Congenital high airway obstruction syndrome: natural history and management

Background

Congenital high airway obstruction syndrome (CHAOS) is a life-threatening condition with a poorly understood natural history.

Methods

A retrospective review of five patients with CHAOS between 1997 and 2002 was performed.

Results

All fetuses had large echogenic lungs, dilated airways, inverted diaphragms, and massive ascites. One fetus with a laryngeal cyst was terminated at 22 weeks. A twin fetus with findings suggestive of a tracheal web had progressive hydrops, which led to fetal demise. The remaining 3 patients delivered via the ex utero intrapartum treatment (EXIT) procedure survived. The first patient tolerated progressive hydrops for 12 weeks in utero. He had tracheal atresia but underwent laryngotracheoplasty successfully. He is the first long-term CHAOS survivor and is speaking at 5 years of age. The 2 patients with relatively stable lung volumes prenatally have laryngeal atresia with a pinpoint posterior laryngeal fistula. Their postnatal clinical courses were much more benign than the first survivor.

Conclusions

The prenatal natural history and postnatal course of CHAOS depends on whether the airway obstruction is complete. The EXIT procedure offers the potential for salvage of this otherwise lethal condition. Hydrops may be well tolerated prenatally for weeks with potential resolution if airway fistulization is present.     

Congenital high airway obstruction syndrome (CHAOS): Natural history,prenatal management strategies,and outcomes at a single comprehensive fetal center

PurposeCongenital high airway obstruction syndrome (CHAOS) is a devastating fetal condition of complete airway discontinuity resulting in significant hydrops and extreme lung hyperplasia. It is universally fatal with survival reported only in the rare spontaneous fistulization or EXIT intervention (Ex Utero Intrapartum Treatment). Even in these cases, mortality remains high, and current investigations are targeting prenatal interventions. This report describes our experience with management and fetal interventions for CHAOS, including laser laryngotomy.MethodsWe retrospectively reviewed all patients diagnosed with CHAOS at a single academic institution between 2006 and 2017.ResultsFifteen patients were identified. Eight had obstruction at the trachea and seven at the larynx. In the laryngeal obstructions, three expired shortly after birth, and one survived after spontaneous fistulization and subsequent EXIT to tracheostomy. The remaining three underwent in-utero treatment with laser laryngotomy. One had preterm premature rupture of membranes (PPROM), delivered 3 days post-operatively, and died. Two underwent EXIT to tracheostomy with one surviving to discharge and is currently 2 years old.ConclusionOur study demonstrates the outcomes of a large series of patients diagnosed with CHAOS. While mortality remains high, options for fetal intervention are being explored to allow alterations in the prenatal natural history and improve postnatal outcomes.Type of StudyRetrospective Treatment Study.Level of EvidenceLevel IV.     

Long-term outcomes after fetal therapy for congenital high airway obstructive syndrome

Background/PurposeCongenital high airway obstructive syndrome (CHAOS) is a rare and devastating condition that is uniformly fatal without fetal intervention. We sought to describe fetal treatment and long-term outcomes of CHAOS at a single referral center.MethodsThe medical records of patients with fetal CHAOS evaluated at our center between 1993 and 2011 were reviewed. Maternal history, radiographic findings, antenatal management, and postnatal outcomes were compared.ResultsTwelve fetuses with CHAOS were identified. Eleven had concomitant hydrops at diagnosis. Six were electively terminated, and 2 had intra- or peripartum demise. Four patients underwent fetal intervention. Two underwent delivery via ex utero intrapartum treatment (EXIT) procedure with tracheostomy placement only, and 2 underwent fetal bronchoscopy with attempted wire tracheoplasty followed by EXIT with tracheostomy at delivery. All 4 patients who underwent EXIT were alive at last follow-up. One patient was ventilator and tracheostomy free and feeding by mouth.ConclusionLong-term and tracheostomy-free survival is possible with appropriate fetal intervention even in the presence of hydrops. Fetal intervention earlier in pregnancy may improve long-term outcomes, but patient selection for intervention remains challenging. Magnetic resonance imaging may help select those patients for whom fetal intervention before EXIT delivery may be beneficial.     

