The SCIA-based conjoined flap for covering extremely large lower limb defects / 中华显微外科杂志

Objective:To investigate the results of the superficial circumflex iliac artery(SCIA)-based conjoined flap for covering extremely large lower limb defects.Methods:From February 2017 to June 2019, 15 patients were admitted, who suffered from severe degloved injury for the lower limb, including 9 males and 6 females with a median year of 45 (ranged, 36-67 ) years old. All were taken thorough and radical debridement and covered by the VSD device during the emergency operation. The dimension of defects was ranged from 25.0 cm ×8.0 cm to 50.0 cm ×15.0 cm. Using the lower abdominal wall or side chest wall as the donor site, the conjoined flap was dissected when the wound surface became granulating. The perforator match fashions included bilateral SCIA, and ipsilateral SCIA and thoracodorsal artery(TA). The donor sites were primary closure. The follow-up was accomplished by the same surgeon.Results:Fourteen flaps survived completely without significant complications, and distal necrosis was observed in one longitudinal flap, which was healed with the skin graft in the second stage. All flaps were available for a mean follow-up of 18 (ranged 16-24) months. The aesthetic outcomes were achieved on the recipient site without hairy appearance and hyper-pigmentation. A concealed line scare was left on the donor site, without the hernia and limited function. At the last follow-up, 7 cases were excellent and 1 case was good, evaluated with the LEFS criteria. And 6 cases were excellent and 1 case was good, assessed by the AOFAS criteria.Conclusion:The simultaneous reconstruction of extreme lower limb defects and better salvage treatment could be achieved by the SCIA-based conjoined flap. And as a versatile flap, it was blessed with concealed donor site, various design fashions, and larger dissection size in selected cases.

The external locking compression plate combined with inferior abdominal conjoined flap for fixing open fracture and covering soft tissue defects on tibia / 中华显微外科杂志

Objective:To discuss the clinical effect about the external locking compression plate(LCP) combined with lower abdominal conjoined flap for fixing the open fracture and covering the soft tissue defects on tibia.Methods:From August 2017 to December 2020, 18 patients with serve tibial open fracture were admitted into the trauma center, including 15 males and 3 females with a median age of 38 (ranged, 25-58) years old. The etiology involving: 9 cases by traffic accident, 3 by downfall, 6 by crushing, which classified as type III B( n=6) and III C( n=12) by the Anderson-Gustilo criterion. All wounds were taken radical debridement, fixed by the femur LCP and covered by the VSD during the emergency operation. The lower abdominal conjoined flap was dissected to cover the soft tissue defect, of which the dimension and pedicle length were tailored to the defect. Primary closure was performed on the donor site. Followed-up was conducted by telephone and WeChat. Results:One flap was changed to gastrocnemius myocutaneous flap because of the venous crisis. Seventeen flaps survived completely without significant complications. All the donor and recipient sites had primary healing. A mean follow-up of 15 (ranged, 12 to 18) months. The fracture healed without bone infection and bone nonunion. The aesthetic outcomes were satisfied without overgrown hairy and hyperpigmentation for all flaps. The concealed linear scar was left without hernia or other morbidity on the donor site. At the final follow-up, 12 cases were excellent and 6 cases were good evaluated by the Johner-Wruhs criteria.Conclusion:The external LCP can immobilise the knee and ankle joint with the preservation of the soft tissue, and the free lower abdominal conjoined flap was useful for covering extreme defects with concealed donor site, with enough tissue volume. The combination of both could lower the postoperative infection, reduce the operation time and shorten the hospital stay.

Antenatal Diagnosis of Parapagus Conjoined Twins: 3D Virtual and 3D Physical Models / Diagnóstico antenatal de gêmeos unidos parapagus: Modelos 3D virtuais e físicos

Abstract Conjoined twins (CTs) are a rare complication from monochorionic and monoamniotic twin pregnancies. We describe the use of 3D technologies, including 3D virtual and 3D physical models on prenatal evaluation of one parapagus CT. A 16-year-old G1P0 woman was referred for fetal magnetic resonance imaging (MRI) anatomical evaluation of a CT at 28 weeks of gestation. 3D images of the fetal surface were generated by the software during the examination for spatial comprehension of the relationship between the fetal parts. The pair of CTs died at the 32nd week of gestation, a few hours after cesarean section. 3D technologies are an important tool for parental counseling and preparation of the multidisciplinary care team for delivery and neonatal assistance and possible surgical planning for postnatal separation in CTs cases.

