Transient neonatal diabetes mellitus in a pair of twins

The occurrence of transient neonatal diabetes mellitus in male twins with almost identical courses of illness is reported. A trial with chlorpropamide treatment of twin A had no obvious influence on the insulin consumption or on duration of treatment. Very low values of plasma C-peptide and serum proinsulin with no detectable insulin antibodies supports the theory of delayed maturation of the beta-cell.     

Imaging of conjoined twins

The incidence of conjoined twins is estimated to be around 1 in 250,000 live births. There is a distinct female predominance. In this paper the imaging of conjoined twins both antenatally and postnatally is reviewed, in particular taking into consideration recent advances with multidetector CT. Accurate counselling of parents regarding the likely outcome of the pregnancy and the likelihood of successful separation is dependent on good prenatal imaging with ultrasound and MRI. Planning of postnatal surgical separation is aided by accurate preoperative imaging which, depending on the conjoined area, will encompass many imaging modalities, but often relies heavily on CT scanning.Not all the examinations presented as figures in this article were performed at our institution, but all these patients were referred to us for review. No patient therefore is identifiable from these anonymized images.     

Parasitic conjoined twins: a case report

Parasitic twins are a rare phenomenon and a challenge for caregivers. Occurring as a result of abnormal processes in the first several weeks of embryologic development, this condition is oftentimes diagnosed by prenatal ultrasound. This unusual condition may go undiagnosed until the time of delivery, however, despite the availability and use of advanced ultrasound techniques. The challenges presented by this condition are multiple and complex. The following case report demonstrates some of these challenges.     

Angiographic evaluation of conjoined twins

Two sets of conjoined twins were studied by angiocardiography and cerebral angiography. Conjoined heart was demonstrated in the thoracopagus twins and surgical separation was impossible. Cerebral angiography disclosed the separate circulations in craniopagus twins and surgical separation was performed.Presented at 14th Meeting of the European Society of Pediatric Radiology, Luzern, May 11–14, 1977     

Neuroblastoma in a pair of identical twins

Hereditary neuroblastoma in a pair of identical twins is reported. In one of the twins the tumor occurred in fetal life and caused death of the twin before birth; in the other one, the tumor occurred at two months of life as IV-S neuroblastoma in the liver with primary tumor undetected. The unusual clinical course of the latter case is described: twice there was tumor regression, but in spite of treatment, fatal uncontrolled growth occurred. The family history, characterized by neoplasms of dissimilar cell types but without additional cases of neuroblastoma, is also detailed.     

先天性联体畸形的外科处理

目的 总结4例联体儿分离术的经验,对联体儿诊治提供参考。方法 回顾性分析自1982～2001年,我院小儿外科共收治的4例先天性联体儿外科分离手术的临床资料,着重在手术时机、产前诊断、合并畸形、分离技术、术后监护、随访等方面。结果 4例联体儿畸形,其中胸部-脐联合畸形3例,坐骨联体1例,男性1例,余均为女性。均不同程度合并先天性心脏缺损,坐骨联体儿合并严重大血管畸形,泄殖腔畸形及Pieer-Robin综合征。2例产前超声检查获得诊断。2例紧急分离术时间为出生后7d内,存活1名。另2例延迟分离手术时间>1个月,均存活。随访存活的5名患儿,1982年分离的剑突-脐联体儿其中1名于术后4年“肺炎”死亡。另1名已20岁,健在。另一对胸部-脐联体儿术后1年发育良好。1例坐骨联体儿1名死亡,1名失随访。结论 手术时机与分离方式必需依据每一对联体儿局部环境及器官结构而定。紧急分离术存活率低,能延迟行分离术则存活率高。     

Assessment of the cardiovascular system in conjoined thoracopagus twins

The thoracoomphalopagus conjoined twins comprise 75% of all conjoined twins. In the assessment of the organ systems of the twins, the cardiovascular system is important since union of this system in the twins has occurred in 75% of the cases. Of the five cases reported here, two had separate hearts and vessels with a common pericardium only. Two twins were joined at the right atrial level. The fifth pair were connected at both atrial and ventricular levels. It has been suggested that the presence of two separate QRS complexes in the ECG indicates complete separation of the two hearts. Case 4 shows that even in the presence of atrial union, the existence of an atrioventricular block in one twin may produce a very slow heart rate, while the other twin may have a normal rate that will not influence the ventricular rate of the other. In our experience, angiocardiography has been the most useful diagnostic procedure.     

Asplenia syndrome in a pair of monozygotic twins

Asplenia syndrome is one of the heterotaxy syndromes, which many familial and animal studies suggest are caused by the loss of adequate genetic control of normal left–right asymmetry development. Moreover, there has not been any environmental factor documented to cause these syndromes. Asplenia syndrome occurring in a pair of monozygotic twins is reported. In view of the negative family history, a new germline mutant gene might be the aetiology of our patients. Both twins are associated with some degree of discordant complex heart defects within the context of a high degree of "mirror-image" of the unpaired thoracoabdominal organs.
Conclusion: This report implies that sporadic asplenia syndrome might associate with new mutations and further genetic study may be indicated. These monozygotic twins' discordant phenotypes imply that some unidentified factors play an important role in their ultimate development of the same genetically determined abnormalities.     

