Les anomalies vasculaires pendant l'enfance : quand traiter les patients et quand les diriger vers une ressource spécialisée.

Les anomalies vasculaires sont des affections hétérogènes qui touchent les vaisseaux sanguins ou lymphatiques. Les enfants atteints peuvent éprouver de la douleur ou une perte fonctionnelle, présenter une infection ou une coagulopathie ou être confrontés à des difficultés psychologiques. Le diagnostic et la prise en charge exigent souvent une approche interdisciplinaire. Sept cliniques d'anomalies vasculaires au Canada offrent des soins interdisciplinaires. Le présent point de pratique propose une approche thérapeutique des anomalies vasculaires pédiatriques les plus fréquentes (hémangiomes). On y passe en revue les indications de diriger les patients vers une clinique spécialisée, en s'attardant sur les anomalies vasculaires complexes, et notamment les hémangiomes infantiles, qui peuvent provoquer des complications.

[Cutaneous signs of occult cranial and spinal dysraphism]. / Signes cutanés des dysraphies occultes cranioencéphaliques et spinales.

Dysraphism refers to neural tube closure abnormalities and midline closure abnormalities of the skin, paravertebral muscles, vertebrae and meninges. Cranial dysraphism (CD) and occult spinal dysraphism (OSD) may be discovered via evocative skin signs present at birth or appearing later in childhood or even in adulthood. This review describes the various types of skin signs associated with CD and OSD. All congenital midline skin lesions, particularly on the frontonasal area, the vertex or the occipitocervical and low back regions, should prompt suspicion of underlying dysraphism. The main evocative midline skin abnormalities are: (i) for underlying DCEO: a nodule, swelling, skin openings and hair collar sign or hair tuft; (ii) for underlying DSO, localized hypertrichosis, an atypical or complex lower back dimple, a dermoid fistula, infantile haemangioma, caudal appendage and lipoma. In the event of suspected DCEO or DSO, spinal or medullary MRI constitutes the reference examination.

[What's new in pediatric dermatology?] / Quoi de neuf en dermatologie pédiatrique?

In order to describe the latest news in Pediatric dermatology (pathophysiology, clinical aspects and therapy), a literature review was performed from September 2017 to September 2018. This article is not an exhaustive review but present the new data that may infuence the daily practice. The results are presented by disease that may be common or rare. Some references are available as supplements.

[What's new in pediatric dermatology?] / Quoi de neuf en dermatologie pédiatrique ?

The year 2017 in pediatric dermatology was marked by several consensus recommendations and meta analyzes on childhood psoriasis, atopic dermatitis or PHACE syndrome, case series on the role of anti-JAK treatment in adolescent with alopecia areata, sirolimus for vascular malformations, ivermectine for rosacea, inhibitors of MEK for type 1 neurofibromatosis or on the side effects of the oral isotretinoin for acne or propranolol for immature hemangioma. Only few randomized controlled studies have been published on the interest of adalimumab in the treatment of psoriasis or topical sirolimus for angiofibroma in tuberous sclerosis complex for example. There were also clinical articles on various affections such as the sign of the scalp hair collar sign, childhood prurigo, ichthyosis, atopic dermatitis, warts or Zika virus infection. Finally, numerous publications reported new genes responsible for dermatosis in mosaics with new questionings and perspectives.

[Principles of plastic pediatric surgery: Thirty years of surgical practice]. / Principes généraux de la chirurgie plastique pédiatrique. Expérience tirée de 30ans de pratique chirurgicale.

In this document, Pr Guy Magalon presents a reflection on the plastic pediatric surgery drawn from 30 years of surgical practice. His thinking is supported by several examples of plastic surgery results from children reviewed in adulthood. These cases highlight the evolution of surgical practices and the need for continuing education for surgeons to adapt to technical progress. Professor Magalon honors his masters and shares his personal vision of the principles of pediatric plastic surgery.

[Infantile segmental hemangioma without facial involvement: A cutaneous marker of vascular malformations such as in PHACE syndrome?]. / Hémangiome infantile segmentaire extra-facial : un marqueur cutané de malformations vasculaires comme au cours du syndrome PHACE ?

