RESUMO
BACKGROUND: Infantile hemangiomas (IHs) can be part of PHACE (posterior fossa anomalies, hemangioma, arterial anomalies, cardiac anomalies, eye anomalies) syndrome when they are segmental, extensive, and located on the face or neck. The initial assessment is codified and well known, but there are no recommendations for the follow-up of these patients. The aim of this study was to assess the long-term prevalence of different associated abnormalities. METHODS: Patients with a history of large segmental IHs of the face or neck. diagnosed between 2011 and 2016 were included in the study. Each patient underwent an ophthalmological, dental, ENT (ear, nose, and throat), dermatological, neuro-pediatric, and radiological assessment at inclusion. Eight patients including five with PHACE syndrome were prospectively evaluated. RESULTS: After a mean follow-up of 8.5 years, three patients presented with an angiomatous aspect of the oral mucosa, two with hearing loss, and two with otoscopic abnormalities. No patients developed ophthalmological abnormalities. The neurological examination was altered in three cases. Brain magnetic resonance imaging follow-up was unchanged in three out four patients and revealed atrophy of the cerebellar vermis in 1 patient. Neurodevelopmental disorders were found in five of the patients and learning difficulties were observed in five patients. The S1 location appears to be associated with a higher risk of neurodevelopmental disorders and cerebellar malformations, while the S3 location was associated with more progressive complications, including neurovascular, cardiovascular, and ENT abnormalities. CONCLUSION: Our study reported late complications in patients with a large segmental IH of the face or neck, whether associated with PHACE syndrome or not, and we proposed an algorithm to optimize the long-term follow-up.
Assuntos
Coartação Aórtica , Anormalidades do Olho , Hemangioma , Síndromes Neurocutâneas , Humanos , Criança , Lactente , Estudos Prospectivos , Seguimentos , Anormalidades do Olho/diagnóstico , Anormalidades do Olho/complicações , Anormalidades do Olho/patologia , Coartação Aórtica/complicações , Coartação Aórtica/diagnóstico , Coartação Aórtica/patologia , Síndromes Neurocutâneas/diagnóstico , Síndromes Neurocutâneas/complicações , Síndromes Neurocutâneas/patologia , Hemangioma/diagnóstico , Hemangioma/patologia , SíndromeRESUMO
The aim of this study was to evaluate the diagnostic accuracy of mobile cone beam computed tomography (MCBCT) versus multi-detector computed tomography (MDCT) in orbital floor fractures. Twenty-four fresh cadaver heads were used, and one orbital floor was fractured for each head by transconjunctival approach. MDCT and MCBCT were performed on each of the heads. The images obtained were then analysed independently by eight evaluators. The radiological characteristics of the orbital floor fractures were visualized with good interpretation agreement between the two images. The location of the fracture and enophthalmos were identified in a comparable manner with strong agreement (κ=0.93 and κ=0.85, respectively). Measurements of fatty hernias and bone defects showed a strong correlation between the two imaging modalities (Pearson coefficient between 0.64 and 0.71 and between 0.67 and 0.71, respectively). The fracture limits and the presence of bone fragments, an intrasinus fatty hernia, and a fracture of the associated medial orbital wall were visualized in both examinations with good agreement (κ=0.68, κ=0.51, κ=0.57, and κ=0.46, respectively). The soft tissue study showed superiority for MDCT, with a κ<0.0009. MCBCT showed good diagnostic performance in the study of orbital floor fracture characteristics.