Ex utero intrapartum

BACKGROUND Agnathia-otocephaly complex (AOC) is a rare congenital malformation due to a first-branch arch disorder and has been considered lethal. However, milder variants of the isolated type of AOC have been reported as nonlethal. The ex-utero intrapartum treatment (EXIT) procedure is basically indicated for a fetus with a high risk of airway obstruction immediately after birth; it is not indicated for all AOC cases but is chosen to treat cases until the airway can be evaluated to achieve a better prognosis...

Advances in prenatal diagnostic techniques have enabled early detection of potentially correctable foetal anomalies. Here, we summarise recent developments in anaesthesia for foetal surgery. Types of foetal surgery include minimally invasive, open mid-gestational and ex-utero intrapartum treatment (EXIT) procedures. Foetoscopic surgery avoids hysterotomy, with risk of uterine dehiscence, preserving the possibility of subsequent vaginal delivery. Minimally invasive procedures are performed under local or regional anaesthesia; open or EXIT procedures are usually done under general anaesthesia...

Airway obstruction diagnosed antenatally in the fetus due to congenital high airway obstruction syndrome or nasopharyngeal masses are the primary indications for performing an operation on placental support. Prenatally diagnosed nasopharyngeal masses pose the risk of total airway obstruction upon delivery. Placental support utilises uteroplacental blood flow to facilitate an airway intervention on the fetus while maintaining oxygenation. These interventions must be completed in under 20 min due to amniotic fluid loss and uterine contractions...

OBJECTIVE: Previous studies have shown that ex utero intrapartum therapy (EXIT) is safe and feasible for newborns with congenital diaphragmatic hernia (CDH). This study reports our experience with EXIT in fetuses with CDH in an attempt to explore the efficacy of EXIT on the survival rate of this population. METHODS: A retrospective analysis of the clinical data of 116 children with CDH was conducted. The children were assigned to EXIT and non-EXIT groups. Propensity score matching (PSM) toward clinical data was performed, and the clinical characteristics and outcomes were compared...

Congenital high airway obstruction syndrome (CHAOS) is a rare condition that can be diagnosed antenatally by ultrasound. It is usually lethal without immediate intervention at delivery. A 24-year-old woman was diagnosed with fetal CHAOS at 27 weeks' gestation. The couple declined termination of pregnancy. A multidisciplinary team including obstetricians, geneticists, paediatric surgeons, neonatologists and anaesthetists was constituted to plan an ex utero intrapartum treatment (EXIT) procedure. After several simulations, a caesarean section was performed at 38 weeks' gestation under deep inhalational anaesthesia...

Lymphatic malformations (LMs) are uncommon congenital abnormalities of the lymphatic system. As more than half of these lesions develop in the head and neck, LMs can be life-threatening if associated with airway involvement. LMs necessitate a multidisciplinary treatment approach, frequently including surgery and sclerotherapy. We present a case report of a 32-week pre-term male infant with a massive cervicofacial LM necessitating delivery via ex-utero intrapartum treatment (EXIT). The patient was treated with numerous rounds of sclerotherapy, systemic sirolimus, and surgical debulking, but ultimately died at 4 months of age due to acute pulmonary hemorrhage, which may have been related to sirolimus due to the absence of any other associable organ involvement or derangement...

INTRODUCTION: Congenital high airway obstruction syndrome (CHAOS) is a rare condition that can progress to fetal hydrops and demise in utero or at birth unless interventions are undertaken to alleviate the tracheal obstruction. While the ex-utero intrapartum treatment (EXIT) procedure for airway stabilization is technically feasible, abnormal pulmonary development as a result of the antenatal obstructive process may result in severe postnatal respiratory complications. CASE PRESENTATION: We describe a case of CHAOS with secondary hydrops treated in utero at 24 0/7 weeks' gestation by fetoscopic tracheal decompression via laser perforation of the airway obstruction...

BACKGROUND: Antenatally diagnosed sacrococcygeal teratoma has been associated with risks of perinatal complications and death, especially when the foetus has symptoms of cardiac insufficiency, hydrops or anemia in utero ; however, the method of intervention remains controversial. CASE: A 25-year-old pregnant woman was found to have a cystic and solid tumor in the fetal sacrococcygeal region at 16 weeks of gestation. As the tumour grew, the mother developed polyhydramnios accompanied with gestational diabetes...

Neck tumors in newborns are very rare. Teratomas usually include all three germ cell layers, as well as tissues that are not native to the anatomic site of genesis. Teratomas of the head and neck make up a smaller percentage of congenital teratomas. Because of the external compression that oropharyngeal or neck masses produce, they can cause serious airway obstruction. In addition, the larynx or trachea may have an underlying lesion. We presented a mature, 1-day-old newborn with an isolated giant neck tumor and difficulty breathing...

