title: Treatment of complex periorbital venolymphatic malformation in a neonate with a combination therapy of sirolimus and prednisolone.
abstract: Venolymphatic malformations (VLMs) are vascular anomalies consisting of both veins and lymph vessels. A 2-week-old newborn presented with large VLMs on the left forehead, temple, preauricular area, and orbit. Patient was at imminent risk for permanent vision loss due to a localized mass effect. Surgical excision or debulking was contraindicated due to its complexity and proximity to the left eye, and the patient failed to respond to the sildenafil treatment and sclerotherapy. Patient was subsequently started on oral sirolimus 0.8 mg/m(2) twice daily in combination with prednisolone 2 mg/kg daily. The patient had an excellent therapeutic outcome for 7 months with complete preservation of vision before treatment was discontinued. However, 2 months after the medical treatments were discontinued, her VLM rebounded. She responded to the combination therapy again after a failed treatment with the mTOR inhibitor alone. This case demonstrates that the sirolimus and prednisolone combination therapy could be beneficial for treatment of complex VLM intractable to other treatments.
authors.affiliation: Department of Dermatology, Stanford University School of Medicine, Stanford, California.
authors.forename: David
authors.initials: D
authors.lastname: Kim
authors.affiliation: Department of Dermatology and Pediatrics, Stanford University School of Medicine, Stanford, California.
authors.forename: Latanya
authors.initials: L
authors.lastname: Benjamin
authors.affiliation: Department of Dermatology, Stanford University School of Medicine, Stanford, California.
authors.forename: Ashley
authors.initials: A
authors.lastname: Wysong
authors.affiliation: Department of Radiology, Interventional Radiology, Stanford University School of Medicine, Stanford, California.
authors.forename: David
authors.initials: D
authors.lastname: Hovsepian
authors.affiliation: Department of Dermatology and Pediatrics, Stanford University School of Medicine, Stanford, California.
authors.forename: Joyce
authors.initials: J
authors.lastname: Teng
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D004359:Drug Therapy, Combination / Q000379:methods; D005123:Eye; D005260:Female; D005546:Forehead; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007231:Infant, Newborn; D044148:Lymphatic Abnormalities / Q000188:drug therapy*; D011239:Prednisolone / Q000627:therapeutic use*; D012008:Recurrence; D019233:Retreatment; D020123:Sirolimus / Q000627:therapeutic use*; D054079:Vascular Malformations / Q000188:drug therapy*
chemical_list: D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D011239:Prednisolone; D020123:Sirolimus
keywords: prednisolone; sirolimus; vascular malformation; venolymphatic malformation