RESUMO
Background: 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. Methods and Results: Our Lymphedema Program database was reviewed for all referrals between 2009 and 2019. Diagnosis was determined based on history, physical examination, and lymphoscintigraphy. Lymphedema type (primary, secondary, and obesity-induced), location of swelling, morbidity, previous management, accuracy of referral diagnosis, the geographic origin of the patients, and treatment in our center were analyzed. Seven hundred patients were referred with a diagnosis of "lymphedema"; 71% were female and 38% were children. Lymphedema was confirmed in 71% of the cohort: primary (62%), secondary (22%), and obesity-induced (16%). Twenty-nine percent of individuals labeled with "lymphedema" had another condition. One-half of patients had not received treatment, and 36% resided outside of our local referral area. One-third of subjects with lymphedema had an infection and 30% had >1 visit to the center. Patients with confirmed lymphedema were managed with compression stockings (100%), pneumatic compression (69%), and/or an excisional procedure (6%). Conclusions: Patients with lymphedema typically are adequately managed with conservative compression therapies and rarely require excisional operations. Diagnostic confusion is common and individuals with possible lymphedema are best managed by physicians focused on the disease.
Assuntos
Linfedema , Encaminhamento e Consulta , Adulto , Criança , Feminino , Humanos , Sistema Linfático , Vasos Linfáticos , Linfedema/terapia , Linfocintigrafia , MasculinoRESUMO
Background: 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. Study Design: Patients treated in our Lymphedema Program between 2009 and 2018 were reviewed. Diagnosis was determined based on history, physical examination, and imaging studies. Patients with primary lymphedema developing in adulthood (>21 years) were identified. Sex, age of onset, location, severity, morbidity, family history, associated features, and lymphoscintigraphy findings were documented. Results: Twenty-six patients (10%) of 259 with primary lymphedema developed the disease during adulthood. Sixteen individuals were female, and the disease occurred at an average age of 40 years (range, 24-72). It affected the lower extremity (85%) (unilateral 82%, bilateral 18%) or upper limb (15%) (unilateral 80%, bilateral 20%). Twenty-seven percent of patients suffered infections. Ninety-five percent of lymphoscintigrams exhibited delayed transit of radiolabeled tracer and 73% showed dermal backflow. None of the patients had systemic lymphatic involvement or associated vascular anomalies. One patient had a family history of lymphedema. Conclusions: Adult-onset primary lymphedema is typically unilateral, affects the lower extremity, and is not associated with systemic lymphatic anomalies; hereditary transmission is rare. Because adult-onset lymphedema is much less common than the pediatric condition, the disease should be confirmed with lymphoscintigraphy. Imaging of the axillary or inguinal nodes is also considered to rule out a lesion causing secondary lymphedema.
Assuntos
Linfedema/diagnóstico , Linfocintigrafia , Fenótipo , Adulto , Idade de Início , Idoso , Comorbidade , Feminino , Humanos , Extremidade Inferior/patologia , Linfedema/etiologia , Linfocintigrafia/métodos , Masculino , Pessoa de Meia-Idade , Tamanho do Órgão , Índice de Gravidade de Doença , Extremidade Superior/patologia , Adulto JovemRESUMO
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. METHODS: Patients treated in our Lymphedema Program between 2009 and 2018 were reviewed. Only subjects with bilateral lower extremity primary lymphedema were included (individuals with unilateral leg lymphedema were excluded because the tracer will reach the venous circulation and organs through the normal extremity). Disease severity and lymphoscintigraphy findings were documented. RESULTS: Sixty-one patients were included. Ten subjects had no radiolabeled tracer transit to the inguinal lymph nodes on lymphoscintigraphy. However, 8 of these individuals had tracer uptake to the liver, kidney, and/or bladder, illustrating clearance of tracer into the systemic venous circulation. All 8 patients had infant-onset primary lymphedema and mild disease. The 2 patients who did not have clearance of tracer to the systemic venous circulation developed lymphedema in adolescence and had clinically moderate or severe disease. CONCLUSIONS: Patients with primary lower extremity lymphedema often have pathways for lymph fluid to reach the venous circulation other than through the inguinal nodes and thoracic duct. Documentation of systemic tracer uptake during lymphoscintigraphy to confirm a patent thoracic duct has limited clinical significance in subjects with primary disease of the legs.
