New Use of Rapamycin Stent in Non-Responding Facial Lymphatic Malformation
Journal Title: Biomedical Journal of Scientific & Technical Research (BJSTR) - Year 2019, Vol 18, Issue 4
Abstract
Introduction: Vascular anomalies represent a diagnostic and therapeutic difficulty. A lymphatic malformation (LM) corresponds to a low-flow vascular malformation. Sclerotherapy is the preferred treatment. Recently, angiogenesis inhibitors such as Rapamycin have been used with promising results. Case Report: A pediatric patient presented with a large facial LM and poor response to Sildenafil and sclerotherapy. After a persistent enlargement of the lesion, with painful ocular occlusion a new sclerotherapy was performed obtaining partial improvement. As a rescue treatment an IRS was installed. The patient achieved a rapid and evident response, with better ocular aperture and pain control. Discussion: LM correspond to vasculogenesis disorders with persistent growth over time. They can present high morbidity. The complications in the facial area are infection, hemorrhage, exophthalmos, and amblyopia. Rapamycin is a potent angiogenesis inhibitor. Rapamycin stents have been safely used in cardiology. They provide local concentration with less adverse effects. There are no reports on their use in lymphatic malformation. The patient had a favorable and persistent response. Conclusion: IRS should be considered as a therapeutic alternative in severe nonresponding lymphatic malformation. The necessity of a multidisciplinary approach is fundamental.Vascular anomalies represent a diagnostic and therapeutic difficulty. The classification system of the International Society for the Study of Vascular Anomalies (ISSVA) divides them in two groups: tumors and vascular malformations based on clinical, genetic and pathological findings [1]. Lymphatic malformations (LM) -low flow vascular malformations- are classified as macrocystic, microcystic or mixed [1-3]. The incidence of LM reaches 1.2–2.8 per 1,000 births and 2.8 patients per 100,000 hospital admissions [1]. They are located in the head and neck area in 70% of the cases, and their growth is proportional to the patient’s development unless infection, trauma or hormonal changes are present [1,4]. Imaging is an essential diagnostic tool, based on the presence of thin-walled cysts of variable size, generally clustered in the affected area. Depending on the location, depth and size of the lesion, the most useful tests are ultrasound (US) and magnetic resonance (MRI). [2]. The classic therapeutic approach includes sclerotherapy or surgical excision, depending on each case. However, in some cases an optimal result is not accomplished [5]. Recently, angiogenesis inhibitors agents such as Rapamycin (Sirolimus) have been used with promising outcomes. In particular cases when LM or venouslymphatic malformations have not responded to other treatments, oral Rapamycin has been reported to be successful [1,6]. Herein, we report a clinical case of LM nonresponding to conventional management treated with an intralesional biological therapy: Rapamycin stent. To the best of our knowledge, there are no previous reports on the use of this kind of stent in LM.
Authors and Affiliations
Patricio Vargas, Carolina Whittle, Francisca Recule, Ximena Fajre
Keywords
Related Articles
- EP ID EP621892
- DOI 10.26717/BJSTR.2019.18.003179
- Views 116
- Downloads 0
Ozone Effects on Plants: Two Opposite Sides of the Same Coin
Ozone, or trioxygen, is an inorganic molecule with the chemical formula O3 [1]. It is formed from dioxygen by the action of ultraviolet light (UV) and electrical discharges within the Earth’s atmosphere and touches on ou...
Human and Animal Thermophilic Campylobacter infections in East African countries: Epidemiology and Antibiogram
Purpose: Campylobacter mediated diarrhoea is a leading cause of gastroenteritis worldwide. The organisms colonize the gastrointestinal tract of different animal species without causing disease symptoms. Humans acquire in...
Ovarian Surgery and Ovarian Reserve: The Application of Temporary Compression for Natural Hemostasis to Eliminate Exposure of the Ovary to Chemical Agents and Physical Energies
Surgical interventions are primarily associated with the need for hemostasis. All types of energy (mechanical, electrical, thermal, welding, laser, etc.) as well as chemical adhesives and sealants that are used in surger...
Time Dependent Changes of Nicotine and Cotinine In Mouth Cavity After Smoking Studied By MALDI MS Using Functional Organic Analog of Zeolite
Functional organic analog of zeolite (FOAZ) was used as the matrix for laser desorption ionization mass spectrometry and applied to the measurement of nicotine and cotinine in mouth cavity after smoking. In the diffuse r...
Asynchronous Rhythm of Ad4BP/SF-1 and Per2 Expression in Adrenal Tumors of Cushing’s Syndrome Volume 7 - Issue 1
The relationship between circadian rhythm and autonomous cortisol secretion of adrenocortical lesions associated with Cushing’s syndrome is still unknown. We show here that in Y1 adrenocortical tumor cells, circadian rhy...