Secuencia de perfusión arterial reversa en embarazo gemelar; coagulación de arteria nutricia. Reporte del primer caso en el país / Twin reversed arterial perfusion sequence; coagulation of the umbilical cord. A report on the first case in Uruguay / Seqüência reversa de perfusão arterial na gravidez gemelar; coagulação da artéria nutritiva. Relato do primeiro caso no país

Resumen: La secuencia de perfusión arterial reversa (TRAP) es una complicación muy poco frecuente y grave de los embarazos gemelares monocoriónicos. Generalmente ocurre cuando el corazón de un gemelo de apariencia normal sirve como bomba para uno o más gemelos dismórficos cuya cabeza, órganos torácicos y extremidades superiores no se desarrollan completamente o no se desarrollan en absoluto y, por lo tanto, carecen de actividad cardíaca. La arquitectura placentaria vascular anómala provoca un cambio en el flujo arterial hacia el gemelo acardíaco. Los mecanismos fisiopatológicos exactos que conducen a este fenómeno devastador no se conocen bien. Compartiremos el caso clínico de una paciente de 19 años, cursando un embarazo gemelar monocorial monoamniótico, en que realizamos diagnóstico de TRAPS, y realizamos la coagulación laser de la arteria nutricia del feto acárdico.

Abstract: Twin reversed arterial perfusion sequence (TRAPS) is rather an unusual and severe complication of monochorionic twin pregnancies. It usually occurs when the normal-appearance heart of a twin acts as a pump for one or more dysmorphic twins whose head, thoracic organs and upper limbs fail to totally develop or do not develop at all and thus, have no cardiac activity. The abnormal vascular architecture at the placenta changes the arterial flow towards the acardiac twin. The exact pathophysiological mechanisms that result in this devastating phenomenon are still unknown. The study presents the clinical case of a 19-year- old patient pregnant with monoamniotic, monochorionic twins and a diagnosis of TRAPS, treated by laser coagulation of the acardiac twin's umbilical cord.

Resumo: A seqüência reversa de perfusão arterial (TRAPS) é uma complicação muito rara e grave de gestações gemelares monocoriônicas. Geralmente ocorre quando o coração de um gêmeo de aparência normal serve como uma bomba para um ou mais gêmeos dismórficos cuja cabeça, órgãos torácicos e membros superiores não se desenvolvem totalmente ou não se desenvolvem e, portanto, não têm atividade cardíaca. A arquitetura vascular placentária anormal causa uma mudança no fluxo arterial para o gêmeo acardíaco. Os mecanismos fisiopatológicos exatos que levam a esse fenômeno devastador não são bem compreendidos. Descrevemos o caso clínico de uma paciente de 19 anos, portadora de gestação gemelar monocoriônica monoamniótica, na qual fizemos o diagnóstico de TRAPS e realizamos coagulação a laser da artéria nutritiva do feto acardíaco.

Onset of brain injury in infants with prenatally diagnosed congenital heart disease.

BACKGROUND: The exact onset of brain injury in infants with congenital heart disease (CHD) is unknown. Our aim was, therefore, to assess the association between prenatal Doppler flow patterns, postnatal cerebral oxygenation and short-term neurological outcome. METHODS: Prenatally, we measured pulsatility indices of the middle cerebral (MCA-PI) and umbilical artery (UA-PI) and calculated cerebroplacental ratio (CPR). After birth, cerebral oxygen saturation (rcSO2) and fractional tissue oxygen extraction (FTOE) were assessed during the first 3 days after birth, and during and for 24 hours after every surgical procedure within the first 3 months after birth. Neurological outcome was determined preoperatively and at 3 months of age by assessing general movements and calculating the Motor Optimality Score (MOS). RESULTS: Thirty-six infants were included. MOS at 3 months was associated with MCA-PI (rho 0.41, P = 0.04), UA-PI (rho -0.39, P = 0.047, and CPR (rho 0.50, P = 0.01). Infants with abnormal MOS had lower MCA-PI (P = 0.02) and CPR (P = 0.01) and higher UA-PI at the last measurement (P = 0.03) before birth. In infants with abnormal MOS, rcSO2 tended to be lower during the first 3 days after birth, and FTOE was significantly higher on the second day after birth (P = 0.04). Intraoperative and postoperative rcSO2 and FTOE were not associated with short-term neurological outcome. CONCLUSION: In infants with prenatally diagnosed CHD, the prenatal period may play an important role in developmental outcome. Additional research is needed to clarify the relationship between preoperative, intra-operative and postoperative cerebral oxygenation and developmental outcome in infants with prenatally diagnosed CHD.

