Fetoscopic Diagnosis of Type IV Laryngeal Cleft in conjunction with Sever Left Congenital Diaphragmatic Hernia

Candace C. Style, MD, MS; Mariatu Verla, MD; Amy Mehollin-Ray, MD; Michael Belfort, MD, PhD; Oluyinka O. Olutoye, MBChB, PhD, FACS

Product Details
Product ID: ACS-5897
Year Produced: 2019
Length: 6 min.


Fetal tracheobronchoscopy is a new procedure performed as part of fetal endotracheal balloon occlusion for the management of severe congenital diaphragmatic hernia. This report presents the incidental finding of a type IV laryngeal cleft in a fetus diagnosed with severe congenital diaphragmatic hernia (CDH) during endoscopic tracheal balloon occlusion. This is a case presentation of a 30 yo G2P1001 with a fetal diagnosis of a left CDH at 20w2d gestation. MRI revealed a left intrapleural diaphragmatic hernia with an observed-to expected lung-to-head ratio of 29% and 31% of the liver herniated into the chest. Given the disease severity, the patient was offered fetoscopic placement of a tracheal balloon at 26w5d. During fetal bronchoscopy several attempts were made to identify an optimal location to place the balloon to occlude the distal trachea and promote lung growth. Upon close inspection, the posterior wall of the trachea was nonexistent and replaced by redundant esophageal tissue. This anomaly extended from below the vocal cords to the carina, consistent with a Type IV laryngeal cleft. Due to severity of both congenital abnormalities, parents opted for palliative care at 32 weeks. The infant was delivered at 33w4d gestations via spontaneous vaginal delivery. The infant expired at 24 minutes of life. A Type IV laryngeal cleft was confirmed via laryngoscope postnatally and at autopsy. To our knowledge, this is the first diagnosis of a laryngeal cleft prenatally. With improvements in fetal techniques, incidental diagnosis of rare conditions can aid in prenatal counseling.