Resumo Gêmeos unidos (GUs) são uma complicação rara de gestações gemelares monocoriônicas e monoamnióticas. Descrevemos o uso de tecnologias 3D, incluindo modelos 3D virtuais e físicos, na avaliação pré-natal de GU parapagus. Mulher de 16 anos, G1P0, foi encaminhada para avaliação anatômica por ressonância magnética (RM) fetal de GU com 28 semanas de gestação. Imagens 3D da superfície fetal foram geradas pelo software durante o exame para compreensão espacial da relação entre as partes fetais. O par de GUs morreu na 32ª semana de gestação, poucas horas após a cesariana. As tecnologias 3D são uma importante ferramenta de aconselhamento dos pais e de preparação da equipe multiprofissional para o parto e a assistência neonatal, além de possível planejamento cirúrgico para separação pós-natal em casos de GUs.

Deradelphous Cephalothoracoomphalopagus in kittens: case report / Cefalotoraconfalopago derafelfo em gatos: relato de caso

The present report describes a case of conjoined twins of the cephalo-thoraco-omphalopagus deradelphous type in cats. A feline female was transferred to our veterinary hospital as an emergency for dystocic labor. The stillborn was subjected to radiographic evaluation, and a single skull and two complete distinct vertebral columns were found. Anatomopathological examination revealed that the twins presented the head, chest, and umbilicus as the main points of union and were classified as the cephalo-thoraco-omphalopagus type. In addition, the twins had unique and well-developed faces, which allowed them to be classified as deradelphous. This malformation is rare in domestic animals, and to the best of our knowledge, this type has not been reported in felines. Further studies are warranted on this embryonic alteration, primarily because its etiology remains unknown.(AU)

O presente relato descreve um caso de gêmeos siameses do tipo cefalotoraconfalopago deradelfo em gatos. Uma fêmea felina foi atendida na emergência do hospital veterinário em trabalho de parto distócico. Os natimortos foram encaminhados para avaliação radiográfica e constatou-se que apresentavam um único crânio e duas colunas vertebrais completas e distintas. O exame anatomopatológico evidenciou que os gêmeos possuíam cabeça, tórax e umbigo como principais pontos de união, sendo classificados como cefalotoraconfalopago. Além disso, os gêmeos apresentaram face única e bem desenvolvida, o que permitiu classificá-los como deradelfos. Esse tipo de malformação é raro em animais domésticos, e não foi encontrado nenhum trabalho em felino com a mesma classificação do presente relato. Há necessidade de mais estudos sobre essa alteração embrionária, pois a etiologia do processo ainda não foi esclarecida.(AU)

Second-trimester termination due to conjoined twins in a woman with twice previous cesarean sections / 中华围产医学杂志

We report the induced labor of conjoined twins in the second trimester in a woman with a history of two previous cesarean sections, the last one of which was performed in 2017. This 25-year-old patient was found to have thoracolumbar conjoined fetuses with one heart and polyhydramnios through the routine ultrasound examination at 19 +5 gestational weeks and was admitted at 20 +1 gestational weeks. After a full assessment of the fetal and maternal condition through multidisciplinary consultation, it was determined to attempt a vaginal delivery as no absolute contraindication for induction of labor. The patient was given 300 mg mifepristone orally plus an amniotic cavity injection of 100 mg ethacridine lactate. Regular contractions occurred 28 hours after medication. The patient delivered a pair of dead female conjoined twins at 20 +6 gestational weeks following successful induction of labor, with an assisted vaginal breech delivery. There was no soft tissue damage in the birth canal, and the estimated blood loss was 150 ml. Pathological examination and autopsy showed thoracolumbar conjoined deformity twins with a common heart and liver. Adequate prenatal evaluation, a detailed understanding of the indications for induction of labor and vaginal delivery, closed monitoring during labor, and preparation for emergency cesarean section, are essential safety measures for induced labor of conjoined twins in women with a scarred uterus in the second trimester.