Assuntos
Perna (Membro)/diagnóstico por imagem , Linfedema/diagnóstico por imagem , Linfocintigrafia/métodos , Humanos , Traçadores RadioativosRESUMO
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. The purpose of this study was to investigate a cohort of patients with primary arm lymphedema to better understand the disease. METHODS: Patients evaluated in our Lymphedema Program between 2008 and 2018 were reviewed for individuals with upper extremity primary lymphedema. Gender, age of onset, morbidity, associated features, and management were identified. Transit of radiolabeled tracer and dermal backflow on lymphoscintigraphy were recorded. RESULTS: Twenty-three patients of 234 individuals with primary lymphedema had upper extremity disease (9.8%). Eleven subjects were male. Age of onset was infancy (n = 15), adolescence (n = 5), or adulthood (n = 3). The disease affected the left arm (n = 11), right arm (n = 9), or both upper extremities (n = 3). Lymphoscintigraphy in 15 patients exhibited delayed transit of tracer and 2 illustrated dermal backflow. One-half of individuals also had primary lower extremity lymphedema (six unilateral and six bilateral). None of the patients in the cohort exhibited a family history of lymphedema. Two individuals had Turner syndrome. Morbidity included infection (n = 5), other lymphatic anomalies (n = 6), and lymphangiosarcoma (n = 1). CONCLUSIONS: The upper extremities are a rare location for primary lymphedema and patients often also have lymphedema of the legs. Compared with that of the lower extremities, primary disease of the arm is more likely to be associated with systemic lymphatic dysfunction and has a lower risk of familial transmission.
Assuntos
Extremidade Inferior/patologia , Vasos Linfáticos/patologia , Linfedema/patologia , Extremidade Superior/patologia , Adolescente , Adulto , Idade de Início , Feminino , Humanos , Lactente , Extremidade Inferior/diagnóstico por imagem , Extremidade Inferior/fisiopatologia , Vasos Linfáticos/diagnóstico por imagem , Vasos Linfáticos/fisiopatologia , Linfedema/diagnóstico por imagem , Linfedema/fisiopatologia , Linfocintigrafia , Masculino , Índice de Gravidade de Doença , Extremidade Superior/diagnóstico por imagem , Extremidade Superior/fisiopatologiaRESUMO
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. We present a patient with a venous malformation of the lip that presented for the first time after she entered menopause which was temporally related to a significant increase in her serum FSH levels that were measured. This observation supports the hypothesis that FSH might influence the pathophysiology of vascular malformations.
Assuntos
Hormônio Foliculoestimulante/sangue , Menopausa/sangue , Malformações Vasculares/sangue , Malformações Vasculares/patologia , Feminino , Humanos , Lábio , Pessoa de Meia-IdadeRESUMO
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. METHODS: Human proliferating IH tissues obtained during a clinically indicated surgical procedure were used. Paraffin sections and isolated cell populations (endothelial, pericyte, stem cell) were subjected to immunofluorescence for FSHR. Tissues were costained with DAPI, anti-α smooth muscle actin, or biotinylated Ulex Europaeus Agglutinin I to identify nuclei, pericytes, and endothelial cells, respectively. Whole tissue and purified single cell populations underwent polymerase chain reaction (PCR) for FSHR. Positive control specimens (ovary, sertoli cells) and negative control tissues (skin/subcutis, hepatic cells) were included. RESULTS: Immunofluorescence of 9 IHs demonstrated that FSHR was enriched in pericytes compared with endothelial cells. Follicle-stimulating hormone receptor was expressed in 6 of 6 whole tissue IHs along with the positive control via PCR. Follicle-stimulating hormone receptor was not present in the negative control samples. Four of 5 sets of pericytes expressed FSHR by PCR. Neither IH endothelial cells, IH stem cells, nor negative control cells exhibited FSHR by PCR. CONCLUSIONS: Because the secretion of FSH correlates with the growth pattern of IH, FSH might be involved in the disease process. Follicle-stimulating hormone receptor is enriched in the pericytes of IH, suggesting that this cell type may be involved in the pathogenesis of the tumor.
