The use of expandable metallic airway stent in a baby with tracheomalacia: A case report.

Tracheobronchomalacia is a rare condition in the pediatric age group which may be life-threatening when it occurs. The common form of tracheomalacia is congenital, presenting with wheezing and cough. We report a case of a 65-day-old baby who was treated with non-invasive mechanical ventilation due to respiratory distress since the day of birth. Tracheomalacia was diagnosed based on the physical examination and the thorax computerized tomography (CT) findings. Patient was initially treated with noninvasive positive pressure ventilation and thereafter, fitted with a tracheobronchial conical fully-covered self-expandable nitinol stent. After stent insertion and the respiratory situation of the patient improved, ventilatory weaning and extubation were possible. A careful selection of suitable patients, appropriate stent type and the site, where it has to be placed is mandatory for successful airway stenting. Also, children must be adequately followed-up to prevent the possible life-threatening complications after stent insertion.

Predictive Factors for Perinatal Outcomes of Infants Diagnosed With Micrognathia Antenatally.

INTRODUCTION: Advances in fetal imaging have allowed us to identify abnormalities previously not appreciated. With this study, we hope to identify factors predicting a difficult airway at birth and review the perinatal outcomes of these patients. METHODS: Sixteen patients with antenatally diagnosed micrognathia were reviewed from a tertiary care hospital database from 2011 to 2016. Jaw index (JI), amniotic fluid index (AFI), glossoptosis, gastric size, and oropharynx obliteration were assessed. The airway support required at birth, specialist team involvement, and outcomes were evaluated. RESULTS: Nine (56.3%) of 16 patients had JI <5th percentile, 3 (33.3%) of 9 had difficult intubation, 2 (22.2%) of 9 needed an emergency tracheostomy, and 1 (11.1%) of 9 died. Seven patients had polyhydramnios, 2 (28.6%) of 7 had difficult intubation, 2 (28.6%) of 7 required tracheostomy, and 1 (14.3%) of 7 died. Twelve patients had either JI <5th percentile or abnormal AFI, 5 (41.7%) of 12 had difficult intubation, 2 (16.7%) of 12 required tracheostomy, and 1 (8.33%) of 12 died. For the group without otolaryngology consultation, 8 (50%) of 16, 1 (12.5%) of 8 had difficult intubation and 1 (12.5%) of 8 died because airway was not secured after 45 minutes of resuscitation. CONCLUSION: Jaw index <5th percentile or abnormal AFI predicts a difficult airway. A multidisciplinary approach with otolaryngology involvement for airway intervention may be required at birth.

Treacher Collins Syndrome.

Treacher Collins syndrome is a rare genetic disorder of craniofacial development with a highly variable phenotype. The disorder displays an intricate underlying dysmorphology. Affected patients may suffer life-threatening airway complications and functional difficulties involving sight, hearing, speech, and feeding. Deformation of facial structures produces a characteristic appearance that includes malar hypoplasia, periorbital soft tissue anomalies, maxillomandibular hypoplasia, and ear anomalies. Management requires a specialized craniofacial team, as comprehensive care starts at birth and may require life-long follow-up. Standard craniofacial procedures for bony and soft tissue reconstruction are used. This article outlines current treatment strategies and future concepts for surgical management.

Ankyloglossum Superius Syndrome compromising a neonatal airway: Considerations in congenital oral airway obstructions.

We report the case of a 37-week old newborn presenting on day 1 of life with an apparent congenital fusion of the tongue to the hard palate, consistent with Ankyloglossum Superius syndrome. Physical exam along with endoscopy showed apparent fusion of the floor of the mouth to the anterior hard palate displacing the tongue into the nasal cavity and obstructing the oral airway. The child was nasotracheally intubated and brought to the operating room for lysis of the fusion under binocular microscopy. We review the literature on this rare condition and provide an algorithm for evaluating the neonatal airway in the setting of congenital oral abnormalities.

Heterotopic neuroglial tissue causing airway obstruction in the newborn.

BACKGROUND: Heterotopic neuroglial (brain) tissue is a rare cause of airway obstruction in newborns. Fewer than 30 cases have been reported in the English literature. Brain heterotopias can mimic more common congenital anomalies of the head and neck. OBJECTIVE: To review our experience in the diagnosis and treatment of children with heterotopic pharyngeal neuroglial tissue. DESIGN: Case series. SETTING: Tertiary care children's hospital. PATIENTS: Four newborns with airway obstruction caused by heterotopic neuroglial tissue. RESULTS: All patients were infants (3 full-term girls and a 32 weeks' gestation boy) who had airway obstruction in the newborn period. All patients underwent preoperative computed tomography and magnetic resonance imaging, which revealed a heterogeneous mass involving the pharynx, neck, and parapharyngeal space. Bony deformities of the skull base and mandible were present in all patients, although intracranial connection was absent. Multiple surgical procedures were performed in all 4 patients. Tracheotomy was performed in 2 patients, gastrostomy tube placement was required in 3, and a nasopharyngeal tube was used in 1. Combined cervicofacial and transoral approaches were used for resection, preserving vital structures. Histopathologic evaluation revealed mature glial tissue and choroid plexus-like structures. CONCLUSIONS: Heterotopic neuroglial tissue must be considered in the differential diagnosis of airway obstruction in the newborn. Management is surgical resection, with attention to vital structures and function-analogous to surgery for lymphangioma. Multiple surgical procedures might be necessary in the treatment of these patients.

