Congenital hemangioma exhibiting postnatal growth.

Congenital hemangiomas are vascular tumors that are fully formed at birth, typically without postnatal growth. Noninvoluting congenital hemangiomas (NICH) have a distinctive clinical, radiologic, and histopathological profile and lack of expansion or involution over time. Herein, we describe two cases of NICH with atypical postnatal growth.

Late growth of infantile hemangiomas in children >3 years of age: A retrospective study.

BACKGROUND: The proliferative phase of infantile hemangiomas (IHs) is usually complete by 9 months of life. Late growth beyond age 3 years is rarely reported. OBJECTIVE: To describe the demographic and clinic characteristics of a cohort of patients with late growth of IH, defined as growth in a patient >3 years of age. METHODS: A multicenter, retrospective cohort study. RESULTS: In total, 59 patients, 85% of which were female, met the inclusion criteria. The mean first episode of late growth was 4.3 (range 3-8.5) years. Head and neck location (55/59; 93%) and presence of deep hemangioma (52/59; 88%) were common characteristics. Posterior fossa malformations, hemangiomas, arterial anomalies, cardiac defects, eye abnormalities (PHACE) syndrome was noted in 20 of 38 (53%) children with segmental facial IH. Systemic therapy (corticosteroid or ß-blocker) was given during infancy in 58 of 59 (98%) and 24 of 59 (41%) received systemic therapy (ß-blockers) for late IH growth. LIMITATIONS: The retrospective nature and ascertainment by investigator recall are limitations of the study. CONCLUSION: Late IH growth can occur in children after 3 years of age. Risk factors include head and neck location, segmental morphology, and involvement of deep dermal/subcutaneous tissues.

Congenital capillary hemangioma arising from palpebral conjunctiva of a neonate.

Capillary hemangioma is the most common benign vascular eyelid tumor in childhood. The periocular lesion appears within the first few weeks after birth and usually has superficial or deep components. Primary conjunctival capillary hemangiomas are rarely reported. We present the case of a 2-day-old child with a pedunculated capillary hemangioma arising from superior palpebral conjunctiva. A complete surgical excision was performed under general anesthesia, and the child was asymptomatic at follow-up of two months.

Hemangiomatosis capilar pulmonar congénita en un recién nacido / Congenital pulmonary capillary hemangiomatosis in a newborn

La hemangiomatosis capilar pulmonar es una entidad poco frecuente, caracterizada por la proliferación de capilares que infiltran paredes alveolares, septos interlobulillares, pleura e intersticio pulmonar, sin características de malignidad, con asociación casi constante a hipertensión pulmonar. Hasta el momento, solo se han reportado en la literatura, dos casos de presentación congénita; este es el tercer caso en un recién nacido y que no se asocia a hipertensión pulmonar. Esta se encuentra en la mayoría de los pacientes con dicha patología, con mayor incidencia entre los 20 y los 40 años de edad. Se presenta a un recién nacido pretérmino de 36 semanas de gestación con dificultad respiratoria progresiva, que requirió asistencia ventilatoria mecánica por desaturaciones constantes en su evolución clínica, sin signos clínicos, radiológicos o ecográficos de hipertensión pulmonar.

Pulmonary capillary hemangiomatosis is a rare entity characterized by the proliferation of capillaries into alveolar walls, interlobular septa, pleura and pulmonary interstitium, without malignant characteristics, with almost constant association with pulmonary hypertension. Until now two cases of congenital presentation have been reported in the literature. This is the third case in a newborn; he has not followed the usual pattern associated with pulmonary hypertension as occurs in most patients with this pathology; the highest incidence is among 20-40 years old. We report a preterm newborn patient of 36 weeks of gestation with progressive respiratory distress requiring mechanical ventilation by constant desaturation during his clinical evolution without clinical, radiological or ultrasonographic signs of pulmonary hypertension.

[Congenital pulmonary capillary hemangiomatosis in a newborn]. / Hemangiomatosis capilar pulmonar congénita en un recién nacido.

Pulmonary capillary hemangiomatosis is a rare entity characterized by the proliferation of capillaries into alveolar walls, interlobular septa, pleura and pulmonary interstitium, without malignant characteristics, with almost constant association with pulmonary hypertension. Until now two cases of congenital presentation have been reported in the literature. This is the third case in a newborn; he has not followed the usual pattern associated with pulmonary hypertension as occurs in most patients with this pathology; the highest incidence is among 20-40 years old. We report a preterm newborn patient of 36 weeks of gestation with progressive respiratory distress requiring mechanical ventilation by constant desaturation during his clinical evolution without clinical, radiological or ultrasonographic signs of pulmonary hypertension.