OBJECTIVE: The anesthetic management of fetal operative procedures (FOP) is a highly debated topic. Literature on fetal pain perception and response to external stimuli is rapidly expanding. Nonetheless, there is no consensus on the fetal consciousness nor on the instruments to measure pain levels. As a result, no guidelines or clinical recommendations on anesthesia modality during FOP are available. This systematic literature review aimed to collect the available knowledge on the most common fetal interventions, and summarize the reported outcomes for each anesthetic approach...

BACKGROUND: Congenital 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...

Intact atrial septum (IAS), occurring in ∼10% of patients with hypoplastic left heart syndrome (HLHS), conveys significant neonatal morbidity and mortality. Perinatal interventions have been described, but outcomes remain poor. We present a fetus with HLHS with IAS who underwent immediate novel postnatal atrial appendage anastomosis, thus achieving rapid left atrial decompression. ( Level of Difficulty: Advanced. ).

Fetal cervical teratoma is a rare congenital neck tumor. Here, we report a case of a fetus with an anterior solid neck tumor that was confirmed to have an immature teratoma by histology. A duplication was found at chromosome 14q24.1-q24.3 of the fetus in chromosome microarray (CMA) and whole exome sequencing (WES), which was a copy number variation (CNV) and a probably new-onset. Ultrasound coupled with magnetic resonance imaging (MRI) can be considered to be a relatively reliable diagnostic tool, whereas ex-utero intrapartum therapy or resection of the tumor mass on placental support may improve the chances of the newborn's survival...

OBJECTIVE: In cases of suspected neonatal airway obstruction, the ex-utero intrapartum treatment (EXIT) procedure is used to secure the airway while a fetus remains on placental circulation. We report indications and outcomes from all EXIT procedures at a tertiary obstetric unit between 1997 and 2020. METHOD: Retrospective cohort study with data collected from maternal and neonatal medical records. RESULTS: Indications for EXIT procedures were micrognathia (n = 7), lymphatic malformations (n = 5), cervical teratomas (n = 4), goiters (n = 2), and intra-oral epulis (n = 1)...

This case report documents a rare and unique presentation of an oropharyngeal duplication cyst and subsequent neonatal airway management. A one-day-old premature female presented with postpartum respiratory distress requiring emergent intubation secondary to an oropharyngeal mass of the left tongue. After being stabilized and transferred to an academic center, imaging revealed a cystic lesion that was then marsupialized and drained by the otolaryngology team. Pathology demonstrated mature colonic tissue and was consistent with an enteric duplication cyst...

BACKGROUND: A fetal-onset cervical mass may cause postnatal airway obstruction, and ex utero intrapartum treatment (EXIT) to secure the airway while maintaining fetal-placental circulation may be life-saving. Malignant rhabdoid tumors (MRT) are highly aggressive tumors, and when they develop in utero, the prognosis is even worse, with almost no reports of survival beyond the neonatal period. Herein, we report a case of a primary cervical MRT and describe our treatment using EXIT for securing the airway, wherein the infant's life was saved...

Fetal airway obstruction that present during birth are challenging to manage. Cervical and thoracic mass may cause significant airway compression that leads to irreversible fetal morbidity and mortality. With the current advancement in prenatal diagnosis and assessment for surgical planning, EXIT has become an accepted, recognized and preferred option for safe delivery in complex head and neck masses. Ex utero intrapartum treatment (EXIT) is a life-saving procedure that provides time to establish an airway while maintaining uteroplacental circulation...

Vascular anomalies are rare lesions of diverse nature that may affect the head and neck region. Any mass in or around the upper airway has the potential to obstruct or compromise it. The absolute priority, before etiologic treatment, is the evaluation of the risk for the airway and its management. Prenatal diagnosis of an upper airway obstruction requires a planned delivery in a center having a specialized team experienced in managing a compromised feto-neonatal airway, and who could perform an ex-utero intrapartum treatment to secure the airway...

Ex-utero intrapartum treatment (EXIT) is a method of securing the airway of a fetus while maintaining umbilical circulation for newborns who are experiencing life-threatening airway obstruction. Cesarean section is completed only after ensuring the neonate's safety. However, managing the airway of a neonate while maintaining umbilical circulation is a major challenge for anesthesiologists. Anesthesiologists must understand the physiology of both the mother and fetus, and extensive discussions with obstetricians, pediatricians, otolaryngologists, and nursing staff prior to the procedure are essential...

INTRODUCTION: Without fetal or perinatal intervention, congenital high airway obstruction syndrome (CHAOS) is a fatal anomaly. The ex utero intrapartum treatment (EXIT) procedure has been used to secure the fetal airway and minimize neonatal hypoxia but is associated with increased maternal morbidity. CASE PRESENTATION: A 16-year-old woman (gravida 1, para 0) was referred to our hospital at 31 weeks gestation with fetal anomalies, including echogenic lungs, tracheobronchial dilation, and flattened diaphragms...