Multiple Conjoined Oocytes In A Patient With Polycystic Ovary Syndrome Undergoing In Vitro Fertilization

@#<p>Conjoined oocytes are characterized by a follicle containing two oocytes surrounded by a single zona pellucida or their fusion in a zonal region. Gonadotropin stimulation, coupled with PCOS, predisposes to the occurrence of conjoined oocytes. Although a result of developmental accident, conjoined oocytes have the potential to develop into genetically normal embryos and babies. In this paper, the authors describe the aspiration of multiple conjoined oocytes in a single IVF cycle, leading to normal fertilization after selective intracytoplasmic sperm injection (ICSI). Development to blastocyst stage allowed trophectoderm biopsy resulting to a euploid embryo that was eventually transferred, and resulted to a live term birth.</p><p>Key words: conjoined oocytes, in vitro fertilization (IVF), intracytoplasmic sperm injection(ICSI)</p>

Asymmetric parasitic twins - Heteropagus

SUMMARY BACKGROUND: Asymmetric or heteropagus conjoined twins is a rare occurrence, with an incidence of one case in 1-2 million. Conjoined twins are classified according to their symmetry, place of fusion, and grade of duplication. METHODS: We report here an extremely rare presentation of parasitic twins not described before. We describe macro and micro anatomic alterations and discuss the aspects of this peculiar presentation and the importance of prenatal diagnosis. RESULTS: The case of a twenty-three-year-old patient, with monochorionic, monoamniotic asymmetrically-conjoined twin pregnancy, discovered at 29 weeks of gestational age. We believe that this report calls attention to this presentation and the importance of prenatal care and management. The twins were delivered vaginally without life. The twins' combined weight was 1.300 gr. They were bonded in the left cervical region. CONCLUSION: This report may help to find strategies for clinical decisions in future cases. Antepartum diagnosis is important to the management, preoperative planning, and outcomes. Prenatal imaging exams like echocardiography, CT, MRI, and ultrasonography are feasible and can provide relevant information about malformation severity and prognosis.

RESUMO BACKGROUND: Gêmeos parasitas assimétricos ou heterópagos são uma ocorrência rara, com incidência de um caso em 1-2 milhões. Os gêmeos siameses são classificados de acordo com sua simetria, local de fusão e grau de duplicação. MÉTODOS: Relatamos aqui uma apresentação extremamente rara de gêmeos parasitários não descritos anteriormente. São descritas alterações macro e microscópicas e discutidos aspectos relevantes dessa malformação e da importância do diagnóstico pré-natal. RESULTADOS: Um caso de uma paciente de 23 anos de idade, com gestação monocoriônica, monoamniótica de gêmeos siameses assimétricos diagnosticada com 29 semanas de idade gestacional. Acreditamos que este relato chama a atenção para esta apresentação e para a importância do cuidado e manejo pré-natal. Os fetos nasceram de parto vaginal já sem vida, pesando em conjunto 1.300 gramas, e eram unidos pela região cervical esquerda. CONCLUSÃO: Este relato pode ajudar a encontrar estratégias para a decisão clínica em casos futuros. O diagnóstico pré-natal é fundamental para o manejo e planejamento pré-operatório. Exames de imagem como ecocardiografia, tomografia computadorizada, ressonância magnética e ultrassonografia são factíveis e podem fornecer informações-chave sobre a gravidade e prognóstico da malformação.

A rare case of dicephalic parapagus conjoined twins

Dicephalic parapagus are one of the rare forms of conjoined twins. Their prognosis is usually very poor. Early prenatal diagnosis of these serious congenital abnormalities remains a real challenge for health systems in the low-income countries. The late antenatal diagnosis of a case of this abnormality was presented. The diagnosis was made at the 33rd week on the only ultrasound performed during the pregnancy. The pregnancy was interrupted with the agreement of the couple and delivery was performed by caesarean section.

Parapagus dicephalus conjoined twin: a case report

A conjoined twin is a rare event. As per recent literature, 60% of conjoined twins are aborted spontaneously and hence true incidence of conjoined twin is approximately 1 in 200,000 live births. Classification is based upon site of attachment out of which parapagus with dicephalic variety is extremely rare (0.5% amongst reported cases). Author report a case of dicephalic twin born at 36 weeks of gestation to a multigravida mother in central India.