Assuntos
Hemangioma Capilar/metabolismo , Receptores do FSH/metabolismo , Biomarcadores/metabolismo , Estudos de Casos e Controles , Células Endoteliais/metabolismo , Feminino , Humanos , Lactente , Masculino , Pericitos/metabolismo , Reação em Cadeia da Polimerase , Células-Tronco/metabolismoRESUMO
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. We collected data pertaining to the timing and reasons for cancellation. Reasons for cancellation were retrospectively classified by the investigators as either "preventable," "possibly preventable," "unpreventable," or "undocumented." We also measured the frequency of cancellations based on type of surgery. RESULTS: Of 10,730 scheduled operating room cases, 444 (4.1%) were cancelled within 24 hours of the procedure. Sixty-seven percent (297/444 cases) were cancelled on the same day as the planned procedure, and the remaining cases were cancelled the day prior after 1 PM. Forty-two percent of cancellations were deemed preventable, and 45.3% of cases were deemed possibly preventable. The majority of procedures were cancelled because of illness (44%), inadequate fasting (9%), and parental inconvenience (7%). The highest frequency of cancellation was found in skin lesion (36%) followed by dentoalveolar (14%) and cleft lip and palate (12%) cases. CONCLUSIONS: In our study, most late surgical cancellations were preventable or possibly preventable. The timing of the cancellation is important because those that occur near the scheduled procedure time disallow adequate and timely redistribution of operating room resources and personnel. Analyzing and addressing the preventable and possibly preventable causes outlined in this study will significantly improve efficiency and patient access.
Assuntos
Agendamento de Consultas , Procedimentos Cirúrgicos Bucais , Pediatria , Recusa do Paciente ao Tratamento/estatística & dados numéricos , Humanos , Estudos RetrospectivosRESUMO
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.
Assuntos
Hemangioendotelioma/complicações , Síndrome de Kasabach-Merritt/complicações , Linfedema/complicações , Sarcoma de Kaposi/complicações , Criança , Diagnóstico Diferencial , Glucocorticoides/uso terapêutico , Hemangioendotelioma/tratamento farmacológico , Humanos , Síndrome de Kasabach-Merritt/tratamento farmacológico , Extremidade Inferior/patologia , Linfedema/diagnóstico , Masculino , Sarcoma de Kaposi/tratamento farmacológicoRESUMO
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. METHODS: Referrals to our Vascular Anomalies Center between 2008 and 2017 were reviewed for patients treated with propranolol at an outside institution. Patient history, photographs, imaging studies, and/or histopathology were evaluated by an interdisciplinary team to diagnose the vascular anomaly. Our center's diagnosis was compared to the referral diagnosis to categorize patients into 3 groups: Group 1 (patients were appropriately labeled with an IH); Group 2 (individuals were erroneously diagnosed with IH); and Group 3 (subjects were diagnosed with a vascular anomaly other than IH). RESULTS: Two hundred thirty-six patients met inclusion criteria. Group 1 (39%; n = 91) had an IH and were treated appropriately. Group 2 (20%; n = 49) was misdiagnosed with IH and incorrectly received propranolol. Group 3 (41%; n = 96) was given propranolol to treat another vascular anomaly. Propranolol did not have efficacy for vascular anomalies other than IH. CONCLUSIONS: Propranolol commonly is used to treat lesions other than IH; misdiagnosis of a lesion as IH is a common cause. Propranolol should be used with caution to treat other types of vascular anomalies because patients are subjected to the risks of the drug without data supporting its efficacy.