Mandibular distraction osteogenesis for neonates with Pierre Robin sequence and airway obstruction.

Neonates with Pierre Robin Sequence (PRS) usually present with varying degrees of upper airway obstruction and difficulty feeding. Early treatment is important for such children in order to prevent impaired cognitive development resulting from hypoxemic episodes. Various procedures aimed at widening the pharyngeal space have been proposed, including prone position, tongue-lip adhesion, mandibular traction, non-invasive ventilation and palatal plates. Mandibular distraction osteogenesis (MDO) using external or internal devices has become increasingly popular as an alternative treatment option when other medical or surgical techniques do not prove to be satisfactory. This review summarizes current evidence on the effectiveness of MDO in infants with PRS. Because of a lack of studies comparing this treatment with other procedures, general recommendations cannot be drawn and treatment of infants with PRS still requires individualization.

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

BACKGROUND/PURPOSE: Congenital 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. METHODS: The 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. RESULTS: Twelve 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. CONCLUSION: Long-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.

Management of obstructive sleep apnea: role of distraction osteogenesis.

Distraction osteogenesis to expand the facial skeleton is an alternative to standard orthognathic surgery for selected patients with obstructive sleep apnea. For children with congenital micrognathia or midface hypoplasia, distraction osteogenesis allows large advancements without the need for bone grafting and with less risk of relapse. For later-onset obstructive sleep apnea, distraction osteogenesis may represent an alternative when acute bone movement is expected to be difficult (scarring from previous surgery or radiation therapy) or when the risk for inferior alveolar nerve damage is unacceptable (patients older than 40 years).

Heterotopic neuroglial tissue as a congenital laterocervical mass: a case report.

Heterotopic neuroglial tissue in the head and neck area is a rare clinical entity which can cause airway obstruction and feeding problems during the neonatal period. The case is presented of heterotopic neuroglial tissue as a congenital laterocervical and intraoral mass in a neonate.

Case series: Combined spinal epidural anesthesia for Cesarean delivery and ex utero intrapartum treatment procedure.

PURPOSE: To report the use of regional anesthesia and iv nitroglycerin to provide anesthesia and uterine relaxation for three Cesarean deliveries (CD) involving ex utero intrapartum treatment (EXIT) of potentially life-threatening airway obstruction in the newborn. CLINICAL FEATURES: Case 1--a 36-yr-old woman at 38 weeks' gestation was scheduled for an elective CD for fetal skeletal dysplasia and micrognathia. Case 2--a 34-yr-old woman at 35 weeks gestation had a fetal ultrasound revealing fixed neck flexion and micrognathia consistent with fetal arthrogryposis. Case 3--a 27-yr-old woman presented at 38 weeks gestation for CD for severe fetal micrognathia, with mandibular growth below the fifth percentile. For each case, a combined spinal epidural anesthetic was performed with 0.75% bupivacaine, fentanyl and morphine intrathecally followed by placement of a multiorifice epidural catheter. Prior to uterine incision patients received a loading dose followed by an iv infusion of nitroglycerin. Uterine relaxation was sufficient in all cases for delivery of the fetus, and allowed for evaluation by direct laryngoscopy and intubation while maintaining fetal-placental circulation. The surgical procedures were completed without incident. CONCLUSIONS: Anesthesia and uterine relaxation for CD and EXIT procedures can be safely provided with regional anesthesia and iv nitroglycerin.

Perinatal management of nasopharyngeal teratoma.

Nasopharyngeal teratoma is a rare benign entity, with most of the published literature represented by sporadic case reports. We present a recent case of a 23-year-old woman found to have elevated maternal serum alpha-fetoprotein and ultrasound findings of a fetal cystic mass in the mandibular region. The infant had a large nasopharyngeal teratoma protruding from the oral cavity making airway access extremely difficult. The presence of severe coexisting cardiac anomalies and respiratory disease led to death in the neonatal period. This report and the review of the literature stress the importance of recognizing suggestive prenatal data and preparing for perinatal management including maintenance of fetal oxygenation and aggressive early provision of an adequate airway.