Fariha Sheikh1, Adesola Akinkuotu1, Oluyinka O Olutoye2, Sheena Pimpalwar3, Christopher I Cassady4, Caraciolo J Fernandes3, Rodrigo Ruano5, Timothy C Lee1, Darrell L Cass6. 1. Texas Children's Fetal Center, Texas Children's Hospital, Houston, TX; Department of Surgery, Baylor College of Medicine, Houston, TX. 2. Texas Children's Fetal Center, Texas Children's Hospital, Houston, TX; Department of Surgery, Baylor College of Medicine, Houston, TX; Department of Obstetrics and Gynecology, Baylor College of Medicine, Houston, TX; Department of Pediatrics, Baylor College of Medicine, Houston, TX. 3. Department of Radiology, Baylor College of Medicine, Houston, TX. 4. Texas Children's Fetal Center, Texas Children's Hospital, Houston, TX; Department of Radiology, Baylor College of Medicine, Houston, TX. 5. Department of Obstetrics and Gynecology, Baylor College of Medicine, Houston, TX. 6. Texas Children's Fetal Center, Texas Children's Hospital, Houston, TX; Department of Surgery, Baylor College of Medicine, Houston, TX; Department of Obstetrics and Gynecology, Baylor College of Medicine, Houston, TX; Department of Pediatrics, Baylor College of Medicine, Houston, TX. Electronic address: dcass@bcm.edu.
Abstract
PURPOSE: To determine long-term outcomes of fetuses with neck masses (NM), including functional and cosmetic results. METHODS: A retrospective review was performed of all fetuses evaluated for NM from November 2001 to March 2014. Quality of life (QOL) was evaluated using the validated PedsQL™ questionnaire. RESULTS: Of 35 fetuses evaluated, 9 died perinatally and 1 died late from tracheostomy complications. NM ranged from 4 to 20cm (mean, 9.1cm); 18 were delivered by EXIT. Of 25 surviving patients, 22 had mass resection, 7 requiring more than one procedure. Surviving patients with lymphatic malformations (LM) had the highest incidence of moderate and severe disfigurement and a higher rate of persistent/recurrent disease (100% vs. 31%, p=0.002) and cranial nerve dysfunction (50% vs. 0%, p=0.005) compared to those with non-LM diagnoses. Of 9 children attending school, 78% achieve grades of A/B's. QOL for 13 patients revealed a mean score of 83/100 for physical and 78/100 for psychosocial functioning. Median follow-up was 6 years (7 months-17 years). CONCLUSION: Unlike those with teratoma or other lesions, children with congenital cervicofacial LM are at high-risk for persistent disease, nerve dysfunction and moderate-severe disfigurement. There is substantial perinatal morbidity for fetuses with neck masses, but for those surviving, the long-term functional and cognitive outcomes are good.
PURPOSE: To determine long-term outcomes of fetuses with neck masses (NM), including functional and cosmetic results. METHODS: A retrospective review was performed of all fetuses evaluated for NM from November 2001 to March 2014. Quality of life (QOL) was evaluated using the validated PedsQL™ questionnaire. RESULTS: Of 35 fetuses evaluated, 9 died perinatally and 1 died late from tracheostomy complications. NM ranged from 4 to 20cm (mean, 9.1cm); 18 were delivered by EXIT. Of 25 surviving patients, 22 had mass resection, 7 requiring more than one procedure. Surviving patients with lymphatic malformations (LM) had the highest incidence of moderate and severe disfigurement and a higher rate of persistent/recurrent disease (100% vs. 31%, p=0.002) and cranial nerve dysfunction (50% vs. 0%, p=0.005) compared to those with non-LM diagnoses. Of 9 children attending school, 78% achieve grades of A/B's. QOL for 13 patients revealed a mean score of 83/100 for physical and 78/100 for psychosocial functioning. Median follow-up was 6 years (7 months-17 years). CONCLUSION: Unlike those with teratoma or other lesions, children with congenital cervicofacial LM are at high-risk for persistent disease, nerve dysfunction and moderate-severe disfigurement. There is substantial perinatal morbidity for fetuses with neck masses, but for those surviving, the long-term functional and cognitive outcomes are good.
Authors: Jayesh P Thawani; Michael J Randazzo; Nickpreet Singh; Jared M Pisapia; Kalil G Abdullah; Phillip B Storm Journal: J Neurol Surg Rep Date: 2016-07