Infantile hemangioma: Predicting proliferation by infrared thermography.

BACKGROUND AND OBJECTIVE: Infantile hemangiomas (IHs) are benign lesions found in infants. Predicting the cosmetic outcome of these lesions is very difficult. Therefore, in this prospective study, we assessed whether using an infrared thermometer (IRT) to measure the surface temperature of IHs would help to predict their proliferative potential. MATERIALS AND METHODS: Between January 2012 and March 2014, we prospectively investigated 103 children up to 6 months of age with a diagnosis of IH. None of them required immediate treatment. Two projection plain photographs of the IHs were obtained and the temperature of the IH surface was measured with the IRT at each visit. The IHs in these patients were divided into three groups: stable, slightly growing and growing IHs. We analyzed temperature differences between the groups, relative operating characteristic (ROC) curves, and possible application of this method to clinical practice. RESULTS: The median initial temperatures in the groups were 36.7°C for the stable group, 37°C for the slightly growing group, and 37.4°C for the growing group (P<0.01). The area under the ROC curve for the temperature values to predict growth was 0.929. Temperatures at or above 37.4°C showed a specificity of 95%, a sensitivity of 75%, a positive predictive value 81%, and a negative predictive value of 95%. CONCLUSIONS: IRT is a time and cost effective tool, and is easy to learn. The surface temperature of IH reflects its remaining growth potential and could be used in the outpatient setting for the evaluation and follow-up of IH.

Cloacal reconstruction after a complex treatment of perineal haemangioma in a variant of PELVIS syndrome.

BACKGROUND: PELVIS is an acronym defining the association of perineal hemangioma, malformations of external genitalia, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus and skin tag. Eleven cases have been reported according to the Orphanet data. Acronyms of LUMBAR and SACRAL syndrome have been used and most probably represent a spectrum of the same entity. Very little is known about the success and timing of cloacal reconstruction after the treatment of hemangioma. We present a variant of PELVIS syndrome and discuss the possibilities and optimal timing of surgical reconstruction. CASE PRESENTATION: Female infant was born with persistent cloaca and multiple hemangiomas of genitals, perineal area and left thigh. Colostomy was performed after birth. In order to treat hemangioma and to make the reconstruction of cloaca possible, corticosteroid treatment orally and multiple laser treatments were performed alternating Nd:YAG laser and pulsed dye laser therapy. Cystoscopy confirmed hemangiomatosis in the mucosa of the common channel, bladder neck and septate vagina. Oral propranolol treatment was started at the age of 18 months and continued for 1 year. It induced rapid improvement of hemangiomas. Two more pulsed dye laser treatments were performed to remove residuals of hemangiomas from the perineum and genital area. Posterior sagital reconstruction by separation of the rectum, mobilization of urogenital sinus and vaginal reconstruction was performed with no major bleeding at the age of 4 years. Postoperatively, after a period of progressive rectal dilatation colostomy was closed. Girl is now 6 years old, dry day and night without residual urine and normal upper tracts. Rectal calibration is normal, fecal continence is still to be evaluated but constipation is easily manageable. CT of the spine and the perineum showed sacral dysplasia and spina bifida with lumbo-sacral lipoma and tethering of terminal filum without neurological deterioration at the moment but requiring close neurological monitoring. CONCLUSIONS: Large perineal hemangiomas are commonly associated with extracutaneous abnormalities. Successful reconstructive surgery is possible after significant reduction of hemangioma by complex treatment.

A randomized, controlled trial of oral propranolol in infantile hemangioma.

BACKGROUND: Oral propranolol has been used to treat complicated infantile hemangiomas, although data from randomized, controlled trials to inform its use are limited. METHODS: We performed a multicenter, randomized, double-blind, adaptive, phase 2-3 trial assessing the efficacy and safety of a pediatric-specific oral propranolol solution in infants 1 to 5 months of age with proliferating infantile hemangioma requiring systemic therapy. Infants were randomly assigned to receive placebo or one of four propranolol regimens (1 or 3 mg of propranolol base per kilogram of body weight per day for 3 or 6 months). A preplanned interim analysis was conducted to identify the regimen to study for the final efficacy analysis. The primary end point was success (complete or nearly complete resolution of the target hemangioma) or failure of trial treatment at week 24, as assessed by independent, centralized, blinded evaluations of standardized photographs. RESULTS: Of 460 infants who underwent randomization, 456 received treatment. On the basis of an interim analysis of the first 188 patients who completed 24 weeks of trial treatment, the regimen of 3 mg of propranolol per kilogram per day for 6 months was selected for the final efficacy analysis. The frequency of successful treatment was higher with this regimen than with placebo (60% vs. 4%, P<0.001). A total of 88% of patients who received the selected propranolol regimen showed improvement by week 5, versus 5% of patients who received placebo. A total of 10% of patients in whom treatment with propranolol was successful required systemic retreatment during follow-up. Known adverse events associated with propranolol (hypoglycemia, hypotension, bradycardia, and bronchospasm) occurred infrequently, with no significant difference in frequency between the placebo group and the groups receiving propranolol. CONCLUSIONS: This trial showed that propranolol was effective at a dose of 3 mg per kilogram per day for 6 months in the treatment of infantile hemangioma. (Funded by Pierre Fabre Dermatologie; ClinicalTrials.gov number, NCT01056341.).