Local anatomy around terminal ureter related to the anterior leaf of the vesicouterine ligament in radical hysterectomy.

OBJECTIVES: Radical hysterectomy is performed for invasive cervical cancer. In this surgery, separation of the anterior leaf and posterior leaves of the vesicouterine ligament (VUL) is important. We studied the local anatomy of the anterior leaf of the VUL, especially the branches of the umbilical artery from the view point of surgery and cadaver dissection. STUDY DESIGN: We observed the cervicovesical blood vessels and the connective tissue layer continued from umbilical artery and searched for the origin of the cervicovesical blood vessels in radical hysterectomy. We also dissected a formalin-fixed female cadaver, and observed the same points. RESULTS: After separation of the connective tissue of urinary bladder from the cervical fascia, we could discern the outline of the distal ureter near the ureterovesical junction. We separate the connective tissue of the so-called anterior leaf of the VUL enwrapping the ureter gently, and then the ureter with the connective tissue is completely rolled out laterally. We identified a cervicovesical vessel crossing over the ureter. We looked for the central side of the cervicovesical vessel and found that cervicovesical vessel was a branch of the superior vesical artery. And, during cadaver dissection, we found that the connective tissue and the branches of the superior vesical artery were similarly observed. CONCLUSIONS: We found the precise anatomy of the connective tissue layer from umbilical artery to urinary bladder and the superior vesical artery. Our procedure based on the precise anatomy obtained in this study is reasonable anatomically as a method for separation of the vesicouterine ligament during radical hysterectomy.

Failed umbilical artery catheterization and adverse outcomes in extremely low birth weight infants.

Purpose: To determine whether successful catheterization of the umbilical artery is associated with a reduced risk of death or neurodevelopment impairment among critically ill extremely low birth weight (ELBW) infants. Study design: A retrospective chart review was conducted between 2007 and 2014 at Stanford University for all ELBW infants that required intubation immediately after birth. The primary outcome was death or neurodevelopmental impairment at 18-22 months. We measured the association of successful umbilical artery catheterization with the primary outcome using multivariable logistic regression with adjustment for gestational age. Bayesian analysis was also performed due to small sample size. Results: Eighty-four ELBW infants met inclusion criteria. Successful umbilical artery catheterization occurred in 88% of infants and failed catheterization in 12%. Death or neurodevelopmental impairment occurred in 41% of infants with successful catheterization, compared to 60% of infants with failed catheterization of the umbilical artery, adjusted odds ratio 0.3, 95% confidence interval 0.1-1.3, p = .11. The Bayesian analysis indicated a 92% posterior probability of reduced death or neurodevelopmental impairment with successful catheterization and a 68% posterior probability of reduced death or neurodevelopmental by absolute risk difference of 20% or more, adjusted relative risk 0.74, 95% confidence interval 0.45-1.14. Conclusions: Among critically ill ELBW infants, successful catheterization of the umbilical artery compared to failed catheterization was not statistically significantly associated with the primary outcome. However, the Bayesian analysis indicated a high likelihood of benefit associated with successful umbilical artery catheterization.

Endovascular Management of a Large Persistent Sciatic Artery Aneurysm.

The persistent sciatic artery (PSA) is a remnant of the fetal circulatory system that is preserved in less than 0.1% of the population. Up to 60% of patients with this vascular anomaly will go on to development of a PSA aneurysm (PSAA), which can produce a variety of symptoms including neuropathy, claudication, and acute limb-threatening ischemia. Historical management is by open operation and interposition grafting, which can be highly morbid. We describe successful management of a large, symptomatic PSAA by endovascular stent grafting with intermediate term follow-up.