Assuntos
Antagonistas Adrenérgicos beta/uso terapêutico , Propranolol/uso terapêutico , Neoplasias Cutâneas/tratamento farmacológico , Malformações Vasculares/tratamento farmacológico , Adulto , Criança , Pré-Escolar , Erros de Diagnóstico , Feminino , Hemangioma Capilar/diagnóstico , Hemangioma Capilar/tratamento farmacológico , Humanos , Prescrição Inadequada , Lactente , Masculino , Resultado do Tratamento , Malformações Vasculares/diagnósticoRESUMO
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. METHODS: Individuals referred to our lymphedema program with "lymphedema" between 2009 and 2016 were analyzed. Subjects were assessed by history, physical examination, and lymphoscintigraphy. Patient age at presentation, duration of lymphedema, location of disease, gender, previous infections, and lymphedema type were analyzed. RESULTS: The study included 227 patients (454 limbs); lymphedema was diagnosed clinically in 169 subjects and confirmed by lymphoscintigraphy in 162 (117 primary, 45 secondary; 96% sensitivity). Fifty-eight patients were thought to have a condition other than lymphedema, and all had negative lymphoscintigrams (100% specificity). A subgroup analysis of the 7 individuals with lymphedema clinically, but normal lymphoscintigrams, showed that all had primary lymphedema; duration of disease and infection history were not different between true-positive and false-negative lymphoscintigram results (P = 0.5). Two patients with a false-negative test underwent repeat lymphoscintigraphy, which then showed lymphatic dysfunction consistent with lymphedema. CONCLUSION: Lymphoscintigraphy is very sensitive and specific for lymphedema. All patients with false-negative studies had primary lymphedema. A patient with a high clinical suspicion of lymphedema and a normal lymphoscintigram should be treated conservatively for the disease and undergo repeat lymphoscintigraphy.
RESUMO
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. STUDY DESIGN: Patients treated in our Lymphedema Program between 2009 and 2017 were reviewed. Diagnosis of lymphedema was determined by history, physical examination, and lymphoscintigraphy. Severity was defined by increased volume of the limb as follows: mild (<20%), moderate (20% to 40%), and severe (>40%). Candidate variables included location (arm, leg), age, duration of symptoms, infection history, and lymphedema type (primary, secondary). An association between lymphedema severity and lymphoscintigraphy findings was determined using the Pearson chi-square test and multivariate logistic regression. RESULTS: One hundred and thirty-four patients with 181 affected extremities (24 upper, 157 lower) were included. Clinical severity was as follows: 54% mild, 30% moderate, and 16% severe. Delayed tracer transit to the regional nodes was as follows: 45 minutes (34%), 2 hours (18%), and 4 hours or longer (48%). Thirty-six percent of extremities demonstrated dermal backflow. Abnormal transit time or dermal backflow was identified in 97% of extremities by 45 minutes and in 3% of limbs by 2 hours. Transit time and dermal backflow were not predictive of clinical severity when adjusting for candidate variables (p > 0.1). CONCLUSIONS: Clinical severity of lymphedema is not associated with lymphoscintigraphy findings. A lymphoscintigram should be interpreted as normal or abnormal, and does not need to exceed 2 hours.
Assuntos
Linfedema/diagnóstico por imagem , Linfocintigrafia , Índice de Gravidade de Doença , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Adulto JovemRESUMO
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. This observation may provide greater insight into the pathophysiology of the disease.
RESUMO
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. There are many causes of leg overgrowth in the pediatric population and misdiagnosis is common. While extremity enlargement secondary to hemihypertrophy may occur in 15% of patients with Beckwith-Wiedemann syndrome, progression and pitting edema only occur in primary lymphedema. This report highlights the importance of ensuring an accurate diagnosis so that patients are managed appropriately.