Anesthesia for Ex Utero Intrapartum Treatment (EXIT procedure) in Fetus with Prenatal Diagnosis of Oral and Cervical Malformations: Case Reports

Background and objectivesFetus prenatally diagnosed with neck tumors, or with any other disease that obstructs the airways, should not be treated conventionally, as the assistant physician has to face two challenges right after the infant's delivery: the limited time to establish the access to the potentially difficult airways and the lack of anesthesia of the neonate in case of instrumentation of the airways. The ex utero intrapartum treatment, i.e., the EXIT procedure consists of maintaining the fetoplacental circulation during the cesarean section, until the airways of the fetus be secured.Case ReportsFemale patient, 37 years old, G3P2, 38 weeks pregnant, having polyhydramnios and fetus diagnosed with large cervical masses by prenatal ultrasound. A cesarean section was performed using the EXIT procedure to enable safe access to the infant's airways. After hysterotomy, the fetus was intubated by direct laryngoscopy. The neonate was immediately transferred to another operating room, where cervical tumor resection of the neck tumor and tracheostomy were successfully performed. Female patient, 27 years old, G3P1A1, 32 weeks pregnant, whose fetus was prenatally diagnosed with a large oral tumor. As the tumor obstructed the fetus’ airways, a tracheostomy was performed when the fetus underwent EXIT procedure. It was then possible to use direct laryngoscopy for neonate intubation. The fetus underwent tumor resection and was sent to the Neonatal Intensive Care Unit.ConclusionsReports describe the successful use of general anesthesia with isoflurane for cesarean delivery followed by the EXIT procedure in fetus diagnosed with tumors obstructing the airways.     

Report of a case: the anesthetic management for fetal tracheal intubation with intact uteroplacental circulation during cesarean section

We experienced a case of cesarean section in a patient with a fetus having a prenatally diagnosed huge cervical mass, which could cause airway obstruction immediately after delivery. The fetal cervical mass was confirmed at 19 gestational weeks, and amniocentesis was performed at 33 weeks. At 35 weeks, MRI showed the large mass that could disturb the airway patency after birth, and elective cesarean section was scheduled at 37 weeks. In the anesthetic management, anesthesia was maintained with isoflurane in oxygen, and ritodorine and nitroglycerin were used for obtaining uterine relaxation and keeping uteroplacental circulation. Fetal head was exposed from the uterine incision site and tracheal intubation was successfully performed. After confirming the neonatal oxygenation under manual ventilation, the baby was delivered. After delivery, the mass was diagnosed as cystic hygroma and he was maintained under mechanical ventilation in NICU. Five months later subtotal excision of the cervical cystic hygroma and tracheostomy were performed.     

Management of a prenatally diagnosed huge teratoma arising from the soft palate

Teratomas arising from the oral cavity are relatively rare and can cause life-threatening airway obstruction immediately after birth. We report a case of a huge teratoma arising from the soft palate detected prenatally. To save the patient, a caesarean section and ex utero intrapartum treatment (EXIT) were planned at 29 weeks of gestation. However, during EXIT, the patient's cardiopulmonary status deteriorated due to unstable cord blood flow secondary to uterine contractions. EXIT was abandoned, the patient was delivered and an emergency tracheotomy performed. The tumour was successfully excised 4 hours after tracheotomy. The tumour weighed 1,591 g and was 20 x 22 x 12 cm. The patient, a female, weighed 715 g. Histopathology showed Grade II teratoma. The postoperative course was uneventful and she is now 5 years old with no neurological sequelae. EXIT is indicated in patients who have a high risk for airway obstruction immediately after birth. However, if EXIT must be abandoned, as in this case, urgent tracheotomy is mandatory. Planned prevention of airway obstruction at delivery is indispensable for successful outcome and requires excellent coordination among obstetricians, neonatologists, anaesthesiologists and paediatric surgeons.     