Catheter-Related Arterial Thrombosis in Neonates and Children: A Systematic Review.

Catheter-related arterial thrombosis (CAT) is increasingly recognized in children. Available data are scarce and based on expert opinions. This systematic review aimed to identify knowledge on paediatric CAT. Among 3,484 publications, 22 met inclusion criteria. Fourteen reported on CAT due to umbilical arterial catheter (UAC), two to extremity indwelling catheter (EIC), one to both and five to cardiac catheter (CC). The overall cumulative incidence of CAT was 21% (95% confidence interval [CI], 13-31) with a relative incidence of 20% (95% CI, 10-33) for UAC and 11% (95% CI, 3-21) for CC-related CAT. The incidence of EIC-related CAT ranged from 3.4 to 63%. Clinical presentation of CAT included symptoms of acute limb ischaemia (79%, 95% CI, 54-97), arterial hypertension (55%, 95% CI, 23-86) and congestive heart failure (28%, 95% CI, 7-53). Underlying conditions of UAC-related CAT included prematurity (70%, 95% CI, 31-98), respiratory distress syndrome (56%, 95% CI, 46-65), asphyxia (41%, 95% CI, 15-69), infection (32%, 95% CI, 13-55), persistent ductus arteriosus (28%, 95% CI, 13-45), meconium aspiration (16%, 95% CI, 8-25) and congenital heart disease (9%, 95% CI, 2-19). Congenital heart disease was the likely condition in EIC- and CC-related CAT. Antithrombotic treatment included thrombolysis (71%, 95% CI, 47-91), heparin (70%, 95% CI, 41-94) and thrombectomy (46%, 95% CI, 10-95) alone or in combination. Complete resolution rate of CAT was 82% (95% CI, 65-96). Long-term complications included arterial hypertension (26%, 95% CI, 0-66) and limb amputation (12%, 95% CI, 1-31). The overall all-cause mortality rate was 7% (95% CI, 2-14). In conclusion, CAT occurs at an increased incidence in neonates and children and is potentially associated with poor outcome. However, limited data are available on paediatric CAT. This systematic review identifies the rationale for further studies on CAT in paediatric patients.

Anatomical description of the umbilical arteries and impact of their ligation on pelvic and perineal vascular supply after cystectomy in women.

OBJECTIVE: In radical cystectomy, the surgeon generally ligates the umbilical artery at its origin. This artery may give rise to several arteries that supply the sexual organs. Our aim was to evaluate pelvic and perineal devascularisation in women after total cystectomy. PATIENTS AND METHODS: We carried out a prospective anatomical and radiological study. We performed bilateral pelvic dissections of fresh adult female cadavers to identify the dividing branches of the umbilical artery. In parallel, we examined and compared the pre- and postoperative imaging investigations [magnetic resonance imaging (MRI) angiography] in patients undergoing cystectomy for benign disease to quantify the loss of pelvic vascularisation on the postoperative images by identifying the occluded arteries. RESULTS: The anatomical study together with the radiological study visualised 35 umbilical arteries (n = 70) with their branching patterns and collateral arteries. The uterine artery originated from the umbilical artery in more than 75% of cases (n = 54) of the internal pudendal artery in 34% (n = 24) and the vaginal artery in 43% (n = 30). The postoperative MRI angiograms showed pelvic devascularisation in four patients. Devascularisation was dependent on the level of surgical ligation. In the four patients with loss of pelvic vascular supply, the umbilical artery had been ligated at its origin. CONCLUSION: The umbilical artery gives rise to various branches that supply the pelvis and perineum. If the surgeon ligates the umbilical artery at its origin during total cystectomy, there is a significant risk of pelvic and perineal devascularisation.

Cord Occlusion in Monochorionic Twins with Early Selective Intrauterine Growth Restriction and Abnormal Umbilical Artery Doppler: A Consecutive Series of 90 Cases.

