Estudios primarios incluidos en esta revisión sistemática

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Estudio primario

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Revista The Laryngoscope
Año 2012
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OBJECTIVES/HYPOTHESIS: To describe the clinical presentation and airway characteristics of infants with airway hemangiomas and concomitant PHACE syndrome and to determine the prevalence of airway hemangiomas in PHACE subjects at our institution. STUDY DESIGN: Case series. METHODS: Retrospective review including clinical presentation, airway findings, treatment measures, and outcomes. RESULTS: A total of 23 subjects were diagnosed with definite PHACE at our institution between September 1, 2005 and September 1, 2011. Twelve (52%) of these subjects had documented airway hemangiomas, six of whom were diagnosed and treated at our institution. All six subjects underwent direct laryngoscopy and bronchoscopy by a pediatric otolaryngologist. Five (83%) subjects had subglottic hemangioma. Three subjects (50%) had additional hemangioma within the airway located on the epiglottis, vocal folds, posterior pharyngeal wall, and tracheal wall. Five subjects (83%) were treated with propranolol, five (83%) were treated with systemic steroids, and one subject received vincristine. One subject required laser ablation of subglottic hemangioma and tracheotomy. All subjects were airway symptom free at last follow-up (average, 35 months; range, 13-76 months). CONCLUSIONS: Airway hemangiomas can be a life-threatening complication of PHACE syndrome. At our institution, 52% of all PHACE subjects were diagnosed with airway hemangiomas. Early detection of airway involvement is paramount. Given the high rates of airway hemangiomas, we recommend performing direct laryngoscopy and bronchoscopy in all PHACE patients with respiratory symptoms. We recommend having a low threshold for airway evaluation in asymptomatic PHACE patients, especially those who will not be otherwise started on propranolol.

Estudio primario

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Revista Ophthalmic plastic and reconstructive surgery
Año 2012
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An 11-year-old boy presented with progressive right-sided proptosis and an orbital mass on neuroimaging. Incisional biopsy revealed an epithelioid hemangioma. The patient underwent an orbitotomy with subtotal excision of the mass. However, the tumor recurred and progressed to the cavernous sinus, despite oral steroids, tacrolimus, and a second subtotal excision. Because of encouraging results reported with beta-blockers in the treatment of pediatric capillary hemangiomas, the patient was subsequently started on oral propranolol, 2 mg/kg/day, with discontinuation of steroids and tacrolimus. A rapid and impressive interval decrease in tumor size was observed, with improvement in proptosis and exposure keratopathy. The patient remains without recurrence approximately 9 months after initiation of propranolol. Our results suggest that oral propranolol may be a potential alternative therapy when complete excision of an epithelioid hemangioma is not practical.

Estudio primario

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Revista The Turkish journal of pediatrics
Año 2011
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Propranolol, a non-selective beta-blocker, has recently been introduced as a treatment for infantile hemangiomas. In this study, we evaluated the effect of propranolol in 12 infants with hemangioma. Twelve infants (9 girls) with a median age of 4.5 months were included in the study. All of the patients in the study group received short-term (1-9 weeks, median: 4 weeks) systemic corticosteroids as a first-line therapy. All patients received propranolol 2 mg/kg/day, divided into three doses. They were treated in an inpatient setting for the first 72 hours of the treatment. Vital signs, blood pressure and blood glucose were monitored. Propranolol treatment was given for 4-9 months (median: 5 months). In the study group, regression rate of the mean dimension of the lesion was 38% +/- 15 (range 15%-50, median 45%) at the 2nd month of therapy. Over 9 months, which was the maximum follow-up period, the regression rate of the mean dimension of the lesion was 55% +/- 31 (range 20%-80, median 50%). One patient had transient bradycardia, which improved spontaneously. No other side effect was observed in the study population. Propranolol appears to be an effective drug for infantile hemangiomas with good clinical tolerance. We suggest that propranolol is the preferable drug as the first-line therapy for infantile hemangiomas.

