Prenatal sonographic appearance of laryngeal atresia: A case report.

Detailed assessment of the larynx is not easy because of its complex structures and the associated technical difficulties. We performed sonography in multiple planes to assess the laryngeal anatomy and movements of a fetus with laryngeal atresia. The distended trachea ended abruptly with an echogenic non-structured larynx which showed shallow rapid "flutter-like" movements and up and down but not adduction- abduction movements during swallowing. Shadowing from the chin could be reduced by scanning through fluid in the oral cavity or between the transverse processes of vertebrae in a coronal plane.

Fetoscopy-Assisted Percutaneous Decompression of the Distal Trachea and Lungs Reverses Hydrops Fetalis and Fetal Distress in a Fetus with Laryngeal Atresia.

We present a case of prenatal hydrops secondary to congenital high airway obstruction syndrome (CHAOS) that was treated with fetoscopy-assisted needle decompression. A 22-year-old G3P2 woman presented after a 21-week ultrasound demonstrated CHAOS. The fetus developed hydrops at 25 weeks, characterized by abdominal ascites, pericardial effusion, and scalp edema. Fetal MRI showed complete obstruction of the glottis and subglottic airway, suggestive of laryngeal atresia. At 27 weeks, due to the progression of the hydrops, operative fetoscopy was proposed and performed. Fetal laryngoscopy confirmed fusion of the vocal cords and laryngeal atresia. The atretic segment was a solid cartilaginous block, preventing intubation. Using the fetoscope to stabilize the fetal head and neck, we performed ultrasound-guided percutaneous needle drainage of the cervical trachea through the anterior fetal neck. We removed 17 mL of viscous fluid from the lower trachea, resulting in immediate lung decompression. Two weeks later, ultrasound confirmed hydrops resolution. The patient was delivered and tracheostomy performed at 30 weeks via an ex utero intrapartum treatment (EXIT) procedure after progression of preterm labor. At 27 days of life, the infant was stable on minimal ventilator support. To our knowledge, this is the first successful report of an ultrasound-guided percutaneous tracheal decompression through the anterior neck of a fetus with CHAOS secondary to laryngeal atresia.

Locating the Level and Extent of Congenital High Airway Obstruction: Fluid in the Airway Tract as Reference Points.

Described here are a series of four cases of congenital high airway obstruction in the fetus. All of the patients presented in the second trimester and all had hydrops fetalis. Three cases had bilateral hyperinflated lungs, midline shift of heart, flattening or inversion of the diaphragm, and fetal ascites. Autopsy was performed in one of these three and showed laryngeal atresia. In one fetus, there was only a unilateral huge enlargement of the lung with mediastinal shift. On autopsy, this fetus had atresia of right main bronchus. All parents had terminated the pregnancy following the prenatal diagnosis. Laryngeal atresia is an extremely rare fetal anomaly with dismal prognosis. It is important to differentiate the condition from other lesions with a more favorable prognosis, such as congenital adenomatoid malformation of the lung. Much research is needed in the future to explore the therapeutic options, including fetoscopic intervention or transplantation of stem cell-derived airways.

Absent upper blind Pouch in a case of tracheo-esophageal fistula.

A common upper airway and digestive tract is a rare congenital anomaly that is usually fatal and its exact incidence is not known. It is a diagnostic challenge as it requires high index of suspicion. It should be considered in a neonate with respiratory distress in a non-vigorous baby requiring endotracheal intubation, which is difficult even in expert hand. We present a newborn with suspected tracheo-esophageal fistula that was diagnosed intraoperatively to have absent upper blind pouch of the esophagus and on autopsy found to have laryngeal atresia with absent vocal cords and a common aerodigestive tract continuing distally with trachea. The neonate was ventilated with endotracheal tube (ETT) placement which in retrospect we came to know that it was in the esophagus. The neonate also had associated multiple congenital anomalies of VACTERL association. The importance of teamwork between neonatologist, pediatric surgeon, anesthesiologist, and radiologist is highlighted for diagnosis and management of such rare cases.