OBJECTIVE: To describe perinatal outcomes achieved with cord occlusion (CO) in monochorionic twins with severe selective intrauterine growth restriction (sIUGR) and abnormal umbilical artery Doppler in the IUGR twin (types II and III). METHODS: We studied a consecutive series of 90 cases of sIUGR with abnormal Doppler treated with CO of the IUGR fetus. Abnormal Doppler was defined as continuous (type II, n = 41) or intermittent (type III, n = 49) absent/reversed end-diastolic flow. All cases presented at least one of the following severity criteria: gestational age (GA) <22 weeks, inter-twin estimated weight discordance >35%, reversed end-diastolic umbilical artery flow or ductus venosus pulsatility index >95th centile. We prospectively recorded pregnancy course and perinatal outcome. RESULTS: Median GA at surgery was 20.6 weeks and mean duration 22.4 min. Miscarriage (<24 weeks) occurred in 3.3% (3/90) and preterm delivery <32 weeks in 7.1% (6/84) of continuing pregnancies. GA at delivery was 36.4 weeks and neonatal survival of the larger twin was achieved in 93.3%. CONCLUSION: In a consecutive series studied by an experienced team, CO in monochorionic twins with severe sIUGR type II or III was associated with delivery >32 weeks in 92.9% and neonatal survival of the normal twin in 93.3% of pregnancies.

Transumbilical arterial embolization of a large dural arteriovenous fistula in a low-birth-weight neonate with congestive heart failure.

PURPOSE: The purpose of this study was to report transumbilical arterial embolization of a large dural arteriovenous fistula (AVF) in a low-birth-weight neonate with congestive heart failure (CHF). CASE PRESENTATION: A female neonate was delivered by cesarean section at 31 weeks of gestation. Her birth weight was 1538 g and Apgar scores were 6 at both 1 and 5 min. Because of dyspnea and retracted respiration immediately after birth, she required mechanical ventilation. Ultrasound revealed right cardiac overload and a large cystic mass at the posterior brain. Magnetic resonance imaging on day 1 showed a large dural AVF (dural sinus malformation with arteriovenous (AV) shunts) at the torcular herophili. Umbilical artery and vein catheterization were performed on the same day for neurointervention. CHF prompted emergency embolization on day 8. The transfemoral arterial route could not be used because of its small size and compromised femoral artery blood flow. Transumbilical arterial embolization shrank the AV shunts markedly, resulting in clinical improvement, thus requiring no further intervention. Follow-up angiography at 4 months confirmed no residual AVF. Her growth and development were normal at the last follow-up at age 4 years. CONCLUSION: This patient apparently was the lowest birth weight neonate with a large AVF successfully treated by embolization, which is usually performed through the transfemoral arterial route. The transumbilical arterial route is an alternative for neonates with birth weight <2000 g and very small femoral arteries.

Microwave Ablation for Twin-Reversed Arterial Perfusion Sequence: A Novel Application of Technology.

INTRODUCTION: Twin-reversed arterial perfusion sequence is a rare complication of monochorionic pregnancies that is characterized by the presence of an acardiac mass perfused by an apparently normal pump twin. The risk of death to the pump twin has led to a range of therapeutic interventions aimed at separating their vascular connection. We report a novel application of microwave ablation for vessel coagulation in the treatment of twin-reversed arterial perfusion sequence. MATERIAL AND METHODS: Microwave ablation has been adopted by surgical subspecialties as a superior energy source for vessel and tissue ablation as it creates heat without a circuit and has less thermal spread. We describe the use of a 2.45-GHz microwave system using a 1.8-mm antenna to coagulate the intra-abdominal portion of umbilical vessels of the acardiac mass. RESULTS: We report 6 cases of twin-reversed arterial perfusion sequence treated by microwave ablation. All patients were treated with microwave ablation with successful coagulation of intra-abdominal umbilical cord vessels of the acardiac mass with cessation of flow. DISCUSSION: Microwave ablation is an excellent energy source for vessel coagulation due to its thermal properties and can be used effectively in the treatment of twin-reversed arterial perfusion sequence.

Perinatal survival in cases of twin-twin transfusion syndrome complicated by selective intrauterine growth restriction.

