Segmental facial infantile haemangiomas in the era of propranolol: evaluation at 6 years of age

Abstract

Background The long‐term evolution of children with segmental facial infantile haemangioma (SFIH) treated with propranolol remains unstudied. Objectives The objective of this study was to evaluate the neurodevelopmental features of children with SFIH treated with propranolol at 6 years of age. Methods This retrospective case series study was conducted from January 2008 to June 2020 using data from medical files, patient examinations and appointments spanning 6 years. To be included, patients should present SFIH and have previously received propranolol. A complete physical examination, magnetic resonance imaging (MRI) of the head, echocardiography and ophthalmologic examination should have been performed. Neurodevelopmental features were divided into cognition, audition, vision, orality, motor skills and the occurrence of new symptoms. Results Thirty children with SFIH were included. Of these, 11 presented criteria of PHACES. Evaluation of neurodevelopmental features of the children at 6 years of age showed learning difficulties in one case but grade skipping in three cases. There were six cases of unilateral hearing loss that had not been diagnosed at birth, two of oral difficulties and one of minor hypotonia. Early headache was primarily reported as the main new outcome. All children were treated with propranolol, with three following oral steroid therapy. No severe adverse effects were reported. The median length of treatment with propranolol was 16 months, and the median age at treatment cessation was 21 months. Analysis based on segment implication showed the median length of treatment to vary from 12 months (if S3 was spared) to 25 months (if at least S3 was involved). Vascular laser therapy was used in 16 patients (53.3%) and surgery in four. Conclusion In this case series, children with SFIH, including patients with PHACES criteria, presented a good tolerance of propranolol, as well as encouraged neurodevelopmental data. Segmental implication appears to have a significant impact on treatment duration and associated complications.

Medical management of vascular anomalies of the head and neck

“…Bradycardia is more rarely observed, and asymptomatic hypotension and diarrhea are sometimes present at the start of treatment. Propranolol is also the first‐line medical treatment for PHACE syndrome, 13 as short‐ and long‐term safety data have been found to be reasonable 10,13 …”

“…A recent study on large facial IHs showed that the median length of propranolol treatment was 16 months with a median age at treatment cessation of 21 months, and this was extended to 25 months if the V3 segment was involved (i.e., location in the beard area). 10 Unlike corticosteroids, resistance to treatment is rare and late treatment of IHs beyond the proliferative phase is also possible. 11 The acceptability and tolerance of propranolol is usually good in infants.…”

“…Propranolol is also the first-line medical treatment for PHACE syndrome, 13 as short-and long-term safety data have been found to be reasonable. 10,13 Other beta-blockers including nadolol, 14 acebutolol, 15 and atenolol 16 have worked successfully in small series of IH patients. To date, however, there is not sufficient data to conclude these beta-blockers are superior, nor a have better tolerance, moreover none of these molecules have a marketing authorization for this indication.…”

“…In clinical studies, complete or almost complete disappearance of the IH was observed in 60% of cases after 6 months of treatment 4 and in up to 75% of cases if the treatment was given until 1 year of age 8 . When stopping propranolol, a relapse of the IH was observed in 10% to 15% of cases, and most often the regrowth was moderate and responsive to a further course of propranolol if necessary 9,10 . Relapses are frequently observed in large facial IHs, and the standard of care is to maintain treatment until 1 year of age in these patients.…”

The riddle of PHACE syndrome

“…After 25 years, reports of cases of PHACE syndrome have multiplied and the list of associated malformations is getting longer and longer 4 and includes both organs originating from the ectoderm (such as the cerebellum) and endoderm (such as the thyroid) and not only from the mesoderm, which is the embryonic tissue from which the vessels originate.…”