La hemangiomatosis capilar pulmonar es una entidad poco frecuente, caracterizada por la proliferación de capilares que infiltran paredes alveolares, septos interlobulillares, pleura e intersticio pulmonar, sin características de malignidad, con asociación casi constante a hipertensión pulmonar. Hasta el momento, solo se han reportado en la literatura, dos casos de presentación congénita; este es el tercer caso en un recién nacido y que no se asocia a hipertensión pulmonar. Esta se encuentra en la mayoría de los pacientes con dicha patología, con mayor incidencia entre los 20 y los 40 años de edad. Se presenta a un recién nacido pretérmino de 36 semanas de gestación con dificultad respiratoria progresiva, que requirió asistencia ventilatoria mecánica por desaturaciones constantes en su evolución clínica, sin signos clínicos, radiológicos o ecográficos de hipertensión pulmonar.

[Clinicopathologic features and differential diagnoses of non-involuting congenital hemangioma in children].

OBJECTIVE: To investigate the clinicopathologic features and differential diagnoses of non-involuting congenital hemangioma (NICH) in children. METHODS: The clinical, morphologic and immunophenotypic characteristics of 22 cases of NICH were retrospectively analyzed. RESULTS: The mean patients' age at diagnosis was 4.2 years, with a male to female ratio of 1.75:1. The tumors were located in the head and face (5 cases), neck (3 cases), body (6 cases), upper limbs (5 cases), and lower limbs (3 cases). Histologically, the tumor was dominated by rather large lobules of small vessels that were mostly rounded, curved, small and thin-walled, and were lined by endothelial cells surrounded by one or more layers of pericytes. The center of the lobules was occupied by one or more thin or thick walled vessels, which were surrounded by fibrous and fatty tissue, which contained abnormal arterial and venous structures. At the edge of the lobules there were lymphatic vessels. Immunohsitochemical study showed that tumor cells in NICH were positive for CD34 (22/22), CD31 (22/22), SMA (22/22), vimentin (22/22) and Glut1 (0/22). D2-40 expression was located at the edge of the capillary lobules. CONCLUSIONS: NICH is a benign lesion. Clinically and pathologically, it needs to be differentiated from rapidly involuting congenital hemangioma, infantile hemangiomas, tufted angioma, vascular malformation, and others.

Oral Nadolol for Children with Infantile Hemangiomas and Sleep Disturbances with Oral Propranolol.

BACKGROUND/OBJECTIVE: Oral propranolol has been shown to be safe and effective in infants with infantile hemangioma (IH). Side effects such as sleep disturbances have been associated with propranolol. The aim of this study was to evaluate the efficacy and safety of oral nadolol in a small series of patients whose propranolol therapy was discontinued due to sleep disturbances. METHODS: A retrospective study of patients with IHs who were treated with oral nadolol due to propranolol-related sleep disturbances at a pediatric tertiary care center between July 2008 and March 2013. Clinical response to oral nadolol and disappearance of propranolol-related side effects were analyzed. RESULTS: A total of 97 patients presenting IH received oral propranolol. Nine patients (9.3%) developed sleep disturbances. Oral propranolol was discontinued in seven patients and switched to oral nadolol, with resolution of these side effects in 5 (71%) of the cases. One patient developed sleep disturbances again after four months of oral nadolol. LIMITATIONS: The sample size was too small to draw generalizable conclusions and to draw any statistical inference as to the incidence of sleep disturbances with nadolol therapy. CONCLUSIONS: The use of oral nadolol in the treatment of IH in our series of 7 patients, resolved the propranolol-related sleep disturbances in 5 (71%), while in one patient the symptoms recurred after 4 months of oral nadolol at a dose of 2 mg/kg/day. In most cases, switching beta-blockers did not compromise efficacy, and is recommended when sleep disturbance necessitates discontinuation of beta-blocker therapy of IH.

Blood Pressure Monitoring During the Induction and Maintenance Period of Propranolol Therapy for Complicated Infantile Hemangiomas: A Prospective Study of 109 Infants.