Assuntos
Síndrome de Beckwith-Wiedemann/complicações , Linfedema/complicações , Síndrome de Beckwith-Wiedemann/diagnóstico por imagem , Criança , Erros de Diagnóstico , Feminino , Humanos , Extremidade Inferior/patologia , Linfedema/diagnóstico por imagem , LinfocintigrafiaRESUMO
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/m2) who had lower-extremity lymphatic function evaluated by lymphoscintigraphy. Candidate variables included age, sex, BMI, duration of lymphedema, infection history, and lymphoscintigraphy findings. A possible association between candidate variables and presence of MLL was determined using multivariable logistic regression. Optimal cutoff for BMI in predicting MLL was identified by receiver operating characteristic curve analysis. RESULTS: Eighty-two patients were included in the study population. In patients with MLL (n = 17), all had OIL and none had primary or secondary lymphedema (median BMI 66 kg/m2; interquartile range 62 to 78). Massive localized lymphedema involved the thigh (n = 16; bilateral = 10, unilateral = 6), genitalia (n = 3), and suprapubic area (n = 2). Control patients without MLL (n = 65) had primary (46%), secondary (37%), or obesity-induced (17%) lymphatic dysfunction (median BMI 36 kg/m2; interquartile range 32 to 45). Logistic regression indicated a significant relationship between BMI and MLL condition; patients with a BMI >56 kg/m2 had a 213-times greater odds of MLL developing vs patients with BMI ≤56 kg/m2 (p < 0.0001). Age, sex, duration of obesity, and infection history were not associated with development of MLL (all p > 0.2). CONCLUSIONS: Massive localized lymphedema is a consequence of OIL and affects approximately 60% of obese patients with lower-extremity dysfunction; a BMI >56 kg/m2 significantly increases the risk. Obese individuals should be referred to a bariatric weight-loss center before their BMI reaches a threshold for OIL and MLL to develop.
Assuntos
Linfedema/etiologia , Obesidade/complicações , Adulto , Idoso , Estudos de Casos e Controles , Feminino , Humanos , Modelos Logísticos , Linfedema/diagnóstico , Linfedema/patologia , Masculino , Pessoa de Meia-Idade , Curva ROC , Fatores de RiscoRESUMO
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). METHODS: Our Vascular Anomalies Center and Lymphedema Program databases were searched for patients with both capillary malformation and lymphedema. Diagnosis of lymphedema-capillary malformation was made by history, physical examination, and imaging studies. Because lymphedema-capillary malformation has phenotypical overlap with other conditions, only patients who had imaging confirming their diagnosis were included in the analysis. Clinical and radiological features, morbidity, and treatment were recorded. RESULTS: Eight patients (4 females and 4 males) had confirmed lymphedema-capillary malformation. Referring diagnosis was Klippel-Trenaunay syndrome (n = 4), diffuse capillary malformation with overgrowth (n = 3), or lymphatic malformation (n = 1). The condition was unilateral (n = 6) or bilateral (n = 2). Morbidity included infection (n = 6), difficulty fitting clothes (n = 6), bleeding or leaking vesicles (n = 5), leg length discrepancy (n = 4), and difficulty ambulating (n = 3). All patients were managed with compression regimens. Operative management was liposuction (n = 3), treatment of phlebectatic veins (n = 3), staged skin/subcutaneous excision (n = 1), and/or epiphysiodesis (n = 1). CONCLUSIONS: Lymphedema and capillary malformation can occur together in the same extremity. Both conditions independently cause limb overgrowth primarily because of subcutaneous adipose deposition. Compression garments and suction-assisted lipectomy can improve the condition. Lymphedema-capillary malformation should not be confused with other vascular malformation overgrowth diseases that have different morbidities and treatments.
RESUMO
BACKGROUND: A somatic mutation in GNAQ (c.548G>A; p.R183Q), encoding Gαq, has been found in syndromic and sporadic capillary malformation tissue. However, the specific cell type containing the mutation is unknown. The purpose of this study was to determine which cells in capillary malformations have the GNAQ mutation. METHODS: Human capillary malformation tissue was obtained from 13 patients during a clinically indicated procedure. Droplet digital polymerase chain reaction, capable of detecting mutant allelic frequencies as low as 0.1 percent, was used to quantify the abundance of GNAQ mutant cells in capillary malformation tissue. Six specimens were fractionated by fluorescence-activated cell sorting into hematopoietic, endothelial, perivascular, and stromal cells. The frequency of GNAQ mutant cells in these populations was quantified by droplet digital polymerase chain reaction. RESULTS: Eight capillary malformations contained GNAQ p.R183Q mutant cells, two lesions had novel GNAQ mutations (p.R183L and p.R183G), and three capillary malformations did not have a detectable GNAQ p.R183 mutation. Mutant allelic frequencies ranged from 2 to 11 percent. Following fluorescence-activated cell sorting, the GNAQ mutation was found in the endothelial but not the platelet-derived growth factor receptor-ß-positive cell population; mutant allelic frequencies were 3 to 43 percent. CONCLUSION: Endothelial cells in capillary malformations are enriched for GNAQ mutations and are likely responsible for the pathophysiology underlying capillary malformation.