Surgical management to conjoined twins in Shanghai area

Conjoined twins are very rare congenital malformation. The aim of this study was to summarize our experiences of surgical separation on seven sets of conjoined twins, and improve the treatment of conjoined twins in the future. A retrospective review of surgical separation included data of prenatal diagnosis, associated malformation, timing of separation, intra- and postoperative management, and follow-up of six sets of conjoined twins at Shanghai Xin-Hua Hospital from 1980 to 2005 and one set at Shanghai Children’s Hospital in 2002. Surgical separation was performed on seven sets of conjoined twins; six sets of thoracopagus–omphalapagus (including four sets of xipho-omphalopagus) and one set of ischiopagus. All sets presented varying degrees of severity of congenital cardiac malformations. Four sets were diagnosed prenatally by ultrasonography. Two sets of conjoined twins (case 2 and case 3) required emergent separation within 7 days after birth; both members of case 2 died within 2 days post operation, one member of case 3 died during operation while the other member survived. Five sets had scheduled separation undertaken more than 30 days after birth. One member of a set (case 6B) died 13 days after operation due to severe congenital cardiovascular anomalies. All other members of conjoined twins survived. Case 6A had a severe defect of the anterior thoracic cage and prosthesis of titanium alloy scaffold filled with silicone rubber was used to repair the defects successfully. Following up from 1980 to 2005, one member of a set (case 1A) died 4 years after operation due to pneumonia. Contact was lost to the surviving member of case 3 (ischiopagus). Other survivors of the separations had normal development. (1) Timing of operation and separation plan should be given according to the circumstances and the nature of the organ shared in each individual set of twins. (2) Prosthesis of titanium alloy scaffolds filled with silicone rubber may become one of viable methods for repairing severe defects of the thoracic cage.     

Ocular findings in conjoined (Siamese) twins

Conjoined twinning is a rare form of congenital anomaly. The ocular findings in six sets of conjoined twins as well as those reported elsewhere include abnormal optic nerve decussation, pseudosynophthalmos, microphthalmia, abnormal eyelids, orbital encephalocele, occipital encephalocele, and eyelid coloboma. These findings are interpreted as due to deformations from appositional fusion-related factors or malformations from developmental factors.     

Discordant and nondiscordant twins: comparative multimethod risk assessment in the neonatal period

Full-term neonate twins from weight-discordant pairs were compared with nondiscordant pairs on perinatal variables, minor physical anomalies, developmental status, and temperament/interactive measures. For perinatal variables, twins from the top quartile of discordance were found to be at higher risk than other twins. Both the total group of discordant twins and twins with the most extreme discordance had higher weighted minor physical anomaly scores, more minor physical anomalies, and lower developmental status scores than nondiscordant twins. For temperament, twins in the extreme discordant group were more active while awake than twins in the nondiscordant group. The results are discussed in relation to expected group differences in perinatal measures, the timing during gestation of insults related to discordance and minor physical anomalies, and the significance of behavioral differences between cotwins versus between groups. Implications for developmental risk for both twins from discordant pairs are discussed.     

Separation of ischiopagus tetrapus conjoined twins in the sultanate of oman

Premature male ischiopagus tetrapus twins weighing 2.2 kg were born unexpectedly. They required initial ventilation for respiratory distress syndrome and an emergency laparotomy for intestinal obstruction at 10 days of age. At 8 months of age, formal separation was carried out. Primary skin closure was possible without the use of any tissue expander. Anaesthesia was supplemented with continuous intra- and post-operative epidural analgesia, with great benefit. Both twins are alive and well 18 months after surgery. Details of the anatomical findings and preparation for surgery are discussed.     

联体畸形的诊断与治疗方法探讨

目的 探讨联体畸形的诊断和治疗方法，增进对该少见疾病的认识，提高疗效，以及生存率。方法 采用B超、CT 消化道钡餐等检查，判定内脏连接情况，手术分离2对联体畸形婴儿。2对病例分别为剑脐联体畸形及胸腹联体畸形，均有肝脏连接，但胆囊及胆管各自独立，于生后第28d、第96d时分别实施分离手术，对于肝脏连接的处理，采用“局部血流阻断法”，行共用肝离断术。结果 2对联体畸形经充分术前准备，均手术分离成功，术后患儿生长及发育正常。经采用局部血流阻断共用肝离断术方法，术中出血少，术后肝功能恢复顺利。结论 联体畸形较为罕见，充分的术前准备及合理的分离术式可提高患儿的双双存活率。局部血流阻断共用肝离断术，仅阻断了离断线局部的肝脏血流，对非操作区的肝脏血液供应毫无影响。有利于对创伤及失血耐受性很差的婴幼儿术后康复。     

Unusual cardiac malformations in conjoined twins: thoracopagus twins with conjoined pentalogy of Cantrell and an omphalopagus twin with atretic ventricles

Two unrelated cases of conjoined twins were found to have cardiac malformations that apparently have not been reported previously. In one case, thoracopagus twins had an extensive thoracoabdominal wall defect that resulted in ectopia cordis of a conjoined heart along with evisceration of the shared liver and intestine along with one spleen. These malformations, accompanied by defects in the sternum, diaphragm, and supraumbilical abdominal wall, constitute a conjoined pentalogy of Cantrell. In the second case, the heart of one of omphalopagus twins consisted of a solid ventricular mass with only a minute aortic cavity but no atrioventricular communication-an ineffective heart that could develop only in a conjoined or chorioangiopagus twin. In both cases, a common atrium lay in the primitive (embryologic) position caudal to the ventricles.