Estudio primario

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Revista Archives of otolaryngology--head & neck surgery
Año 2011
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OBJECTIVES: To report the efficacy of propranolol as first-line treatment of head and neck hemangiomas in children and to present an optimized protocol for treating hemangiomas. DESIGN: Multi-institutional retrospective study. SETTING: Two tertiary care referral pediatric centers. PATIENTS: Thirty-nine children with head and neck infantile hemangiomas were treated. MAIN OUTCOME MEASURES: Review of clinical records. RESULTS: Propranolol was the sole treatment in 60% of patients and was started at a mean age of 4.1 months (age range, 1-11 months) for early interventions among 33 of 39 patients. Propranolol therapy resulted in lightening and reduction of hemangiomas at 37 of 39 locations within 2 days to 2 weeks. One subglottic hemangioma and 1 nasal tip hemangioma did not respond or showed only a partial response; in these patients, propranolol therapy was delayed and followed other treatment failures. After successful therapeutic regression, 6 recurrences occurred; when reintroduced, propranolol was again effective. Recurrences were avoided by prolonged treatment. Twenty-six hemangiomas occurring at locations for which corticosteroid treatment previously would not have been initiated (nose, lips, and parotid area) unless a complication had occurred were treated with propranolol and were rapidly controlled. The mean duration of propranolol therapy was 8.5 months. No instances of β-blocker discontinuation because of complications occurred, but propranolol was substituted by acebutolol in 5 patients because of trouble sleeping. CONCLUSIONS: Propranolol is an effective treatment of head and neck infantile hemangiomas, especially when started early within the rapid growth phase, and is first-line treatment of orbit and larynx hemangiomas. The efficacy and tolerability of propranolol led us to treat some hemangiomas in patients whom we previously would have observed rather than subject to corticosteroid therapy. Relapse was avoided if treatment was prolonged after theoretical involution (age 12 months). Questions remain about optimal dosing and age at treatment cessation.

Estudio primario

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Revista International journal of pediatric otorhinolaryngology
Año 2011
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INTRODUCTION: Infantile subglottic hemangiomas are rare causes of airway obstruction. They begin to proliferate at 1-2 months of age and can cause biphasic stridor with or without respiratory distress. Diagnosis requires direct visualization by direct laryngoscopy and bronchoscopy. Various therapeutic options have been utilized for treatment, including tracheotomy, open surgical excision, laser ablation, intralesional steroid injection, systemic steroids, and now oral propranolol. METHODS: We present a retrospective chart review of infantile subglottic hemangiomas over a 5-year span (January 2005-2010) at a tertiary care pediatric hospital. IRB approval was obtained, and charts were reviewed to find patients with subglottic hemangiomas, including patient characteristics, presentation, workup, medical and surgical management, and outcomes. A case presentation demonstrates diagnostic, management, and treatment strategies and dilemmas encountered. RESULTS: Nine patients were found to have infantile subglottic hemangiomas. Six of nine patients were treated with laser excision, with five of the six having localized subglottic hemangiomas. In 2009, three of four patients were initiated on propranolol as first-line treatment; the fourth had comorbidities which precluded this. Of the three, two showed improvement, while a third, who also had bearded hemangioma, required tracheotomy. DISCUSSION: Infantile subglottic hemangiomas are rare but essential in the differential diagnosis of biphasic stridor. Although propranolol has been effective in treating cutaneous and airway hemangiomas, our experience suggests that this is not consistent for subglottic hemangiomas. In an area where airway compromise can be lethal, we must extend caution and monitor these patients closely as they may require adjuvant therapy.

Estudio primario

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Autores Ghosh PS , Ghosh D
Revista Neurology
Año 2011
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Estudio primario

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Revista Journal of perinatology : official journal of the California Perinatal Association
Año 2011
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We report the case of a 29-week preterm infant with PHACE (posterior fossa malformations, hemangionas, arterial anomalies, cardiac anomalies, eye anomalies) syndrome. PHACE syndrome is a neurocutaneous disorder with large facial segmental hemangionas associated with anomalies of the brain, eye, heart and aorta. The hemangiomas in our patient were problematic, distorting the airway and interfering with respirations to the point of requiring mechanical ventilation. Consultation with several different centers with medical expertize in treatment of congenital hemangiomas revealed different views on the best management strategy. In this infant, the hemangiomas progressed with failure to involute despite currently recommended therapy including corticosteroids and vincristine. Therefore, the infant was treated with propranolol and had significant regression of the hemangiomas. The use of propranolol for the treatment of infantile hemangiomas is reviewed.