Predicting the severity of congenital high airway obstruction syndrome

Background

Congenital high airway obstruction syndrome (CHAOS) is caused by complete or near-complete obstruction of the fetal airway. Obstruction sets in motion a sequence of events that can ultimately lead to fetal demise. However, on rare occasions in utero airway decompression occurs, reversing syndromic findings and improving the prognosis. In our relatively large series of CHAOS patients, we have observed a spectrum of clinical severity. The aim of this study was to identify the prenatal characteristics of CHAOS predictive of a milder postnatal course.

Methods

The medical charts of all fetuses observed at our institution with the diagnosis of CHAOS were reviewed for radiologic findings, delivery information, perinatal course, autopsy or discharge report, and long-term follow-up.

Results

Between 1996 and 2008, 12 fetuses with CHAOS were identified. Four fetuses had no evidence of hydrops on initial imaging. Of the 8 fetuses displaying hydrops, 3 were terminated, 2 died in utero, and 1 with multiple anomalies died at birth. Six fetuses were delivered via the ex utero intrapartum therapy procedure for attempted salvage, and 5 of the 6 survived the neonatal period including all 4 fetuses without hydrops. Serial prenatal imaging demonstrated less severe signs of CHAOS in 3 fetuses, and in 2 of them, direct laryngoscopy revealed a tiny opening in the airway. All 3 fetuses that showed improvement on serial imaging survived the neonatal period and were discharged home by 2-10 weeks of age.

Conclusions

Although the natural history of CHAOS is variable, trends in prenatal ultrasound findings are highly predictive of postnatal outcome and are a valuable guide to prenatal counseling.     

妊娠合并库欣综合征二例报告并文献复习

目的探讨罕见的妊娠合并库欣综合征病例的病因、临床表现、诊断和治疗。 方法回顾性分析2例我院收治的妊娠合并库欣综合征病例的临床资料，复习相关文献并予以讨论。例1：患者29岁，因"妊娠25周，四肢皮肤瘀点、瘀斑2个月，左小腿外伤半天"入住内分泌科。腹部超声检查发现左侧肾上腺区35 mm×29 mm低回声肿块。进一步完善各项检查后患者诊断为妊娠合并库欣综合征，并存在严重精神障碍。患者及家属要求手术治疗后终止妊娠。纠正低钾血症后转入泌尿外科行腹腔镜下左侧肾上腺肿瘤切除术，术中保留部分正常腺体。例2：患者28岁，因"妊娠22周，发现血糖升高1个月"入住我院内分泌科。腹部MRI检查发现左侧肾上腺区45 mm×59 mm占位。经进一步检查患者诊断为妊娠合并库欣综合征，并存在肝功能异常，考虑与患者皮质醇异常增高相关。患者及家属要求手术治疗后终止妊娠，遂转入泌尿外科行腹腔镜下左侧肾上腺肿瘤切除术。 结果2例手术均顺利完成。2例术后病理回报均为肾上腺皮质腺瘤。例1术后监测皮质醇水平低于正常，予小剂量糖皮质激素补充治疗。2例患者均术后2周引产终止妊娠。例1术后随访12个月，该患者术后小剂量糖皮质激素补充3个月后检查皮质醇恢复正常水平予停药，其后随访皮质醇均正常；患者精神障碍症状经过药物对症治疗后逐渐好转并恢复正常。例2术后已随访6个月，皮质醇及转氨酶术后均恢复正常。2例患者电解质及其他相关激素等随访检查均正常。 结论妊娠合并库欣综合征临床罕见，对孕妇及胎儿危害较大，需及时诊断。治疗时应综合评估孕妇及胎儿情况，选择合适的个体化治疗方案。     

The ex utero intrapartum treatment procedure: Looking back at the EXIT

Purpose

The ex utero intrapartum treatment (EXIT) procedure was developed originally for management of airway obstruction after fetal surgery, and indications have continued to expand for a variety of fetal anomalies. The authors review their single-institution experience with EXIT.