OBJECTIVE: To evaluate the impact of selective intrauterine growth restriction (SIUGR) on monochorionic multiples treated with selective laser photocoagulation of communicating vessels (SLPCVs) for twin-twin transfusion syndrome (TTTS). METHODS: Perinatal survival was compared among TTTS patients with and without SIUGR. The TTTS + SIUGR group was defined as TTTS patients with donor twin weight <10th percentile. Multivariable logistic regression analyses identified factors associated with 30-day survival. RESULTS: Of 369 patients, 65% (N = 241) had TTTS + SIUGR. Thirty-day donor twin survival for the TTTS-only group was 84% versus 75% in the TTTS + SIUGR group (p = 0.0348). Stage III donor involved (stage III donor, donor/recipient) TTTS + SIUGR patients (N = 110) had 66% 30-day donor survival. Multivariable logistic regression demonstrated that the TTTS-only group was twice as likely to achieve donor survival compared to the TTTS + SIUGR group (OR 2.01, 95% CI 1.11-3.66, p = 0.0214). In the TTTS + SIUGR group, patients not classified as stage III donor-involved were twice as likely to achieve donor survival as stage III donor-involved patients (OR 2.02, 95% CI 1.10-3.71, p = 0.0226). CONCLUSIONS: SIUGR, present in two-thirds of TTTS patients, was a risk factor for decreased donor survival. Patients with donor SIUGR and umbilical artery persistent or reversed end-diastolic flow (Quintero Stage III Donor-involved) were especially at risk.

Using lateral radiographs to determine umbilical venous catheter tip position in neonates.

We aimed to assess the difference in measurement of the distance of the UVC tip from the diaphragm between (Anteroposterior) AP and lateral radiographs and to determine the reliability of the measurement of UVC tip distance from the diaphragm between the two views. A retrospective review of paired AP and lateral radiographs taken to assess UVC tip position was carried out in 25 infants was conducted and reliability analysis was carried out. There was a significant difference in the mean (SD) distance of the UVC catheter above the diaphragm between the AP and lateral radiographs: 8.7 (7.8) mm versus 11.6 (7.3) mm (p = 0.003) respectively. Measurements using lateral radiographs were more reliable (Intraclass correlation coefficient: 0.99 vs. 0.93). Inter-observer reliability analysis yielded similar results. Lateral radiographs are more reliable in measurement of UVC tip position and should be performed in conjunction with AP films to aid in determining UVC position.

A modified technique for umbilical arterial catheterization.

A modified technique for umbilical artery catheterization was assessed in babies in whom conventional method failed or if the cord was dry. Success rate attained with the modified technique was 90% (19/21). This modified technique could provide an easier and faster method for successful umbilical arterial catheterization.

Congenital umbilical arterio-venous malformation: a word of caution.

We report on an 18-month old girl who presented in good clinical shape with a pulsatile tumour in the umbilical area which had a shape and localization similar to that of an umbilical bowel hernia. The Doppler ultrasound of the umbilical tumour revealed a large arterio-venous vascular malformation with a haemodynamically significant blood shunting. Furthermore, the inferior caval vein and the hepatic veins were dilated. Computed tomography angiography revealed permeable umbilical veins and arteries communicating within a large dilated arterio-venous fistula. The growing tumour was excised without any perioperative complications. Further postoperative recovery was uneventful and the baby was discharged 10 days after surgery. We advocate careful antenatal ultrasound evaluation of these vascular malformations. Early surgical removal in newborns is vital in order to avoid severe complications.

[Interstitial laser coagulation in Twin Reversed Arterial Perfusion sequence]. / Mikroinwazyjna laserowa koagulacja naczyn wewnatrzbrzusznych w zespole odwróconej perfuzji tetniczej.