Estudio primario

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Autores Javia LR , Zur KB , Jacobs IN
Revista International journal of pediatric otorhinolaryngology
Año 2011
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There has been a dramatic evolution in the treatment of laryngotracheal hemangiomas during the past decade and recent accounts and case reports of propranolol treatment have been encouraging. The purpose of the study is to determine the clinical course and outcomes of treating laryngotracheal hemangiomas at The Children's Hospital of Philadelphia in the last 8 years with the various modalities. We review with contemporary surgical techniques, including propranolol, and determine the results, limitations and complications. The study was a retrospective review of all patients referred to the Otolaryngology service at The Children's Hospital of Philadelphia with symptomatic laryngotracheal hemangiomas between January 2002 and December 2010. The study consisted of 30 infants, ranging in age from 1 to 18 months at time of diagnosis. Surgical interventions included open surgical excision, laser surgery, microdebrider excision and/or propranolol therapy. The main outcome measures include improvement in symptoms, decannulation, number of required treatments and airway size. All but two patients underwent an initial trial of steroids. Thirteen patients underwent open surgical excision, 9 requiring cartilage grafts and 12 were done in a single stage. Twelve surgical patients remained asymptomatic. One patient with diffuse mediastinal disease experiencing postoperative airway symptoms despite a normal appearing airway improved on propranolol. Two patients underwent at least 2 laser ablations, 4 responded to systemic steroids alone, and 1 had microdebrider resection. In the last 14 months, 12 patients have had propranolol therapy at a dose of 2mg/kg divided every 8h. Eight patients improved clinically within 1 week of initiating treatment. Four patients failed to respond to propranolol therapy; 1 patient subsequently underwent open excision and the other continued with a tracheostomy for 18 months and finally was decannulated. A third patient had a partial response, but remains relatively asymptomatic. A fourth patient has had no response at all. There were no major complications from propranolol; minor complications included diarrhea and decreased appetite. This study gives an overview of the evolution of hemangioma treatment at our institution over the last 8 years. Surgical excision remains an effective treatment for subglottic hemangiomas. Carefully administered, propranolol may demonstrate efficacy as a first-line agent in most cases avoiding surgery, tracheostomy, prolonged steroids, or as treatment of diffuse and unresectable disease. However, some lesions may be resistant to propranolol and require surgery or long-term steroids.

Estudio primario

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Revista Middle East African journal of ophthalmology
Año 2011
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PURPOSE: To investigate the efficacy and safety of oral propranolol in the management of periorbital infantile hemangioma in four subjects. MATERIALS AND METHODS: Consecutive patients who presented with periorbital capillary hemangioma with vision-threatening lesions were prospectively enrolled in this study between January 2009 and October 2010. All subjects underwent treatment with 2 mg/kg/day oral propranolol. All subjects underwent ocular, systemic, and radiologic evaluations before treatment and at periodic intervals after starting therapy. Side effects from therapy were also evaluated. RESULTS: Four subjects, between 3 months and 19 months of age, with periorbital hemangioma were enrolled in this study. Two subjects had been previously treated with oral corticosteroids with unsatisfactory response. All subjects had severe ptosis, with the potential for deprivation amblyopia. Three subjects had orbital involvement. After hospital admission, oral propranolol was initiated in all subjects under monitoring by a pediatric cardiologist. Subsequent therapy was performed with periodic out-patient monitoring. All subjects had excellent response to treatment, with regression of periorbital and orbital hemangioma. There were no side effects from therapy. CONCLUSIONS: Oral propranolol for periorbital hemangioma was effective in all the four subjects. Oral propranolol may be appropriate for patients who are nonresponsive to intralesional or systemic steroids. In patients with significant orbital involvement and lesions causing vision-threatening complications, oral propranolol can be the primary therapy.

Estudio primario

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Revista Pediatric Dermatology
Año 2011
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El objetivo del presente estudio fue comparar la eficacia clínica de propranolol oral con el de prednisona oral en el tratamiento de los hemangiomas infantiles (IH). Los pacientes tratados por IH con propranolol oral, fueron retrospectivamente emparejados con los pacientes tratados con prednisona oral según el tipo, la ubicación y el tamaño de la IH y la edad al inicio del tratamiento. La respuesta al tratamiento fue evaluada por rating fotografías tomadas médicos serie 1, 2, y 6 meses después del inicio del tratamiento. Grado de mejoría clínica en apariencia general (incluyendo color y tamaño) se calificó como sigue: peor o estable (0), leve (<25%), moderada (25-50%), buena (50-75%), o excelente (> 75%). Una segunda evaluación se realizó mediante una escala analógica visual de 100 mm para evaluar la mejora a los 6 meses. Imágenes pre y post-tratamiento estaba disponible para varios pacientes. Se analizaron doce pares de bebés con IH. En 1 mes, mejoría clínica en el grupo propranolol fue de moderada a buena en todos los pacientes. En el grupo de prednisona, sólo un paciente tuvo mejoría moderada, con los demás mostrando ligero (7/12) o ninguna mejoría o estabilización (3/12) de la línea de base y un caso empeoramiento. A los 6 meses, el grupo propranolol mostró buena a excelente respuesta en todos los casos, mientras que nueve en el grupo de prednisona mostraron ligera respuesta a moderada. Ecografía Doppler y la resonancia magnética correlacionado con la mejora clínica en los casos en que se realizó. No se observaron efectos secundarios importantes en ninguno de los grupos. Propranolol parece superior a la prednisona oral en la inducción de una mejoría clínica más rápida y una mayor-en este estudio. Un estudio prospectivo más grande comparando estas dos modalidades de tratamiento se justifica.