One stage repair of dorsum of hand and multiple fingers soft tissue defect with polyfoliate conjoined anterolateral thigh perforator flap / 中华显微外科杂志

Objective:To investigate the surgical method and clinical effect of one-stage repair of soft tissue defects of dorsum of hand and multiple fingers by using the polyfoliate conjoined anterolateral thigh perforator flap (ALTP) .Methods:From November, 2015 to September, 2019, 9 cases of soft tissue defects of dorsum of hand and multiple fingers were treated, including 8 males and 1 female. The average age was 32 years old. Causes of injury: 4 cases of thermal injury, 3 cases of strangulation injury, and 2 cases of traffic accident injury. Location of injury: 7 cases of right hand, 2 cases of left hand. Three cases of dorsum of hand with 1st-4th fingers, 3 cases with 2nd-5th fingers, 2 cases with 2nd-4th fingers, and 1 case with 3rd-5th fingers; All with bone, joint and tendon exposure. There were 6 cases with fracture or joint dislocation. All of them had no obvious defect of bone and joint. VSD was used to cover them after debridement. According to the shape and size of the wound, all flaps were designed to repair the soft tissue defect as the polyfoliate conjoined ALTP. The flap size was 7 cm×13 cm-12 cm×17 cm. Regular outpatient, telephone and Wechat follow-up were carried out to evaluate the flap survival, appearance, color elasticity, donor scar, sensory recovery, finger function recovery and patient satisfaction.Results:Nine cases of flap survived well. The average followed-up time was 12 (6-18) months. The postoperative followed-up revealed satisfactory shapes of the flaps, protective touch and recovery of functions. The donor site had good healing and no effect on function. In the last followed-up, according to the trial criteria for function evaluation of upper limbs by Chinese Society of Hand Surgery, 5 cases were excellent, 3 cases were good, and 1 case was fair. The hand function recovered well and returned to work and daily life on average 6 weeks after operation.Conclusion:The polyfoliate conjoined ALTP can repair the soft tissue defect of hand dorsum with multiple fingers at one stage, and the flap is in good shape. There is no need to divide the fingers. It is one of the ideal methods to repair the soft tissue defect of hand dorsum.

A rare case of conjoined twins presenting as dicephalus parapagus: a case report

Conjoined twinning is a congenital abnormality resulting in various presentations of the fetuses in utero. It is a rare embryological developmental disorder occurring in one in 100,000 births. We present a case of conjoined twinning, which is characterized by the presence of two heads, single vertebral column and single symphysis pubis, with single genitor-urinary tract and single gastro intestinal tract. The present condition, referred to as dicephalus parapagus, is a rare entity among the conjoined twinning and has seldom positive outcomes. The mechanism of occurrence of conjoined twinning has been proposed either through fusion or fission of the embryos. In either case, surgical separation of the conjoined twins is often unsuccessful and results in increased morbidity and mortality. However, it is essential to screen for the presence of such congenital anomalies early during pregnancy, in order to terminate and provide parental counselling.

Thoracopagus: Autoptic and radiologic study of 2 cases

Conjoined twins are a very rare complication of monozygotic twinning, most common being thoracopagus. Here we report about two cases of thoracopagus male twins illustrating the autopsy details of one case and the prenatal magnetic resonance imaging (MRI) details of another case. While the first case was misdiagnosed as separate twins on antenatal USG, only to be later confirmed as thoracopagus twins after birth, the antenatal MRI done in the second case helped in accurate detection of thoracopagus twins. Bilateral peripheral cortical cysts with dysplasia was noted in one of the twins of the first case, which has not been reported earlier in conjoint thoracopagii. Early prenatal diagnosis of conjoined twins is essential for better counselling of parents regarding post natal surgical management or termination of pregnancy. Importance of prenatal MRI for accurate detection of these cases is thereby highlighted.

A rare case of dicephalous dibrachius dipus conjoined twins:case report

Conjoined twin is a rare complication seen in 1% of monochorionic monoamniotic twins and associated with severe morbidity and mortality. We are reporting a case of 30 years old second gravida referred to our tertiary care center at 35 weeks of gestation with ultra-sonographic diagnosis of dicephalus conjoined twin and further confirmed after caesarean delivery.