Assuntos
Capilares/anormalidades , DNA/genética , Células Endoteliais/patologia , Endotélio Vascular/patologia , Subunidades alfa Gi-Go de Proteínas de Ligação ao GTP/genética , Malformações Vasculares/genética , Adolescente , Adulto , Idoso , Alelos , Capilares/metabolismo , Capilares/patologia , Criança , Análise Mutacional de DNA , Células Endoteliais/metabolismo , Endotélio Vascular/metabolismo , Feminino , Citometria de Fluxo , Subunidades alfa Gi-Go de Proteínas de Ligação ao GTP/metabolismo , Humanos , Masculino , Microscopia Confocal , Pessoa de Meia-Idade , Mutação , Reação em Cadeia da Polimerase , Malformações Vasculares/metabolismo , Malformações Vasculares/patologia , Adulto JovemRESUMO
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. METHODS: A 17 × 17-mm critical-sized defect was made in the parietal bone of 16 rabbits. Four animals had no implant (group 1). Twelve animals had the defect remade 16 weeks postoperatively, which was managed in 2 ways: group 2 (no implant; n = 6) and group 4 (particulate bone graft; n = 6). Particulate graft was obtained using a brace and bit from the frontal bone. Computed tomography was used to determine the area of ossification and thickness of the healed graft. Eight animals previously managed with particulate bone graft over normal dura were used as an additional control (group 3). RESULTS: Critical-sized defects filled with particulate bone graft over scarred dura (group 4) exhibited superior healing of the area (83.8%; range, 73.0%-90.6%) compared to control defects over normal dura (group 1: 62.9%; range, 56.5%-73.4%) or scarred dura (group 2: 56.9%; range, 40.0%-68.3%) (P = 0.0004). Particulate bone on scarred dura exhibited less ossified area (P = 0.002), and thinner bone (0.95 mm, range, 0.71-1.32 mm) compared to defects in which graft was placed over normal dura (group 3: area, 99.2%; range, 96.8%-100%; thickness, 1.9 mm, range; 1.1-3.1 mm) (P = 0.04). CONCLUSIONS: Particulate bone graft ossifies inlay cranial defects over scarred dura although inferior to placement over normal dura. Clinically, particulate bone graft may be used for secondary inlay cranioplasty.
Assuntos
Transplante Ósseo/métodos , Cicatriz , Dura-Máter/patologia , Osso Parietal/cirurgia , Procedimentos de Cirurgia Plástica/métodos , Animais , Craniotomia , Osso Frontal/cirurgia , Osteogênese , Osso Parietal/patologia , Coelhos , ReoperaçãoRESUMO
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. The diagnosis of lymphedema was made by history/physical examination and confirmed with lymphoscintigraphy. Patient sex, age, type of lymphedema (primary or secondary), location of disease, infection history, volume of lipoaspirate, and reduction of extremity volume were recorded. RESULTS: Fifteen patients were included, mean age was 45 years (range, 17-71). Six patients had secondary upper extremity lymphedema, and 9 patients had lower limb disease. Eight patients had a history of repeated cellulitis involving the lymphedematous extremity. Mean lipoaspirate volume was 1612 mL (range, 1200-2800) for the upper extremity and 2902 mL (range, 2000-4800) for the lower limb. Postoperative follow-up averaged 3.1 years. The mean reduction in excess extremity volume was 73% (range, 48% to 94%), and patients reported improvement in their quality of life. CONCLUSIONS: Suction-assisted lipectomy is an effective technique to reduce extremity volume for patients with lymphedema.