A Case of Unsuspected Laryngeal Atresia With Comorbid Tracheoesophageal Fistula and Cardiac Defects.

Laryngeal atresia is a rare congenital condition that presents with hypoxia and failed intubation attempts at birth. When diagnosed prenatally, options exist to obtain airway access during delivery. However, postnatal diagnosis requires a high degree of clinical suspicion and the prompt initiation of surgical airway management in order to avoid morbidity and mortality.

Congenital High Airway Obstructive Syndrome (CHAOS) Survival of a Newborn with Laryngeal Atresia.

Congenital high airway obstructive syndrome (CHAOS) is a rare congenital anomaly, frequently caused by laryngeal or tracheal atresia, tracheal stenosis, and obstructing laryngeal cysts. This is a congenital malformation, often fatal, with an unknown prevalence. Laryngeal atresia is the most frequent cause. We report a case of an intrauterine diagnosis of CHAOS and ascites in a 17-week fetus delivered at 38 weeks of gestation without other associated malformations. A fetoscopic procedure was performed at 22 weeks of gestation. An attempt was made to perforate the affected area to ensure pulmonary fluid circulation and the ascites' resolution. After birth, a tracheostomy was performed. The patient was mechanically ventilated until 11 months of age, when she was discharged with no cerebral or other complications of immediate postnatal anoxia or episodes of respiratory arrest. A laryngotracheoplasty was performed at 2 years old, but decannulation was not possible due to certain complications. At 5 years old, a new surgical intervention was performed, which allowed decannulation 6 months later.

A rare case: Antenatally diagnosed congenital high airway obstruction syndrome.

Congenital high airway obstruction syndrome (CHAOS) is a rare life-threatening fetal condition resulting from obstruction of the upper fetal airway which may be partial or complete. Prenatal diagnosis is crucial as it usually results in stillbirth or death after delivery if unrecognized. We report a case of CHAOS that was diagnosed prenatally due to characteristic ultrasound features. We also briefly review literature in light of current management options.

Congenital airway anomalies.

Congenital airway anomalies (CAA) include a variety of conditions that cause respiratory distress in neonates and infants. These malformations occur at various anatomic levels and manifest in a wide spectrum of airway symptoms, with presentation significantly influenced by the level at which obstruction occurs as well as by the severity of obstruction. The prevalence of congenital airway malformations has been estimated to range between 0.2 and 1 in 10,000 live births. The most frequent CAA are laryngomalacia, bilateral vocal cord paralysis, subglottic stenosis, laryngeal webs, subglottic hemangioma, tracheomalacia, congenital tracheal stenosis, laryngotracheal cleft, and tracheal agenesis.

Surgical Management of Anterior Glottic Webs.

Congenital webs are rare and represent <5% of all congenital laryngeal anomalies. They are usually a partial laryngeal atresia rather than a true web, and present as a thick and fibrotic web with subglottic extension and associated subglottic stenosis. All patients with a congenital anterior glottic web should be evaluated for chromosome 22q11.2 deletion syndrome. Management strategies are mainly based on the severity of airway obstruction and the anatomical extension of the webs. Simple division of the web endoscopically may be adequate for rare thin webs, however, an open approach is usually warranted for thick glottic webs regardless of Cohen grades. Open repair can be either with keel placement or reconstruction of the anterior commissure.

CHAOS: A fetal autopsy report.

Congenital high airway obstruction syndrome (CHAOS) is a rare congenital malformation, which results from deficient recanalization of the upper airways. Laryngeal atresia is the most common cause, other etiologies being trachea atresia, laryngeal or tracheal webs, subglottic stenosis, obstructing laryngeal cysts, and laryngeal or tracheal agenesis. There is decreased clearance of the fluid produced by fetal lungs due to obstruction leading to increased intratracheal pressure and thereby secondary proliferative lung growth. The heart becomes compressed in the midline due to hyperexpansion of the lungs causing elevated intrathoracic pressure, decreased venous return, and fetal cardiac failure. This sequence causes ascites, placento-megaly, and eventually hydrops fetalis. We present a case of antenatal diagnosis of a fetus with CHAOS corroborated by fetal autopsy.

