Sildenafil for microcystic lymphatic malformations of the head and neck: A prospective study.
BACKGROUND
Microcystic lymphatic malformations (LM) are congenital birth defects that can cause severe functional or esthetic deformity. At this time, several treatment interventions are possible, but there is no ideal therapy. A recently published article noted a coincidental improvement in microcystic LMs with the use of sildenafil for pulmonary hypertension, but conclusive and reproducible data is lacking regarding its efficacy.


RESULTS
A prospective study was conducted to examine the subjective and objective results associated with sildenafil use in the treatment of microcystic LMs. Patients under the age of 18 were enrolled, and after evaluation with pre-intervention magnetic resonance imaging (MRI) studies, each was given a 6-week course of sildenafil. Subjective outcomes were obtained, and postoperative MRIs were used to objectively quantify changes. Five patients between the ages of 4 and 11 were enrolled in the study. All patients had microcystic LMs of the head and neck. All patients had undergone previous treatment interventions utilizing various modalities, and each was now seeking treatment for functional and cosmetic purposes. All patients had minimal to no response from a subjective standpoint. Comparison of pre- and post-treatment MRIs also demonstrated minimal to no response.


CONCLUSIONS
The use of sildenafil for the treatment of microcystic lymphatic malformations did not have any appreciable effect on our selected population of pediatric patients who had been resistant to previous therapies. Further studies would be necessary to determine if other cohorts may benefit.
Division of Plastic Surgery, Baylor College of Medicine, United States. Electronic address: Jckoshy@bcm.edu.
John C
JC
Koshy
Division of Plastic Surgery, Baylor College of Medicine, United States. Electronic address: Bradley.Eisemann@bcm.edu.
Bradley S
BS
Eisemann
Division of Plastic Surgery, Baylor College of Medicine, United States. Electronic address: Nikhil.Agrawal@bcm.edu.
Nikhil
N
Agrawal
Department of Radiology, Texas Children's Hospital, United States. Electronic address: Pimpalwa@bcm.edu.
Sheena
S
Pimpalwar
Department of Otolaryngology, Texas Children's Hospital, 6550 Fannin St., Suite 2001, Houston, TX 77030, United States. Electronic address: otodoc@edmondsmd.com.
Joseph L
JL
Edmonds
D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D004334:Drug Administration Schedule; D005260:Female; D006257:Head; D006801:Humans; D044148:Lymphatic Abnormalities / Q000175:diagnosis / Q000188:drug therapy* / Q000601:surgery; D008279:Magnetic Resonance Imaging; D008297:Male; D009333:Neck; D058986:Phosphodiesterase 5 Inhibitors / Q000627:therapeutic use*; D011446:Prospective Studies; D000068677:Sildenafil Citrate / Q000627:therapeutic use*; D016896:Treatment Outcome
D058986:Phosphodiesterase 5 Inhibitors; D000068677:Sildenafil Citrate
Lymphatic malformation; Microcystic; Sildenafil