Study of 700 Referrals to a Lymphedema Program.

Lymphedema results from inadequate lymphatic function due to failure of lymphatic development or injury to a functioning lymphatic system. Patients suffer enlargement of the affected area, psychosocial morbidity, infection, and functional disability. The purpose of this study was to characterize the disease in a cohort of patients referred to a specialized center.

Adult-Onset Primary Lymphedema: A Clinical-Lymphoscintigraphic Study of 26 Patients.

Primary lymphedema results from the anomalous development of the lymphatic system that typically presents during infancy, childhood, or adolescence. Adult-onset primary lymphedema is rare and has not been studied. The purpose of this investigation was to characterize patients with primary lymphedema that developed after the pediatric time period to better understand the condition.

Lymphoscintigraphic Evaluation of Systemic Tracer Uptake in Patients With Primary Lymphedema.

Background: Lymphoscintigraphy is used to confirm the diagnosis of lymphedema. One end point for the test is to ensure a patent thoracic duct by uptake of tracer in the organs. The purpose of this study was to evaluate transit of radiolabeled colloid to the organs to gain insight into the etiopathophysiology of primary lymphedema.

Primary Lymphedema of the Upper Extremities: Clinical and Lymphoscintigraphic Features in 23 Patients.

Background: Primary idiopathic lymphedema is an uncommon condition that typically affects the lower extremities. Patients have a malformed lymphatic system that causes subcutaneous fluid and adipose deposition. Rarely, the disease also has been described in the upper extremities.

Analysis of Follicle-Stimulating Hormone Receptor in Infantile Hemangioma.

Introduction: The life cycle of infantile hemangioma (IH) and secretion of follicle-stimulating hormone (FSH) are identical. We previously have shown that IH contains the FSH receptor (FSHR). The purpose of this study was to identify which cell type(s) in IH expresses FSHR.

Vascular Malformation Enlargement During Menopause.

Vascular malformations enlarge overtime, particularly during adolescence when follicle-stimulating hormone (FSH) rises. Lesions contain the receptor for follicle-stimulating hormone. FSH also becomes elevated during menopause.

Association between extremity kaposiform hemangioendothelioma and lymphedema.

Kaposiform hemangioendotheliomas are pediatric vascular tumors that do not metastasize. We present a patient with a thigh kaposiform hemangioendothelioma successfully treated using a systemic corticosteroid during infancy who was diagnosed with lymphedema in the extremity 9 years later. The observation that extremity kaposiform hemangioendothelioma could possibly be associated with lymphedema has implications for the care of patients with kaposiform hemangioendothelioma.

Factors Associated With Late Surgical Cancellations in Pediatric Plastic and Oral Surgery.

Background: Surgical cancellations that occur within 1 day of the procedure (ie, late cancellations) disrupt the efficiency of the operating room. The aim of the present study was to identify the factors associated with late cancellations in a tertiary pediatric surgical practice.

Methods: We reviewed the medical records of patients treated by plastic and oral surgery services at our institution from 2010 to 2015.

Propranolol Treatment of Vascular Anomalies Other Than Infantile Hemangioma.

Background: Oral propranolol has become first-line intervention for problematic infantile hemangioma (IH) that is not amenable to topical or intralesional therapies. Consensus data supporting its efficacy for other vascular anomalies does not exist. The purpose of this study was to determine the frequency and causes of propranolol use for vascular lesions other than IH.

Diagnostic Accuracy of Lymphoscintigraphy for Lymphedema and Analysis of False-Negative Tests.

Unlabelled: Lymphedema is the chronic enlargement of tissue due to inadequate lymphatic function. Diagnosis is made by history and physical examination and confirmed with lymphoscintigraphy. The purpose of this study was to assess the accuracy of lymphoscintigraphy for the diagnosis of lymphedema and to determine characteristics of patients with false-negative tests.

Correlation Between Lymphedema Disease Severity and Lymphoscintigraphic Findings: A Clinical-Radiologic Study.

Background: Lymphoscintigraphy is used to confirm the diagnosis of lymphedema; pathologic findings are abnormal transit time to regional nodes and dermal backflow. A universal protocol for the test does not exist. The purpose of this study was to determine whether the clinical severity of lymphedema correlates with lymphoscintigraphy findings.

Resolution of Primary Lymphedema: A Case Report.

Primary lymphedema is a rare, progressive disease that typically affects the lower extremity. The condition is not curable, and the limb enlarges over time because of subcutaneous fibroadipose deposition. We present a patient with clinical and radiographical evidence of resolution of primary lymphedema.

Massive Localized Lymphedema: A Case-Control Study.

Background: Massive localized lymphedema (MLL) is an area of skin and subcutaneous overgrowth associated with obesity. The purpose of this study was to determine whether MLL results from obesity-induced lymphedema (OIL) and to characterize the prevalence and risk factors for the condition.