Siameses toracópagos: Reporte de un caso / Thoracic Siamese: Case Report

Los gemelos siameses son una entidad rara, cuya incidencia varía de 1 en 50.000 a 1 en 100.000 nacidos vivos; solo el 18 % sobrevive y aproximadamente el 35 % de los nacidos vivos muere en las primeras 24 horas. Se reportó un caso de siameses toracópagos, con diagnóstico prenatal mediante ultrasonografía a las 32.1 semanas de gestación, cuyas cabezas y extremidades son individuales; poseen genitales femeninos; a nivel del tórax se observó la existencia de un corazón que envía un vaso arterial a lo que se asemeja a una masa cardiaca inerte y rudimentaria al feto contralateral; comparten los órganos abdominales. El embarazo se interrumpió a las 32.1 semanas de gestación y los pacientes sobrevivieron dos horas, luego de lo cual fallecieron por la cardiopatía compleja.

Siamese twins are rare, occurring 1 in 50 000 to 1 in 100 000 live births; only 18% survive and approximately 35% of live births die in the first 24 hours. We report a case of thoracopagu twins. The prenatal diagnosis was made by ultrasound at 32 weeks of gestation. They had separated heads and extremities; they were females; at the joint thorax there was a single heart that sent an arterial vessel to what resembles a rudimentary cardiac mass in the contralateral fetus. They shared abdominal organs. Pregnancy was interrupted at 32.1 weeks of gestation; patients survived 2 hours.

Autopsy Findings Of Dicephalic Dibrachius Parapagus Conjoined Twins: A Case Report

This is a case report of a set of dicephalic parapagus twin delivered by caesarean section and who survived for22 days postpartum. At autopsy each twin was found to have a separate upper gastrointestinal tract whicheventually became shared in the mid gut region. Their vertebral columns ran side by side and also fused at thelevel of the lumbo-sacral region. Each twin possessed a pair of lungs but they shared a single fused heart. Acomplex cardiopulmonary system was identified which had the potential to support both twins.

Anesthesia and perioperative challenges for surgical separation of thoraco-omphalopagus twins: case report / Anestesia e desafios perioperátorios para cirurgia de separação de gêmeos toraco-onfalópagos: relato de caso

Abstract Background and objectives: Conjoined twins are monozygotic twins physically joined at some part of the body. This is a rare phenomenon, estimated between 1:50,000 and 1:200,000 births. The objective of this report is to present the anesthetic management and the perioperative challenges for a separation surgery. Case report: Thoraco-omphalopagus twins were diagnosed by ultrasound and were followed by the fetal medicine team of the service. After 11 h of cesarean surgery, the pediatric surgical team chose to separate the twins. They were monitored with cardioscopy, oximetry, capnography, nasopharyngeal thermometer, urinary output, and non-invasive blood pressure. We chose inhaled induction with oxygen and 4% Sevoflurane. T1 patient was intubated with a 3.5 uncuffed endotracheal tube, and, after three unsuccessful intubation attempts of patient T2, a number 1 laryngeal mask was used. After securing the twins' airway, the induction was supplemented with fentanyl, propofol, and rocuronium. Mechanical ventilation in controlled pressure mode (6 mL.kg−1) and lumbar epidural (L1-L2) with 0.2% ropivacaine (2.5 mg.kg−1) were used. The pediatric surgical team initiated the separation of the twins via sternotomy, ligation of hepatic vessels. After 2 hours of procedure, the separation was completed, continuing the surgical treatment of T1 and the support of T2 until his death. Conclusions: Conjoined twin separation surgery is a challenge, which requires planning and coordination of a multidisciplinary team during all stages.

Resumo Justificativa e objetivos: Gêmeos conjugados são gêmeos monozigóticos conectados por alguma parte do corpo. Esse é um fenômeno raro, estimado entre 1:50.000 a 1:200.000 nascimentos. O objetivo deste relato é apresentar o manejo anestésico e os desafios perioperatórios para cirurgia de separação. Relato de caso: Gêmeos toraco-onfalópagos foram diagnosticados por ultrassonografia e acompanhados pela equipe de medicina fetal do serviço. Após 11 horas da cesárea, a equipe cirúrgica pediátrica optou pela separação dos gêmeos. Foram monitorados com cardioscopia, oximetria, capnografia, termômetro nasofaríngeo, débito urinário e pressão arterial não invasiva. Optou-se por indução inalatória com oxigênio e sevoflurano a 4%. O G1 foi intubado com tubo orotraqueal 3,5 sem cuff e após três tentativas de intubação do G2 sem sucesso usou-se máscara laríngea número 1. Após obtenção da via aérea nos gêmeos, complementou-se indução com fentanil, propofol e rocurônio. Ventilação mecânica no modo pressão controlada 6 ml.kg-1 e peridural lombar L1-L2 com ropivacaína 0,2% (2,5 mg.kg-1). A equipe cirúrgica pediátrica iniciou a separação dos gêmeos através de esternotomia, ligadura de vasos hepáticos. Após duas horas de procedimento, a separação foi concluída, prosseguiram-se o tratamento cirúrgico de G1 e os cuidados de G2 até o óbito. Conclusões: A cirurgia de separação de gêmeos conjugados é um desafio, requer planejamento e coordenação de uma equipe multidisciplinar durante todos os estágios.

