Congenital Laryngeal Cyst: Benefits of Prenatal Diagnosis and Multidisciplinary Perinatal Management

Abstract

Congenital laryngeal cysts are rare and can cause upper airway obstruction if they are misdiagnosed. We describe a case of large vallecular cyst diagnosed at 33 weeks of gestation. The purpose of an early diagnosis is to establish a careful perinatal management. If the airway is compromised, a multidisciplinary medical team is necessary to perform emergency immediate tracheostomy. Elective delivery should be always carried out in a tertiary referral center.

Nomograms of the Fetal Neck Circumference and Area Throughout Gestation

“…The branchial arches contribute extensively to the growth of the face, oronasal cavities, palate, larynx, pharynx, and neck. 6 Congenital abnormalities of the fetal neck include those associated with fetal aneuploidies (cystic hygroma 7,8 ), the presence of embryologic remnants (thyroglossal duct and branchial cleft cysts [9][10][11][12] ), primary structural abnormalities (laryngeal cysts/ stenosis/atresia, [13][14][15][16][17] proximal esophageal atresia, 18 and bronchogenic cysts 19 ), functional abnormalities (goiters 20,21 ), benign tumors (lymphangioma, 22,23 hemangioma, 24 hemangiopericytoma, 25 and teratoma [26][27][28] ), malignant tumors (neuroblastoma 29 and rhabdomyoma 30,31 ), and an extensive array of syndromes (including Schinzel-Giedion, Yunis-Varon, and Escobar syndromes 32 ). In addition, subtle soft tissue changes of the upper posterior aspect of the fetal neck have been used in the second-trimester detection of trisomy 21 (nuchal thickness 33 Currently, a relative paucity of literature exists pertaining to precise sonographic measurements of the fetal neck.…”

Congenital laryngeal cyst causing repeated airway obstruction in a six-year-old girl

“…The common location for this kind of cyst is the aryepiglottic fold, followed by the vallecula, the laryngeal ventricle, and the subglottic region; however, vallecula is considered the most common location. [7] The laryngeal cyst in our case was located in the subglottic area of the larynx. Diagnosis can be made via the clinical examinations and symptoms, but the crucial role of laryngoscopy, as the gold standard method, should be noted.…”

Rapid development of severe respiratory distress due to a vallecular cyst in a 3‐day‐old infant

“…Flexible nasopharyngoscopy is a useful technique for accurate diagnosis in patients with persistent symptoms. The usefulness of ultrasonography, MRI and computed tomography is that the size is measurable 4,6 . Although computed tomography and MRI are more informative than ultrasonography, they may result in the delay of diagnosis and subject neonates to unnecessary irradiation and anesthesia.…”

“…VC may lead to serious morbidity and mortality if diagnosis and treatment are delayed. Although its precise incidence is unknown, 3 in some reports, the annual incidence of VC is 1.82 per 100 000 live births 4 . Prenatal diagnosis with ultrasonography and magnetic resonance imaging (MRI) is therefore important for neonatal management and outcome 4 .…”