A case of laryngeal atresia accompanied by persistent pharyngotracheal ductus.

Laryngeal atresia is generally a fatal congenital anomaly with an incidence of 1: 50,000 births. This congenital anomaly is a condition of multifactorial inheritance, in which the fetus has a dilated trachea, enlarged echogenic lungs, an inverted or flattened diaphragm, fetal hydrops, and ascites. Diagnosis is usually made when there is failure to perform endotracheal intubation in a neonate with severe respiratory distress and absence of audible cry. Here, we present a very rare case of a newborn with laryngeal atresia who had respiratory distress and was sustained for the first few minutes of life using partial ventilation via a persistent pharyngotracheal duct. We would like to draw the attention of all physicians to this issue by reporting a rare fatal case of a newborn with a congenital presentation.

Laringeal atrezi, 50.000 dogumda bir görülen ve üst hava yolu tikanikligi ile giden ölümcül bir dogustan anomalidir. Çok etmenli kalitilir. Fetal ultrasonografide trakeada genisleme, akcigerlerde genisleme ve hiperekojenite, diyafragmada düzlesme ya da tersine dönme, hidrops ve asit saptanir. Dogumda agir solunum sikintisi olan yenidoganlarda endotrakeal entübasyonun basarilamamasi ve aglama çabasina ragmen ses duyulmamasi ile tani konulur. Bu yazida dogumdan sonra solunum sikintisi gelisen, ancak entübe edilemeyen, yasamin ilk dakikalarinda persistan faringotrakeal kanal yardimiyla kismi solunum yaparak hayatta kalabilen laringeal atrezili bir preterm olgu klinisyenlerin dikkatine sunuldu.

Antenatal Ultrasound Diagnosis of Congenital High Airway Obstruction Syndrome: A Case Report and Review of Literature.

Congenital high airway obstruction syndrome (CHAOS) is a rare life-threatening fetal condition resulting from obstruction of the upper fetal airway which may be partial or complete. Prenatal diagnosis is crucial as it usually results in stillbirth or death after delivery if unrecognized. We report a case of CHAOS that was diagnosed prenatally due to characteristic ultrasound features. We also briefly review literature in light of current management options.

Airway Anomalies.

This article reviews congenital anomalies involving the larynx and trachea, including congenital subglottic stenosis, laryngeal webs, laryngeal cleft, and tracheal stenosis. Presenting signs and symptoms, prevailing surgical repair techniques, and postoperative care are discussed.

CHAOS: síndrome de obstrucción congénita de las vías aéreas superiores / CHAOS: congenital high airway obstruction syndrome

Resumen El síndrome de obstrucción congénita de vías áreas superiores (CHAOS) es una condición que se caracteriza por la existencia de una obstrucción en las vías áreas altas en el feto, la cual puede ser parcial o completa. Comúnmente es una situación incompatible con la vida, por lo que su diagnóstico prenatal es importante considerando el pronóstico y los diferentes manejos prenatales y posnatales que existen. Presentamos un caso de CHAOS diagnosticado en la semana 21, con una breve revisión de la literatura sobre su diagnóstico, pronóstico y alternativas terapéuticas.

Abstract Congenital high airway obstruction syndrome (CHAOS) is a condition characterized by the existence of an obstruction of the fetal upper airways, which may be partial or complete. It is commonly incompatible with life, so its prenatal diagnosis is important due to the prognosis and the recently described pre and postnatal management options. We present a case of CHAOS in a pregnancy of 21 weeks with a brief review of the current literature about its diagnosis, prognosis and therapeutic alternatives.

Persistent Left Superior Vena Cava.

Persistent left superior vena cava (PLSVC) is a common cardiac anomaly associated with congenital heart diseases. A diagnosis of PLSVC usually warrants a detailed fetal echocardiography. Lesser known associations are the extra cardiac anomalies notably the upper airway and the gastrointestinal tract anomalies. We highlight here the importance of detailed fetal assessment for extra cardiac anomalies in addition to fetal echocardiography in fetuses diagnosed with PLSVC. We hereby present a preterm infant who presented with a triad of PLSVC, laryngeal atresia, and esophageal atresia.