Liposomal amphotericin B and surgery as successful therapy for pulmonary Lichtheimia corymbifera zygomycosis in a pediatric patient with acute promyelocytic leukemia on antifungal prophylaxis with posaconazole.

Pulmonary zygomycosis, also referred to as mucormycosis, is a fungal infection of lungs caused by fungi of the order Mucorales in the class of Zygomycetes. It is usually associated with high morbidity and mortality. Here, we report the case of a 14-year-old girl with pediatric acute promyelocytic leukemia (APL) on antifungal prophylaxis with posaconazole, who developed pulmonary Lichtheimia corymbifera (formerly Absidia corymbifera) zygomycosis. She was successfully treated by means of liposomal amphotericin B (L-AmB) and surgery. To our knowledge, this is the first published report on pediatric APL and pulmonary zygomycosis in the English language literature. At present, the patient is in complete remission of her APL and without any signs of recurrence of zygomycosis. This report suggests that efficient diagnostics, increased physician awareness, and reliance on adjunctive surgical therapy can result in a favorable outcome of pulmonary zygomycosis in immunocompromised children with hematological malignancies.

[Congenital respiratory cystic dysplasias in children]. / Vaiku kvepavimo trakto igimtos cistines displazijos.

UNLABELLED: Diagnosis and treatment of congenital respiratory cystic dysplasias - more complicated than acquired pulmonary diseases. In 20% of cases treatment should be started in newborn age because of respiratory distress syndrome. MATERIAL AND METHODS: During the period of 1990-2002 we have diagnosed and treated 30 patients due to congenital respiratory cystic dysplasias. Clinical symptoms, diagnostic methods, indications for the operation and results of the surgical treatment were analyzed. RESULTS: We operated on 18 boys and 12 girls. Age of the patients - 6 newborns, 8 infants, 3 children from 1 till 3 years, 13 patients from 7 till 15 years. Lobectomy was performed for 13 patients, segmentectomy in 1 case, and removal of the cyst in 14 cases. In 1 case videotoracoscopy and in 1 case angiography was made. All patients survived. Other diseases were found in 16 cases. CONCLUSIONS: Congenital respiratory cystic dysplasias are serious respiratory disease of newborns and children which can cause respiratory distress syndrome. 1. Because of respiratory distress, possible infection and malignant transformation surgical treatment is recommended. 2. Antenatal diagnosis of congenital respiratory cystic dysplasias could enable the neonatologist and surgeon to prepare for the treatment, because in 20% of cases the treatment is necessary in early postnatal period.

[Pulmonary sequestration]. / Plauciu sekvestracija.

UNLABELLED: Pulmonary sequestration is rare and serious dysplasia, which has clinical signs of pneumonia and respiratory distress syndrome and radiological signs of polymorphic shadows. Pulmonary sequestration masses have autonomic blood supply. For confirmation of the diagnosis we use ultrasound examination, CT scan, MRI, angiopulmography and aortography. AIM OF THE STUDY: To present pathogenesis, diagnostic features and surgical treatment tactics of rare and serious pulmonary dysplasia. MATERIAL AND METHODS: During the period of 1979-2002, we have examined and treated 9 patients because of pulmonary sequestration. We analyzed clinical signs, results of the examination before operation, indications for operation and results of surgical treatment. RESULTS: Seven patients after the operation recovered, 1 patient died due to infection and cardiovascular complications. One patient is supposed to be operated on in the near future. CONCLUSIONS: 1. Pulmonary sequestration is rare and serious bronchial - pulmonary dysplasia, which has clinical signs of pneumonia and respiratory distress syndrome and radiological signs of polymorphic shadows. 2. Pulmonary sequestrations are classified as intralobar, extralobar and extrapulmonary sequestration. 3. Pulmonary sequestration is often associated with cardiovascular dysplasias and diaphragmatic malformations. 4. Methods of examination: X-ray examination, CT scan, MRI, ultrasound examination, aortography. 5. Complications of pulmonary sequestration: pneumonia, pleuritis, necrosis of the sequestration, chylothorax, respiratory distress syndrome, hemothorax, cystic transformation, and pneumothorax. 6. Treatment - surgery or embolisation of abnormal supply vessels.