Conjoined twins — the Cape Town experience

This paper records our experience in the management of 25 sets of conjoined twins seen over a 32 year period (1964–1996). The twins were classified into 14 complete and symmetrical sets and 11 incomplete or heteropagus. The 14 symmetrical sets included 9 thoracopagus, 2 ischiopagus, I craniopagus and 1 omphalopagus twins. In the incomplete heteropagus group there was I ischiopagus, one twin being anencepahlic, 2 dipygus, 5 parasitic, 2 fetus-in-fetu and I cranial and caudal. The management is detailed case by case. Overall 10 of 14 symmetrical sets underwent attempts at separation with 16 surviving the procedure, but there were 3 late deaths. In the incomplete group 10 of 11 were operated on with 9 survivors. The importance of a multi-disciplinary approach, the extensive investigations required pre-operatively to define areas of organ and bony conjuction, congenital anomalies of each twin and surgical teamwork is emphasized. Specific problems encountered were indentified. In thoracopagus twins the hearts were of paramount importance as conjuction was usually fatal, being associated with major congenital defects. The greater the extent of thoracic cage fusion the greater the chance of associated severe anomaly. Skin expansion to assist coverage of the defects after separation was of great assistance, as was the use of collagen coated vicryl. Evaluation of the liver and pancreatico-biliary systems with isotope excretion scanning was crucial to pre-operative planning. Where there was fusion of the duodenum a single pancreatico-biliary system could be expected and prior strategies for separation and Rouxen-Y enteric drainage of both pancreatic and biliary secretion should be planned. Gastro-oesophageal reflux led to considerable morbidity in both twins of a thoraco-omphalopagus set. In ischiopagus and dipygus conjoined twins bilateral posterior iliac osteotomies were an essential component to anatomic reconstruction of the pelvic ring and wound closure. Also in this group, due to the frequency and extent of shared genital, urinary and ano-rectal structures, long-term morbidity was expected and a component of this might be due to spinal cord tethering, or as in one of our cases, a progressive hydrosyringomyelia. Timing of separation was ideally set at between 5 and 9 months with 6 to 8 weeks of prior tissue expansion but earlier operation was frequently required because of cardio-respiratory problems or organ failure in one twin. In most cases the goal of obtaining separate, independant and intact individuals was achieved.     

Conjoined twins in the Philippines: experience of a single institution

Introduction  

We describe our experience with 22 conjoined twins managed from 1974 to 2006.     

Conjoined epigastric heteropagus twins: excision of a parasitic twin from the anterior abdominal wall of her sibling.

Epigastric heteropagus twins (EHT) are an exceedingly rare form of asymmetric conjoined twins in whom the dependent twin (parasite) is attached to the right or left upper abdomen of the dominant part (autosite). Such a case observed at our institution with 34 month follow-up is presented here and the surgical technique described. A magnetic resonance imaging (MRI)-supported surgical separation of the parasite with successful closure of the abdominal wall defect of the autosite was performed. Follow-up studies showed an autosite which was alive and in optimal health. A comprehensive review including data from English and non-English literature is presented.     

Conjoined Hearts in Thoracopagus Twins

This study aimed to identify the anatomic and pathologic structural cardiac abnormalities in conjoined twins and to focus on those that have prevented the successful separation of conjoined hearts. A retrospective review was undertaken to examine consecutive cases of thoracopagus conjoined twins with conjoined hearts evaluated at The Children’s Hospital of Philadelphia from 1 January 1980 through 6 October 2008. The records included autopsy and surgical findings as well as clinical reports. The study group included nine sets of conjoined twins with a mean gestational age at birth of 33.8 ± 5.5 weeks. Three twin pairs were stillborn. Five twin pairs died afterward. One pair died of cardiopulmonary failure. The median age at death was 22 days (range, 0–345 days). Major congenital heart disease was present in 94.4% (17/18) of the hearts, and 72.2% (13/18) of the hearts had single-ventricle physiology. Total anomalous pulmonary venous return occurred in 39% (7/18) of the cases. The clinical outcome for thoracopagus twins with conjoined hearts remains poor because of inability to separate conjoined and single ventricles. Surgical nonintervention and palliative care should be strongly considered for these patients.     

联体婴儿的发病现状与防治

联体婴儿是一类复杂而罕见的先天畸形,其发病率相差较大,临床常根据连接部位进行分类,分为8个基本类型:头联体、胸部联体、脐联体、坐骨联体、侧联体、颅骨联体、臀联体、脊柱联体,其中胸部联体最为常见,出生后实施分离手术是一种合理而有效的治疗方法,手术时机应选择在出生3个月最为理想。但手术难度大,风险高,需要多个医疗学科配合的、有经验的医疗小组共同完成。应注意预防此类畸形的发生。