Repair of large area skin defect of forearm with overlength thoracic umbilical conjoined perforator flap with double blood supply / 中华显微外科杂志

Objective To explore the surgical method and clinical effect of repairing the large area skin defect of forearm with the perforator flap pedicle with the inferior epigastric artery perforator and the lateral cutaneous branch of the posterior intercostal artery.Methods From January,2006 to January,2016,14 cases of forearm large area of skin defects were treated with the ovedength flap at one stage.The proximal flap to the inferior epigastric artery umbilical perforation and the flap distal to the posterior interphalangeal artery perforation were used for the blood supply of superficial flap.The length of the flap was 25-43 cm (average,36 cm).The width of the flap was 5-14 cm (average,9 cm).All patients were followed-up regularly.The content of the follow-up included three aspects:appearance,hand function and the recovery of the donor site.Results Twelve cases of postoperative flaps successfully survived.Arterial crisis was seen in 1 flap 28 hours after surgery.The re-surgical exploration was adopted after conservation treatment for 1 h without remission and the proximal anastomotic flap embolization was confirmed.The flap survived.Venous crisis was seen in 1 case.The flap survived with the help of removing the suture,smoothing drainage and bleeding for 5 d.The wounds and the donor site of the thoracic and abdominal region healed at the first stage.The follow-up time was 8-72 months,with an average of 31 months.The flaps had no obvious bloated,the skin texture was close to forearm skin and the flaps were restored protected sensation.There was no ulceration,infection and other complications.The healing of skin graft was satisfactory in 2 cases in abdominal donor site.No skin graft contracture occurred.The remaining 12 cases had linear scar in the abdomen of the donor site.The edge of the scar was soft and no obvious contracture occurred.Conclusion Super long thoracic umbilical conjoined perforator flap can repair the lager area skin defect of forearm with double blood supply.The length of flap is significantly longer with enoughblood-supply of distal part of the flap.The clinical efffect is satisfactory.

Radial artery pedigreed conjoined perforator flap repair cross-joint long-shaped skin and soft tissue defects in fingers / 中华显微外科杂志

Objective To explore the method and clinical effect of radial artery pedigreed conjoined perfora-tor flap for repairing cross-joint long-shaped skin and soft tissue defects in fingers. Methods From June, 2015 to June,2017,six patients with cross-joint long-shaped skin and soft tissue defects of the fingers were treated with radial artery pedigreed conjoined flap which axis was the artery superficial line, and carried two radial artery perforators, in order to enlarge flap cut range to repair.The size of flaps ranged from 3.0 cm ×6.0 cm to 3.5 cm ×7.5 cm.The donor site was directly sutured. After operation, all patients were followed up for 3 to 8 months. All the necessary parts are observed, such as the flaps appearances, textures, the donor sites, checked the flap sensation, activity functions of the fingers. Results Six cases of flap all survived.The wounds healed well(phase I),and all patients were followed up for 3 to 8 months, with an average of 5 months. All the flaps do not obviously bloat, the textures were soft,the colors are normal,the appearances of flaps were similar to recipient sites. The donor sites healed well only with linear scars. Conclusion Using radial artery pedigreed conjoined perforator flap to repair cross-joint long-shaped skins and soft tissue defects in fingers that it not only can enlarge the cut range but also cut conveniently, the textures are close to recipient sites.Therefore,it is an ideal repair way.