Methods

Retrospective review of all patients who underwent an EXIT procedure from 1993 to 2003 (n = 52) was performed. Variables evaluated include indication for EXIT, gender, gestational age at EXIT, birth weight, maternal blood loss, operative complications, operative time, and survival rate. Technique, personnel, and anesthesic management were reviewed.

Results

Long-term follow-up was available for all patients. Fifty-one of 52 patients were born alive; currently, 27 of 52 patients (52%) are alive. All deaths have been in patients with congenital diaphragmatic hernia. Forty-five patients underwent EXIT for reversal of tracheal occlusion for congenital diaphragmatic hernia. Of these patients, 30 underwent tracheal clip removal. Two patients had repair of tracheal injury from clipping at EXIT. Fifteen patients underwent bronchoscopy and tracheal balloon removal. Five patients underwent EXIT procedure for neck masses. Tracheostomy was performed in 3 of these patients. One patient was intubated successfully, and 1 patient underwent resection of the neck mass while on placental support. Two patients underwent EXIT procedure and tracheostomy for congenital high-airway obstruction syndrome. Average gestational age at delivery was 31.95 ± 2.55 weeks. Average birth weight was 1,895 ± 653 g. Average maternal blood loss was 970 ± 510 mL. Average operating time on placental support was 45 ± 25 minutes with a maximum of 150 minutes.

Conclusions

EXIT procedures can be performed with minimal maternal morbidity and with good outcomes. It is an excellent strategy for establishing an airway in a controlled manner, avoiding “crash” intubation or tracheostomy. Longer procedures on placental support allowing for definitive management of neck masses and airway obstruction have been realized. EXIT procedures have evolved from an adjunct to fetal surgery to a potentially life-saving procedure in fetuses with airway compromise at birth.     

Anesthetic management of the ex-utero intrapartum treatment (EXIT) procedure for congenital high airway obstruction syndrome (CHAOS)

We report an experience of anesthetic management of the ex-utero intrapartum treatment (EXIT) procedure performed in a fetus with congenital high airway obstruction syndrome (CHAOS) due to laryngeal atresia at 30 weeks' gestation. Anesthesia of the mother was induced with rapid sequence, and maintained with 3.5% sevoflurane in 100% oxygen and fentanyl before delivery. Two minimum alveolar concentration (MAC) sevoflurane provided excellent uterine relaxation without maternal hypotension. After hysterotomy, a sterile pulse oxymeter was placed on the fetus hand for monitoring fetal SpO2 and pulse rate, and a Doppler ultrasound transducer was applied to monitor fetal heart rate. Fentanyl (5 microg x dl(-1)) and pancuronium (0.2 mg x dl(-1)) were injected into the fetal upper arm in addition to transplacental anesthetic agents. The fetal heart rate and SpO2 were stable throughout the fetal manipulations, but the rise in SpO2 after initiating ventilation via tracheostomy was very slow. The uterine tone improved soon after discontinuing sevoflurane and oxytocin infusion was started after delivery. Surfactant administration before first ventilation is recommended in preterm babies undergoing EXIT procedure, and capnometer may be useful to confirm the adequate ventilation before cutting the umbilical cord.     

Ex utero intrapartum treatment in the management of giant cervical teratomas

PurposeThe purpose of this study is to present the outcome and technical details of the Ex Utero Intrapartum Treatment (EXIT) procedure performed in the management of the fetus with a giant cervical teratoma.MethodsA retrospective review of the medical records of patients undergoing the EXIT procedure between September 1995 and September 2010 was performed.ResultsEighty-seven EXIT procedures were performed. In 20% of cases (17/87), the indication was giant cervical teratoma. There were 10 females and 7 males. Polyhydramnios was present in 82%. Median gestational age at EXIT was 35 weeks (range, 30-39 weeks). Median birth weight was 2.5 kg (range, 1.7-3.7 kg). Access to the airway under placental support was established in all cases via direct laryngoscopy/bronchoscopy in 8 patients (47%) and via surgical exploration (tracheostomy or retrograde intubation) in 9 patients (53%). The mortality rate under placental support was zero. Seven patients had the tumors resected immediately after the EXIT, 6 patients had the resection later, and 4 patients died before resection. The neonatal mortality rate was 23% (4/17 patients). Patients who died had severe pulmonary hypoplasia that resulted from the upward traction by the giant cervical mass on the airway and compression of the lungs against the thoracic apex.ConclusionsWe conclude that the EXIT procedure continues to be the optimal delivery strategy for patients with prenatally diagnosed giant cervical teratomas and potential airway obstruction at birth. A thorough evaluation of the prenatal images and an experienced multidisciplinary team are key factors for an effective approach to the obstructed fetal airway.     