Study Design: Patients evaluated in our Lymphedema Program between 2009 and 2016 were reviewed for obese individuals (BMI ≥30 kg/m) who had lower-extremity lymphatic function evaluated by lymphoscintigraphy.

Beckwith-Wiedemann Syndrome and Primary Lymphedema of the Lower Extremity.

Beckwith-Wiedemann syndrome is the most common genetic overgrowth syndrome. Patients with Beckwith-Wiedemann syndrome may have hemihypertrophy, but their lymphatic vasculature is intact. We present a child with Beckwith-Wiedemann syndrome and lower extremity enlargement thought to be due to hemihypertrophy that was instead diagnosed with primary lymphedema.

Combined Lymphedema and Capillary Malformation of the Lower Extremity.

Unlabelled: Primary lymphedema and capillary malformation are independent vascular malformations that can cause overgrowth of the lower extremity. We report a series of patients who had both types of malformations affecting the same leg. The condition is unique but may be confused with other types of vascular malformation overgrowth conditions (eg, Klippel-Trenaunay and Parkes Weber).

Operative Treatment of Lymphedema Using Suction-Assisted Lipectomy.

Background: Surgical management of lymphedema includes removal of affected tissues (excisional procedures), or operations that create new lymphatic connections (physiologic procedures). The purpose of this study was to determine the efficacy of one type of excisional procedure, suction-assisted lipectomy, for extremity lymphedema.

Methods: Patients treated in our Lymphedema Program between 2007 and 2015 with liposuction that had postoperative follow-up were reviewed.

Management of Vascular Anomalies and Related Conditions Using Suction-Assisted Tissue Removal.

Unlabelled: Vascular anomalies and related conditions cause overgrowth of tissues. The purpose of this study was to determine the efficacy and safety of liposuction techniques for pediatric overgrowth diseases. Patients treated between 2007 and 2015 who had follow-up were reviewed.

Endothelial Cells from Capillary Malformations Are Enriched for Somatic GNAQ Mutations.

Background: A somatic mutation in GNAQ (c.548G>A; p.R183Q), encoding Gαq, has been found in syndromic and sporadic capillary malformation tissue.

Obesity-induced Lymphedema Nonreversible following Massive Weight Loss.

Lymphedema is the progressive enlargement of tissue due to inadequate lymphatic function. Obesity-induced lymphedema of the lower extremities can occur once a patient's body mass index (BMI) exceeds 50. We report our first patient with obesity-induced lower extremity lymphedema who was followed prospectively before and after weight loss.

Obesity-induced lymphedema: clinical and lymphoscintigraphic features.

Unlabelled: The purpose of this study was to characterize obesity as a novel cause of lower extremity lymphedema. Fifty-one patients with a body mass index greater than 30 kg/m(2) without any potential cause of lymphedema were evaluated with lymphoscintigraphy. Group 1 patients (n = 33) were at their maximum body mass index; individuals with lymphatic dysfunction had a greater body mass index (72.

Lymphatic and other vascular malformative/overgrowth disorders are caused by somatic mutations in PIK3CA.

Objectives: To test the hypothesis that somatic phosphatidylinositol-4,5-bisphospate 3-kinase, catalytic subunit alpha (PIK3CA) mutations would be found in patients with more common disorders including isolated lymphatic malformation (LM) and Klippel-Trenaunay syndrome (KTS).

Study Design: We used next generation sequencing, droplet digital polymerase chain reaction, and single molecule molecular inversion probes to search for somatic PIK3CA mutations in affected tissue from patients seen at Boston Children's Hospital who had an isolated LM (n = 17), KTS (n = 21), fibro-adipose vascular anomaly (n = 8), or congenital lipomatous overgrowth with vascular, epidermal, and skeletal anomalies syndrome (n = 33), the disorder for which we first identified somatic PIK3CA mutations. We also screened 5 of the more common PIK3CA mutations in a second cohort of patients with LM (n = 31) from Seattle Children's Hospital.

An Experimental Study of Particulate Bone Graft for Secondary Inlay Cranioplasty Over Scarred Dura.

Background: Inlay cranioplasty in children is challenging because autologous bone is limited. Cranial particulate bone graft effectively closes defects when placed over normal dura. The purpose of this study was to determine if particulate bone graft will ossify when used for secondary cranioplasty over scarred dura.

Obesity-induced Upper Extremity Lymphedema.

Obesity increases the risk of upper extremity lymphedema following treatment for breast cancer and can cause lower extremity lymphatic dysfunction in extremely obese individuals. We report the first patient with obesity-induced upper extremity lymphedema. A 62-year-old man with a previous body mass index (BMI) of 105.