Late secondary urological reconstruction of separated ischiopagus twins with exstrophic bladder and urinary incontinence / Reconstrução urológica tardia de gêmeos isquiópagos com extrofia de bexiga e incontinência urinária

ABSTRACT We report a case of secondary urinary reconstruction of previously separated conjoined twins with exstrophic bladder and urinary incontinence. Patients were male and aged 13-year-old. Twin one had a history of failed enterocystoplasty that extruded and was visible like an exstrophic neobladder. He underwent a procedure to close bladder neck and reconfigure abdominal wall. After the procedure the patient developed a fistula that was treated, but it persisted and, for this reason, a catheterizable pouch was constructed and native bladder was discarded. Twin two required the immediately construction of catheterizable pouch using the Macedo's technique. Currently, both patients are continent at 4 hour intervals. The mean follow-up was 8 months. Modern continent urinary diversion techniques offer new perspectives and hope for such complex population.

RESUMO Relata-se caso de reconstrução urinária em gêmeos siameses previamente separados com apresentação clínica de bexiga extrófica e incontinência urinária. Os dois pacientes eram do sexo masculino com idade de 13 anos. O primeiro gêmeo apresentava falha da enterocistoplastia com extrusão e visualização da neobexiga extrófica, tendo sido submetido ao fechamento do colo vesical e à reconfiguração da parede abdominal. Após o procedimento, o paciente desenvolveu fístula, que foi tratada, mas persistiu. Posteriormente, optamos por bolsa cateterizável, descartando a bexiga nativa. O segundo gêmeo foi submetido à construção imediata de bolsa cateterizável, por meio da técnica de Macedo. Atualmente, ambos os pacientes estão continentes em intervalos de 4 horas. O seguimento médio foi de 8 meses. As atuais técnicas de derivação urinária oferecem novas perspectivas e esperança para esta população complexa.

Gemelos unidos (siameses): descripción de hallazgos anatomopatológicos / Conjoined twins (siamese): description of anatomopathological findings

Resumen OBJETIVO: Describir los hallazgos anatomopatológicos y placentarios en una serie de autopsias de 5 siameses. CASOS CLÍNICOS: Serie de casos de autopsias efectuadas entre 2013 y 2018 de pacientes entre 14 y 30 semanas de embarazo, con diagnóstico de gemelos unidos. RESULTADOS: Se encontraron 5 casos de gemelos unidos, hijos de madres multíparas, 3 tuvieron muerte intrauterina y 2 fallecimiento a los pocos minutos de vida. Tres casos correspondieron a uniones ventrales (2 toracoonfalópagos y 1 cefalópago) y dos a uniones laterales de tipo parápagos, dicéfalos. Hubo corazón único en los gemelos toracoonfalópagos y sistemas cardiacos separados en los tres restantes, donde hubo fusión aórtica distal. En 4 casos el hígado se encontró fusionado, con dos vías biliares independientes. Los cordones umbilicales de los casos de unión ventral tenían 5 vasos sanguíneos. Los de unión lateral tenían cordón umbilical trivascular. Los discos placentarios mostraron grados variables de hipoxia preplacentaria y cambios de malperfusión vascular materna. CONCLUSIONES: Las fusiones ventrales tienen grados variables de estructuras compartidas; entre mayor sea la fusión de los órganos internos, menor es el número de vasos umbilicales. De las teorías etiológicas propuestas, la esférica es la que mejor soporta los grados variables de fusión.

Abstract OBJECTIVE: Description of the anatomopathological and placental findings in a series of autopsies of 5 conjoined twins. MATERIALS AND METHODS: Series of cases of autopsies performed between 2013-2018 of patients between 14-30 weeks of gestation, with diagnosis of conjoined twins. RESULTS: Five cases of conjoined twins, product of multiparous mothers, 3 of these presented intrauterine death and 2 deaths within a few minutes of life. Three cases corresponded to ventral junctions (2 thoracoomphalopagus and 1 cephalopagus) and two to lateral junctions of parapagus type. A single heart was observed in the thoracoomphalopagus twins and separated cardiac systems in the remaining three where there was distal aortic fusion. In 4 cases the liver was found fused with two independent bile ducts. The umbilical cords of the cases of ventral union presented 5 blood vessels; those with lateral junction presented a trivascular umbilical cord. The placentas showed varying degrees of preuterine hypoxic pattern and changes of Maternal Vascular malperfusion of the Placental Bed. CONCLUSIONS: Ventral fusions have varying degrees of shared structures; the greater the fusion of the internal organs, the smaller the number of umbilical vessels will be. Of the proposed etiological theories, it is the so-called spherical theory, which best supports the variable degrees of fusion and how it can be saltatory on the vertical axis.