Neuraxial anesthesia in ex utero intrapartum therapy for parturients with fetal congenital diaphragmatic hernia: a prospective observational study

BackgroundCongenital diaphragmatic hernia (CDH) is characterized by defects in the fetal diaphragm and thoracic herniation of the abdominal viscera. The ex utero intrapartum treatment (EXIT) procedure is used to establish the fetal airway while on placental support. These EXIT procedures are commonly performed under general anesthesia, which increases maternal bleeding and the risk of insufficient placental perfusion subsequently. This study investigated the feasibility of performing neuraxial anesthesia for the EXIT procedure for fetal congenital diaphragmatic hernia to improve outcomes.MethodsParturients with fetal CDH who underwent an EXIT procedure between January 2019 and May 2021 in our institution were recruited. Variables evaluated included gestational age, surgical time, intra-operative blood loss, peri-operative hemoglobin, maternal complications, fetal lung-to-head ratio, time on placental bypass, and postnatal outcome.ResultsTwenty-two cases were included. All procedures were performed under neuraxial anesthesia. The median gestational age at the time of the EXIT procedure was 37 weeks. The median estimated blood loss was 200 mL. There was no report of an adverse maternal event. The placental bypass time was 142.9 ± 72.6 s, and access to the airway was successfully established within the bypass time. Twenty-one neonates reached an Apgar score of 9 at 5 min. In the first two hours after birth, the average pH of neonatal peripheral arterial blood was 7.35 ± 0.07 (n=19), and lactate level 1.85 ± 0.71 mmol/L (n=19).ConclusionsIn the EXIT procedure to establish an airway for fetal CDH, neuraxial anesthesia proved a feasible technique for maternal anesthesia.     

Perinatal management of congenital oropharyngeal tumors: The ex utero intrapartum treatment (EXIT) approach

Purpose

To present our experience in the perinatal management of fetuses with large oropharyngeal tumors by ex utero intrapartum treatment (EXIT).

Methods

We performed a retrospective chart review of all patients with congenital oropharyngeal tumor who underwent an EXIT procedure between May 2006 and June 2012.

Results

Four patients were included in the series, three females and one male. The diagnoses were epignathus (n = 2) and congenital epulis (n = 2). Three EXITs were done at term and one at late preterm due to premature rupture of membranes. Median maternal time under anesthesia was 185 min (range: 166–281) and median maternal operative time was 99 min (range: 85–153). Median maternal blood loss was 550 ml (range: 350–2000); one mother required a blood transfusion. Mean maternal hospital stay was 4 days. Median hysterotomy-to-cord clamp time was 24 min (range: 18–66). Mean fetal birth weight was 2.7 kg (range: 2.4–3). The airway was successfully accessed and secured under placental circulation in all cases. In the two patients with congenital epulis the tumors were resected at the base of their pedicles and the airway accessed via direct laryngoscopy before the umbilical cord was clamped. One patient with epignathus underwent a retrograde tracheal intubation under placental circulation and had the tumor resected thereafter. The second patient with epignathus had a tracheostomy done under placental circulation and then had tumor debulking immediately after the EXIT. The maternal morbidity was minimal and there were no mortalities.

Conclusions

We conclude that the EXIT procedure is the ideal delivery strategy for fetuses with prenatally diagnosed oropharyngeal tumors and potential airway obstruction at birth. Patients with prenatally diagnosed oropharyngeal tumors should be promptly referred to a fetal treatment center with a dedicated multidisciplinary team and EXIT capabilities.     

