title: Efficacy and safety of oral sildenafil in treatment of pediatric head and neck lymphatic malformations.
abstract: CONCLUSIONS
  Low dose oral sildenafil citrate tablets are safe and effective to decrease the lesions volume in some LMs children, especially with macrocystic lesion or mixed type.
  
  
  OBJECTIVE
  To evaluate the efficacy and safety of oral sildenafil in treatment of pediatric head and neck lymphatic malformations (LMs).
  
  
  METHODS
  In this open-label study, 21 children diagnosed with LMs were given oral sildenafil and stopped at week 24. Volume change of lesions compared with baseline was used to evaluate therapeutic outcome. The improvement of various symptoms and the adverse effects of sildenafil were recorded during the follow-up period.
  
  
  RESULTS
  Eight patients (38.0%) showed excellent improvement (>75-100%), one patient (4.8%) had good (>50-75%), two patients (9.6%) had fair (>25-50%), three patients (14.3%) had minimal (0-25%), and seven patients (33.3%) had no improvement. The snoring or stridor completely disappeared in two subjects, although there was no apparent decrease in LMs size. Moreover, all three patients with macrocystic lesions displayed a positive response to sildenafil treatment, followed by mixed type (9/12, 75%). Only two out of six patients with microcystic lesions had minimal clearance. Only four patients showed transient mild diarrhea, there was no other adverse effect in this series.
authors.affiliation: a Department of Otolaryngology, Head and Neck Surgery , Beijing Children's Hospital, Capital Medical University , Beijing , PR China.|b Beijing Key Laboratory for Pediatric Diseases of Otolaryngology, Head and Neck Surgery , Beijing Pediatric Research Institute, Beijing Children's Hospital, Capital Medical University , Beijing , PR China.
authors.forename: Shengcai
authors.initials: S
authors.lastname: Wang
authors.affiliation: a Department of Otolaryngology, Head and Neck Surgery , Beijing Children's Hospital, Capital Medical University , Beijing , PR China.|b Beijing Key Laboratory for Pediatric Diseases of Otolaryngology, Head and Neck Surgery , Beijing Pediatric Research Institute, Beijing Children's Hospital, Capital Medical University , Beijing , PR China.
authors.forename: Jie
authors.initials: J
authors.lastname: Zhang
authors.affiliation: a Department of Otolaryngology, Head and Neck Surgery , Beijing Children's Hospital, Capital Medical University , Beijing , PR China.|b Beijing Key Laboratory for Pediatric Diseases of Otolaryngology, Head and Neck Surgery , Beijing Pediatric Research Institute, Beijing Children's Hospital, Capital Medical University , Beijing , PR China.
authors.forename: Wentong
authors.initials: W
authors.lastname: Ge
authors.affiliation: a Department of Otolaryngology, Head and Neck Surgery , Beijing Children's Hospital, Capital Medical University , Beijing , PR China.|b Beijing Key Laboratory for Pediatric Diseases of Otolaryngology, Head and Neck Surgery , Beijing Pediatric Research Institute, Beijing Children's Hospital, Capital Medical University , Beijing , PR China.
authors.forename: Yuanhu
authors.initials: Y
authors.lastname: Liu
authors.affiliation: b Beijing Key Laboratory for Pediatric Diseases of Otolaryngology, Head and Neck Surgery , Beijing Pediatric Research Institute, Beijing Children's Hospital, Capital Medical University , Beijing , PR China.
authors.forename: Yongli
authors.initials: Y
authors.lastname: Guo
authors.affiliation: c Department of Radiology, Head and Neck Surgery , Beijing Children's Hospital, Capital Medical University , Beijing , PR China.
authors.forename: Yue
authors.initials: Y
authors.lastname: Liu
authors.affiliation: c Department of Radiology, Head and Neck Surgery , Beijing Children's Hospital, Capital Medical University , Beijing , PR China.
authors.forename: Ning
authors.initials: N
authors.lastname: Wang
authors.affiliation: a Department of Otolaryngology, Head and Neck Surgery , Beijing Children's Hospital, Capital Medical University , Beijing , PR China.|b Beijing Key Laboratory for Pediatric Diseases of Otolaryngology, Head and Neck Surgery , Beijing Pediatric Research Institute, Beijing Children's Hospital, Capital Medical University , Beijing , PR China.
authors.forename: Yuzhu
authors.initials: Y
authors.lastname: He
authors.affiliation: a Department of Otolaryngology, Head and Neck Surgery , Beijing Children's Hospital, Capital Medical University , Beijing , PR China.|b Beijing Key Laboratory for Pediatric Diseases of Otolaryngology, Head and Neck Surgery , Beijing Pediatric Research Institute, Beijing Children's Hospital, Capital Medical University , Beijing , PR China.
authors.forename: Jun
authors.initials: J
authors.lastname: Tai
authors.affiliation: a Department of Otolaryngology, Head and Neck Surgery , Beijing Children's Hospital, Capital Medical University , Beijing , PR China.|b Beijing Key Laboratory for Pediatric Diseases of Otolaryngology, Head and Neck Surgery , Beijing Pediatric Research Institute, Beijing Children's Hospital, Capital Medical University , Beijing , PR China.
authors.forename: Xin
authors.initials: X
authors.lastname: Ni
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D044148:Lymphatic Abnormalities / Q000188:drug therapy*; D008297:Male; D058986:Phosphodiesterase 5 Inhibitors / Q000627:therapeutic use*; D011446:Prospective Studies; D000068677:Sildenafil Citrate / Q000627:therapeutic use*
chemical_list: D058986:Phosphodiesterase 5 Inhibitors; D000068677:Sildenafil Citrate
keywords: Lymphatic malformations; macrocystic lymphatic malformations; sildenafil