CHAOS.

INTRODUCTION: Congenital high airway obstruction syndrome (CHAOS) is a rare, usually lethal abnormality characterized by complete or near-complete intrinsic obstruction of the fetal airway. Laryngeal atresia is the most frequent cause, but other etiologies include laryngeal or tracheal webs, laryngeal cyst, subglottic stenosis or atresia, tracheal atresia and laryngeal or tracheal agenesis. When antenatal diagnosis of possible upper airway obstruction is made, specific type of obstruction is rarely determined making the term CHAOS introduced by Hedrick et al in 1994 more appropriate. USG CHARACTERISTICS: Sonographic findings in CHAOS are characteristic and are secondary to high airway obstruction. The lungs are symmetrically enlarged, echogenic and homogenous. The distended lungs have mass effect on the diaphragm, which appears flattened or inverted, and the heart is displaced anteriorly in the midline. The heart often appears dwarfed by the surrounding enlarged lungs. DISCUSSION: The primary abnormality is an intrinsic obstruction of the upper airway. Normal lung development involves a continuous efflux of fluid from the fetal lungs. Laryngeal atresia/CHAOS stops the efflux of this fluid, and this retained fluid distends the alveoli with fluid giving the lungs voluminous echogenic appearance and inverting the diaphragm. Isolated airway obstruction without hydrops has a relatively favorable prognosis. CHAOS with associated anomalies and with early presentation of hydrops is an ominous sign with a high rate of fetal demise and a poor survival rate even with the ex utero intrapartum treatment (EXIT) procedure.

Management of severe congenital laryngeal webs - a 12 year review.

OBJECTIVE: The aim of this study was to investigate respiratory and voice outcomes after open surgery for severe congenital laryngeal web. METHODS: Included were all patients treated for severe congenital glottic web (Cohen type 3 and 4) between 2002 and 2014. Demographic data, symptoms, endoscopic findings, type of operation, outcome and complications were analyzed. Open surgery was performed with division of the web and enlargement of the subglottis by a laryngotracheal reconstruction or an extended partial cricotracheal resection. RESULTS: Fourteen patients were included. Information on the pre-operative voice was available in 11 patients. Aphonia was present in 6 patients (55%), dysphonia was severe in 1 (9%), moderate in 2 (18%) and mild in 2 (18%). Median age at operation was 19.3 months. As a first line treatment, laryngotracheal reconstruction was performed in 12 patients, and an extended partial cricotracheal resection in 2. Median follow-up was 9.5 months. All patients were successfully decannulated in a median time of 4 months after first surgery. Voice was improved in 10/11 (91%). Post-operatively, 3 patients (27%) had good voice. Dysphonia was severe in 2 (18%), moderate in 3 (27%) and mild in 3 patients (27%). CONCLUSION: Open surgery for Cohen type 3 and 4 glottic web allowed establishing a patent airway with successful decannulation in all cases. Quality of voice was improved in 91%, however, voice results were variable. In our opinion a delicate operative technique described in the paper is important for optimal voice results.

Airway compromise in the fetus and neonate: Prenatal assessment and perinatal management.

The fetus with a potentially obstructed airway can be identified on routine antenatal imaging. These cases should be referred to fetal care centers, which have the necessary expertise to fully evaluate and manage these fetuses and neonates appropriately. Complete airway obstruction may result in fetal hydrops and intrauterine demise. If a newborn infant has a compromised airway at delivery, the inability to secure its airway quickly may result in a hypoxic cerebral insult or death. In the most severely affected cases, prenatal, perinatal, or postnatal surgical intervention may be necessary. The timing of such an intervention will depend on the exact cause of the airway obstruction, other associated findings and the anticipated difficulty in establishing an airway at delivery. Fetal ultrasound and magnetic resonance imaging can differentiate between intrinsic and extrinsic airway obstruction, which allows for the optimal planning and management of the delivery and neonatal resuscitation.