BACKGROUND: Propranolol has become the first-line treatment for complicated infantile hemangiomas (CIHs) worldwide. Recommendations for monitoring infants undergoing propranolol therapy vary. Data on long-term blood pressure (BP) monitoring have not been reported before. OBJECTIVE: The objective of the current study was to monitor BP in full-term infants during the induction and maintenance phase of propranolol therapy. METHODS: BP was monitored prospectively in 109 infants (mean age 2.8 mos, range 1-5 mos) with CIHs during the induction (3-4 days in the hospital during up-dosing from 0.5 to 2.0 mg/kg/day) and maintenance (6 mos) phases of oral propranolol therapy. RESULTS: Four children were excluded from the study because of sinus bradycardia (n = 2 [1.8%]) or lethargy (n = 2 [1.8%]). Mean systolic BP (SBP) decreased by 5 mmHg with the increase in propranolol dosage. Low (<5th percentile) SBP or diastolic BP (DBP) was observed in 2 of 105 children (1.9%) each. During the maintenance phase, 2 of 105 children (1.9%) had occasional SBP readings of less than 70 mmHg. No hypotension was observed after the third month of therapy. Low DBP (<36 mmHg) was recorded in 16 (15.2%) children after the first month, in 8.6% after the second, and in 2.9% during the third and fourth months of therapy. No patients exhibited clinical hypotension, bradycardia, or other known side effects of propranolol. Clinical response to therapy was excellent. LIMITATIONS: Reference BP values were derived from published tables, not from an untreated control group. CONCLUSIONS: In healthy full-term infants, propranolol (2 mg/kg/day divided in three doses) is well tolerated. No clinically significant hypotension was observed. We conclude that for otherwise healthy infants, BP monitoring during long-term propranolol therapy for CIHs is not necessary.

Impact of Screening for Hepatic Hemangiomas in Patients with Multiple Cutaneous Infantile Hemangiomas.

OBJECTIVES: Hepatic hemangiomas are often found in association with multiple cutaneous infantile hemangiomas. Screening abdominal ultrasonography has been recommended for patients with five or more cutaneous lesions. We sought to determine whether hemangiomas found through screening had improved clinical outcomes. METHODS: Patients entered into our hepatic hemangioma registry between 1995 and 2012 were reviewed. RESULTS: Seventy-two patients with multiple cutaneous and hepatic hemangiomas were identified; 43 (60%) were detected through screening. The median age at diagnosis was 41 days for screened patients and 53 days for those not screened. Screening detected 40 (93%) multifocal and 3 (7%) diffuse hemangiomas, compared to 18 (62%) and 11 (38%), respectively, in the nonscreened group. Patients identified by screening had lower incidences of congestive heart failure and hypothyroidism and were less likely to receive treatment for their hemangiomas. The mortality rate in the children not screened was 28% (n = 8). None of the patients found by screening died (p < 0.001). Multivariate analysis of treated patients demonstrated that screening was a significant predictor of reduced mortality (p = 0.04). CONCLUSION: Hepatic hemangiomas found through screening ultrasonography are less likely to develop serious clinical sequelae. Although the reasons for this may include detection of hemangiomas that are less likely to progress to symptomatic disease, it appears that it also allows for earlier intervention for more concerning (e.g. diffuse) subtypes. Screening may allow for closer surveillance and earlier treatment before life-threatening progression in a subset of infants with liver hemangiomas, preventing complications and reducing mortality.

[High frequency electrocoagulation for treating noninvoluting congenital hemangioma].

OBJECTIVE: To investigate the clinical efficiency of electrocoagulation for the treatment of noninvoluting congenital hemangioma. METHODS: Sixteen infants with noninvoluting congenital hemangioma who were admitted to our hospital from January 2011 to June 2013 were included in this study. Color Doppler ultrasound was used to determine the hemangioma location, as well as its size and depth. High frequency electrocoagulation was adopted for the treatment. The output power was set at 10-20 W. The probes were inserted around the tumor or at the surface of the tumor. After switching on for 1-2 seconds, the direction and position of the probe was modulated until covering the whole tumor. After the treatment, the absorption of tumor was about 3-6 months. The efficiency was evaluated during the follow-up. RESULTS: Tumor atrophy was obvious after treatment in all patients. The temperature around the tumor mass was decreased, and the aberrant blood signals were decreased under the ultrasonic examination. Complete or partial atrophy were observed. The efficiency was graded as level I, II, III, IV in 0, 2, 9 and 5 patients, respectively. One patient showed local infection due to improper nursing, which was completely relieved after corresponding treatment. No severe adverse events were observed. CONCLUSIONS: High-frequency electrocoagulation is effective for treating noninvoluting congenital hemangioma through coagulating the aberrant blood vessels in the tumor, interrupting the vascular endothelial cell, blocking the aberrant blood flow, as well as leading to atrophy and absorption of tumor mass. Besides, no obvious scar is observed after the surgery.

Surgically intractable epilepsy associated with focal cortical dysplasia and congenital cutaneous hemangiomas.