The EXIT procedure: experience and outcome in 31 cases

Background: The EXIT (ex utero intrapartum treatment) procedure, although initially designed for reversal of tracheal occlusion in fetuses with congenital diaphragmatic hernias (CDH), has been adapted to treat a variety of fetal conditions. Methods: A retrospective chart review of all consecutive EXIT procedures since 1996 was conducted. Results: Thirty-one women underwent the EXIT procedure, with an average maternal age of 29 years (range, 20 to 38), and average gestational age of 34 weeks (range, 29 to 40). The indication was airway obstruction from fetal neck mass in 13, and reversal of tracheal occlusion from in utero clipping in 13. Singular indications included an EXIT-to-ECMO (extracorporeal membrane oxygenation) procedure for a fetus with CDH and a cardiac defect (n = 1), congenital high airway obstruction syndrome (CHAOS, n = 1), resection of a very large congenital cystic adenomatoid malformation of the lung (CCAM) on uteroplacental bypass (n = 1), unilateral pulmonary agenesis (n = 1), and thoracoomphalopagus conjoined twins. The mean duration on uteroplacental bypass (from uterine incision to umbilical cord clamping) was 30.3 [plusmn] 14.7 minutes (range, 8 to 66). No fetus experienced hemodynamic instability during uteroplacental bypass as recorded by fetal heart rate (FHR), pulse oximeter, and fetal echocardiography, except for one instance of reversible bradycardia from umbilical cord compression. The mean FHR and fetal saturation were 153.0 [plusmn] 38.5 beats per minute and 71.2% [plusmn] 19.9%, respectively. Five fetuses required a tracheostomy. Only 1 death occurred during an EXIT procedure because of inability to secure the airway secondary to extensive involvement by a lymphangioma. The average cord pH and pCO₂ were, respectively, 7.20 [plusmn] 0.11 and 63.2 [plusmn] 14.6. Two maternal complications occurred: bleeding from a hysterotomy site and dehiscence of an old hysterotomy scar noticed at a subsequent cesarean section. The average maternal blood loss was 848.3 [plusmn] 574.1 mL. Conclusion: The EXIT procedure was used successfully to ensure uteroplacental gas exchange and fetal hemodynamic stability during a variety of surgical procedures performed to secure the fetal airway or ensure successful transition to postnatal environment.     

Prenatal diagnosis and postnatal resection of intraabdominal enteric duplications

Purpose

The high definition provided by modern imaging techniques allows the delineation of fetal anatomy with unprecedented accuracy. The early prenatal detection of enteric duplications is possible and facilitates a prompt postnatal treatment strategy, thereby, decreasing the risk of potential complications.

Materials and Methods

We retrospectively reviewed the medical records of all patients treated at our institution with a prenatally diagnosed intraabdominal enteric duplication between 2001 and 2009.

Results

Eighteen patients were included in the series. The diagnosis was made as early as 20 weeks of gestational age and included gastric duplications (3 cases), jejunoileal duplications (11 cases), and duodenal duplications (4 cases). None of the patients had prenatal complications. Two patients had associated intestinal malrotation, whereas the remaining 16 had no other malformations. Postnatally, 14 patients had an uneventful clinical course until the time of surgery, 3 patients had complications within the first 2 weeks of life that required emergency surgery, and 1 patient who had been lost to follow-up presented with duodenal obstruction at 3 years of age. Four patients underwent laparoscopic resection.

Conclusion

Because intraabdominal enteric duplications can cause complications, we believe that prenatally diagnosed enteric duplications should be resected after birth.     

