon the site of attachment. The extent of organ sharing, especially the heart, determines the possibility and prognosis of a separation procedure. Meticulous preoperative evaluation, planning, and preparedness of the team are crucial for a successful separation. Separation of conjoined twins poses several technical, legal, and ethical issues. Clin. Anat. 30:385-396, 2017. © 2017 Wiley Periodicals, Inc.

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Conjoined twins – role of imaging and recent advances

Rishi Philip Mathew, Swati Francis, Ram Shenoy Basti … (2017)

Introduction Conjoined twins are identical twins with fused bodies, joined in utero. They are rare complications of monochorionic twinning. The purpose of this study is to describe the various types of conjoined twins, the role of imaging and recent advances aiding in their management. Material and methods This was a twin institutional study involving 3 cases of conjoined twins diagnosed over a period of 6 years from 2010 to 2015. All the 3 cases were identified antenatally by ultrasound. Only one case was further evaluated by MRI. Results Three cases of conjoined twins (cephalopagus, thoracopagus and omphalopagus) were accurately diagnosed on antenatal ultrasound. After detailed counseling of the parents and obtaining written consent, all the three cases of pregnancy were terminated. Delivery of the viable conjoined twins was achieved without any complications to the mothers, and all the three conjoined twins died after a few minutes. Conclusion Ultrasound enables an early and accurate diagnosis of conjoined twins, which is vital for obstetric management. MRI is reserved for better tissue characterization. Termination of pregnancy when opted, should be done at an early stage as later stages are fraught with problems. Recent advances, such as 3D printing, may aid in surgical pre-planning, thereby enabling successful surgical separation of conjoined twins.

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Author and article information

Contributors

Truong Quang Dinh

Nguyen Minh Duc

Journal

Journal ID (nlm-ta): Radiol Case Rep

Journal ID (iso-abbrev): Radiol Case Rep

Title: Radiology Case Reports

Publisher: Elsevier

ISSN (Electronic): 1930-0433

Publication date PMC-release: 15 July 2021

Publication date Collection: September 2021

Publication date (Electronic): 15 July 2021

Volume: 16

Issue: 9

Pages: 2658-2662

Affiliations

[a ]Department of General Surgery, Ho Chi Minh City Children's Hospital, Ho Chi Minh City, Vietnam

[b ]Department of Radiology, Hanoi Medical University, Ha Noi, Viet Nam

[c ]Department of Radiology, Pham Ngoc Thach University of Medicine, Ho Chi Minh City, Viet Nam

[d ]Department of Radiology, Children's Hospital 2, Ho Chi Minh City, Viet Nam

[e ]Department of Neonatal Intensive Care, Ho Chi Minh City Children's Hospital, Ho Chi Minh City, Vietnam

[f ]Department of Anesthesiology, Ho Chi Minh City Children's Hospital, Ho Chi Minh City, Vietnam

Author notes

[* ]Corresponding authors. truongdinhnd2@ 123456gmail.com bsnguyenminhduc@ 123456pnt.edu.vn

[1]

These authors contributed equally to this article as co-first authors.

Article

Publisher Item ID: S1930-0433(21)00424-6

DOI: 10.1016/j.radcr.2021.06.048

PMC ID: 8319480

PubMed ID: 34345326

SO-VID: 783f1780-3e55-40c3-8d5e-366d8c5c1073

License:

This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

History

Date received : 12 June 2021

Date revision received : 14 June 2021

Date accepted : 16 June 2021

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