We describe a 6-month-old girl with medically intractable seizures, multiple congenital hemangiomas, and developmental delay. The patient underwent two surgical resections. Pathological findings at both the first and second resections were consistent with focal cortical dysplasia. The literature was reviewed on focal cortical dysplasia associated with cutaneous hemangiomas.

Atenolol versus propranolol for the treatment of infantile hemangiomas: a randomized controlled study.

BACKGROUND: Infantile hemangiomas have a dramatic response to propranolol, a nonselective beta-blocker. However, this treatment is not risk-free and many patients are excluded because of respiratory comorbidities. Atenolol is a cardioselective beta-blocker that may have fewer adverse events. OBJECTIVE: We sought to evaluate the effectiveness of atenolol against propranolol in a noninferiority trial. METHODS: In all, 23 patients met the inclusion criteria and were randomized to receive either atenolol or propranolol. Thirteen patients were treated with atenolol and 10 with propranolol. Follow-up was made at baseline, 2 weeks, 4 weeks, and then monthly for 6 months. RESULTS: Patients treated with atenolol had a complete response of 53.8% and 60% with propranolol, respectively. These results were nonsignificant (P = .68). Relevant adverse events were not reported. LIMITATIONS: The reduced number of patients could have influenced our results. CONCLUSION: Atenolol appears to be as effective as propranolol. We did not find significant differences between these results or any adverse events.

Giant intracranial hemangioma in a neonate.

In this report we detail the case of an infant presenting with a giant intracranial congenital hemangioma and describe the clinical features and surgical management. Congenital hemangiomas are benign vascular tumors that typically present as skin lesions in neonates and infants. On rare occasions they present as intracranial tumors. The possibility that these tumors may undergo spontaneous regression poses a treatment dilemma.

Propranolol treatment in life-threatening airway hemangiomas: a case series and review of literature.

OBJECTIVES: Infantile hemangiomas (IHs) in the airway may be potentially life-threatening during the proliferative phase. Available treatments like oral corticosteroids (OCS) and chemotherapeutic agents usually showed variable responses and serious side effects. Propranolol is a new and promising treatment option. METHODS: A case series of five IH patients with airway involvement is presented, supplemented with a review of literature. Propranolol treatment (2.0-3.0mg/kg/day) was initiated between 3 weeks and 6 months of age. Three cases were treated with propranolol monotherapy, 2 cases with OCS primarily and propranolol secondarily, in which treatment with OCS could be reduced rapidly. RESULTS: In our case series a dramatic, fast response was observed in all cases, with a permanent effect after discontinuation in four cases. In one patient a relapse of airway problems occurred two months after discontinuation of propranolol at 16 months of age; this resolved after re-start of propranolol. Review of literature together with these five cases showed 81 patients with airway IHs treated with propranolol. Propranolol was effective in 90% of the cases and seven patients were classified as non-responders. Eight IHs relapsed while weaning of propranolol or after discontinuation; dose adjustment or restart was effective in most cases but one patient appeared resistant to therapy. CONCLUSIONS: Propranolol seems to be a rapidly effective and safe treatment strategy for most IHs obstructing the airway. Because of the fast and important effects of propranolol, randomized controlled trials are hardly justifiable for this specific, relatively rare but, acute treatment indication. Despite the efficacy of propranolol, close monitoring of the patients with an airway IH is required, considering the risk of relapse of symptoms during or after treatment and the reported resistance to propranolol in at least 9% of the published cases. The dose and duration of treatment should be high and long enough to prevent relapse. Further research should focus on the optimal treatment protocol; the actual percentage of non-responders and also the mechanism of resistance to propranolol is unknown and needs to be illuminated.

[Rapid regression of infantile haemangioma with 2% propranolol ointment]. / Régression rapide d'un hémangiome infantile sous propranolol topique à 2 %

BACKGROUND: Oral propranolol produces dramatic results in severe infantile haemangioma. We report a case of rapid and significant regression of an infantile haemangioma in infant treated topically with 2% propranolol ointment. PATIENTS AND METHODS: A female infant aged 11 weeks was seen as an outpatient for two infantile haemangiomas on the front of the left knee and the vulva. After parental consent was given, the child was treated with a topical preparation of 2% propranolol ointment prepared by the pharmacy. Regression was rapid and on the 45th day, lesion size had been reduced by an estimated 75%. No adverse effects were observed. DISCUSSION: Topical propranolol appears to be effective and safe for the treatment of superficial haemangiomas. Clinical trials are required to determine the optimal dosage and pharmaceutical form, method of use and treatment duration.