The impact of 3-dimensional ultrasonography on perinatal management of a large epignathus teratoma without ex utero intrapartum treatment

We report a case of epignathus teratoma diagnosed at 22 weeks of gestation in which 3-dimensional ultrasound (3DUS) was useful to plan perinatal management. A significant enlargement of the tumor, associated with polyhydramnios and preterm labor, was observed at 35 weeks of gestation. After amniotic fluid evacuation, 3DUS was performed in the presence of pediatricians, obstetricians, and otolaryngologists. Three-dimensional ultrasound revealed that great part of the tumor was located outside the fetal mouth and anterior to fetal mandible, suggesting that the newborn could breathe spontaneously by nasal via. A cesarean section with longitudinal hysterotomy was performed at 36 weeks followed by an immediate extirpation of the tumor and the intubation of the newborn. The management of this rare case illustrates that the ex utero intrapartum treatment (EXIT) procedure is not always necessary in this situation. Besides, the actual prenatal goal consists on carefully selecting fetuses with epignathus teratoma that will need the EXIT procedure from those that will not. Three-dimensional ultrasound and magnetic resonance imaging in association with 2DUS can be helpful in this prenatal selection.     

Anesthetic management for cesarean delivery in a parturient with exacerbated hemophagocytic syndrome

Hemophagocytic syndrome is an uncommon disease characterized by cytokine dysfunction and uncontrolled hemophagocytosis. It arises rarely during pregnancy, in which case maternal and fetal mortality are relatively high. It has some similarities with HELLP syndrome. Poor maternal condition increases the risk of preterm labor and the possibility of emergency cesarean delivery in non-optimal conditions, presenting a great challenge to the anesthesiologists. We report a 28-year-old primigravida with the onset of hemophagocytic syndrome and cyopenia at 23 weeks of gestation. A further exacerbation at 28 weeks of gestation brought on preterm labor. General anesthesia was provided successfully for cesarean delivery. The patient recovered completely after this episode. We suggest that early diagnosis, multi-disciplinary intervention, pre-operative correction of the hematological abnormalities, general anesthesia and close postoperative monitoring are necessary.     

Klippel-trenaunay syndrome associated with splenomegaly: Report of a case

We report herein the case of a 19-year-old woman, diagnosed as having Klippel-Trenaunay syndrome at the age of 3 years, who presented to our hospital with severe abdominal pain. Abdominal computed tomography revealed splenomegaly, ascites, and paracentesis confirming an intrabdominal hemorrhage. Thus, an emergency laparatomy was performed for a suspected splenic rupture, and 2.5L of blood was drained from the abdominal cavity. Splenomegaly was confirmed, and a splenectomy was performed. The patient's postoperative course was complicated by disseminated intravascular coagulation, but she recovered and was discharged 3 weeks following surgery. Pathological examination of the spleen suggested that the splenomegaly was caused by high venous pressure due to splenic vein stenosis. To our knowledge, this is the first reported case of Klippel-Trenaunay syndrome associated with marked splenomegaly.     

Anesthetic management of an ex utero intrapartum treatment procedure: a novel balanced approach

Ex utero intrapartum treatment (EXIT) procedures are therapeutic interventions for fetuses with life-threatening airway abnormalities and/or other prenatally diagnosed congenital malformations requiring immediate neonatal extracorporeal membrane oxygenation support. Although certain anesthetic goals are common among EXIT procedures, many different approaches to their management have been described in the literature. Herein, we present a novel anesthetic approach to an EXIT procedure for fetal micrognathia and retrognathia. We also review the indications and anesthetic considerations for these procedures and highlight the need for multidisciplinary collaboration to optimize clinical outcomes.     

Senning operation for transposition of great arteries in a premature baby

A male baby was delivered by emergency cesarean section due to fetal distress at 30 weeks of gestational age with a birth weight of 813 g. By fetal echocardiography, the patient had been diagnosed with transposition of great arteries (type 1). Early two-staged arterial switch operation was planned after 34 gestational age avoiding intracranial hemorrhage under cardiopulmonary bypass. At 19 days of life, vegetation was revealed on the pulmonary valve by echocardiography, so he was diagnosed as infectious endocarditis. Cefotaxime and gamma-globulin were given intravenously for 4 weeks. While waiting for the increase in the body weight, desaturation from chronic respiratory distress syndrome was exacerbated. At 8 months old, urgent Senning operation was performed to improve desaturation. The patient was discharged at 20 post operative day. We conclude that Senning operation can be feasible operation in such a complicated case.