Twin Reversed Arterial Perfusion (TRAP) sequence complicates about 1% of all monochorionic twin pregnancies and about 1 to 35000 of all pregnancies. It involves an acardiac twin whose structural defects are incompatible with life, and an otherwise normal "pump" co-twin. As the blood flow in the acardiac twin is reversed, it keeps on growing owing to the oxygenated blood from the co-twin. Here we report a case of monochorionic, diamniotic twin pregnancy after ICS/-ET complicated with TRAP sequence, diagnosed at 11 weeks of pregnancy The unusual finding in this case was the residual heart in the so called acardiac twin. Gradually the normal twin developed signs of hemodynamic compromise. Reversed a-wave in ductus venosus was observed. The acardiac twin showed subcutaneous oedema. On 24 November 2011 a successful interstitial ultrasound-guided laser coagulation was performed at 16 weeks of gestation. 17G needle and 0.6 mm laser fibre were used. The needle was introduced into the pelvic region of the acardiac twin through the abdominal wall. A series of laser bursts lasting 5-10 seconds were fired, until cessation of blood flow in the pelvic vessels and umbilical cord of the acardiac twin was confirmed using colour Doppler. The course of the intervention was uneventful. Routine steroid therapy was administered at 27 weeks of gestation. At 32 weeks the patient was hospitalized and oral antibiotics were administered due to premature rupture of the membranes and suspicion of intrauterine growth retardation of the pump twin. The patient delivered spontaneously at completed 33 weeks of pregnancy (weight 1805g, Apgar 10). After the delivery a stage 2 intraventricular hemorrhage and jaundice were observed in the neonate. Phototherapy was administered and the mother and the child were eventually discharged from the hospital, both in good general condition. Since then, two more successful interstitial laser coagulations in TRAP sequence were performed in our institution. The essence of the treatment of TRAP sequence is cessation of the blood flow from the pump to the acardiac twin. Fetoscopic cord ligature or coagulation, and laser or radiofreqency ablations of the acardiac twin vessels, are the possible methods of intervention. The interstitial laser coagulation of the acardiac twin is less invasive than fetoscopic umbilical cord coagulation, as the outer diameter of the 17G needle is much smaller. A meticulous comparison of these methods would require a randomised study but at 16 weeks of MCDA twin pregnancy interstitial laser coagulation seems to be the method of choice. The outcome of the procedure and possible treatment options in case of TRAP together with the review of literature, are presented in the article.

Anti-inflammatory therapy in an ovine model of fetal hypoxia induced by single umbilical artery ligation.

Perinatal morbidity and mortality are significantly higher in pregnancies complicated by chronic hypoxia and intrauterine growth restriction (IUGR). Clinically, placental insufficiency and IUGR are strongly associated with a fetoplacental inflammatory response. To explore this further, hypoxia was induced in one fetus in twin-bearing pregnant sheep (n=9) by performing single umbilical artery ligation (SUAL) at 110 days gestation. Five ewes were administered the anti-inflammatory drug sulfasalazine (SSZ) daily, beginning 24h before surgery. Fetal blood gases and inflammatory markers were examined. In both SSZ- and placebo-treated ewes, SUAL fetuses were hypoxic and growth-restricted at 1 week (P<0.05). A fetoplacental inflammatory response was observed in SUAL pregnancies, with elevated pro-inflammatory cytokines, activin A and prostaglandin E(2). SSZ did not mitigate this inflammatory response. It is concluded that SUAL induces fetal hypoxia and a fetoplacental inflammatory response and that SSZ does not improve oxygenation or reduce inflammation. Further studies to explore whether alternative anti-inflammatory treatments may improve IUGR outcomes are warranted.

Balloon dilation of neonatal critical aortic valvar stenosis via the umbilical artery.

OBJECTIVES: To report our experience with balloon dilation of critical aortic valvar stenosis in neonates via the umbilical artery using currently available catheters. BACKGROUND: There is no agreement regarding the optimal vascular approach for balloon dilation of critical aortic valvar stenosis in neonates. METHODS: Since June of 2005, we have attempted to obtain umbilical arterial access within the first week after birth in all neonates with critical aortic valvar stenosis. In patients in whom umbilical artery access was obtained, we proceeded with an attempt at balloon dilation. RESULTS: We were presented with 5 patients with critical aortic valvar stenosis within the first week after birth, and the umbilical arterial approach was obtained in all, with effective relief of the stenosis achieved in 4. CONCLUSIONS: The umbilical arterial approach should always be considered for balloon dilation of neonatal critical aortic valvar stenosis. Using currently available catheters, the procedure is safe, simple, and effective even in patients weighing less than 2.5 kilograms. Further experience using this approach is warranted.