BACKGROUND: Herein we report a case of a possible PHACE syndrome without hemangioma of the head but with a large segmental hemangioma of the trunk. PATIENTS AND METHODS: A 17-year-old female patient with a medical history of transposition of the great arteries with ventricular septal defect diagnosed at 3 days of life and of coarctation of the aorta diagnosed at 14 years was seen in the dermatology department for a long-standing large rectangular, segmental, atrophic and telangiectasic lesion on her back. The lesion appeared to be a sequel of infantile segmental hemangioma of the trunk, and this was confirmed by history-taking. DISCUSSION: This case raises the question of a link between infantile segmental hemangioma and underlying cardiovascular disorders. Infantile segmental hemangioma could be a marker of an underlying vascular development defect. The presence of infantile segmental hemangioma, regardless of site, should prompt vascular explorations.

[What's new in pediatric dermatology?]. / Quoi de neuf en dermatologie pédiatrique?

This article is a selection of the most significant developments in the field of pediatric dermatology through an analysis of the articles published between October 2012 and October 2013. In the field of vascular anomalies, propranolol remains a topic of interest for infantile hemangiomas. New clinical concepts appear in the field of vascular malformations in parallel to genetic progress in this area. New epidemiological data or new pathophysiological concepts apply to atopic dermatitis. Congenital or atypical nevi of the child benefit from genetic progress or improvement of clinical knowledge. Although rare, melanoma of the child concerns by its increasing incidence and its misleadingclinical characteristics. Other data reported here relate to infectious skin of the child, morpheas, neurofibromatosis type 1, psoriasis and other commonly seen dermatoses in children.

[Hemangioma of the tongue: Experience in Mali]. / Prise en charge d'un hemangiome de la langue : Experience au Mali.

PURPOSE: To study the diagnostic and therapeutic aspects of hemangioma of the tongue. OBSERVATION: A 65 year old man admitted to ENT for a swelling of the tongue that appeared two years ago and progressively increased in volume leading to permanent protrusion. The swelling took up the entire anterior third of the tongue. It had a reddish appearance. On palpation, it was a rounded, firm, well-limited, slightly sensitive mass, measuring 5 cm in diameter. The rest of the ENT examination was unremarkable. Lingual CT scan revealed a very limited mass with hyperechogenic content that did not increase in size after injection of the contrast agent. Surgical excision was performed and the postoperative follow-up was simple. Histology concluded that there was a hemangioma of the tongue. CONCLUSION: Hemangioma of the tongue is a rare pathology. It must be considered in front of any lingual mass in adults. Its positive diagnosis is clinical and histological.

BUT: Etudier les aspects diagnostiques et thérapeutiques de l'hémangiome de la langue. OBSERVATION: Un homme de 65 ans admis en ORL pour une tuméfaction de la langue apparue depuis deux ans ayant progressivement augmenté de volume entrainant sa protrusion permanente. La tuméfaction prenait toute le tiers antérieur de la langue. Elle était d'aspect rougeâtre. A la palpation, il s'agissait d'une masse arrondie, ferme, bien limitée, légèrement sensible, mesurant 5 cm de grand diamètre. Le reste de l'examen ORL était sans particularité. La TDM linguale a objectivé une masse bien limitée à contenu hyperechogène ne se rehaussant pas après injection du produit de contraste. L'exérèse chirurgicale a été effectuée et les suites opératoires ont été simples. L'histologie a conclu à un hémangiome de la langue. CONCLUSION: L'hémangiome de la langue est une pathologie rare. Il faut y penser devant toute masse linguale chez l'adulte. Son diagnostic positif est clinique et histologique.

Optical coherence tomography angiography findings in retinal cavernous hemangioma: New cases and review of the literature.

INTRODUCTION: Retinal cavernous hemangioma (RCH) is a rare retinal vascular disease characterized by grape-like clusters of saccular aneurysms, usually unilateral, asymptomatic and non-progressive. The diagnosis is made by multimodal imaging including conventional fluorescein angiography (FA). The recent introduction of swept source optical coherence tomography angiography (SS-OCTA) has allowed new insight into vascular diseases, allowing non-invasive, more precise visualization of retinal and choroidal blood flow, and represents a possible alternative to FA. METHODS: We herein describe two cases of RCH with multimodal imaging, including SS-OCTA, and compare our findings with those previously described. RESULTS: On OCTA, the presence of a draining vessel, a reduction in flow signal in the SCP and DCP, and a fluid level can be observed. CONCLUSION: These OCTA signs are in accordance with those described on conventional fluorescein angiography, allowing this invasive exam to be avoided in typical cases.

[Hemangioma of the tongue: Experience in Mali]. / Prise en charge d'un hemangiome de la langue : Experience au Mali.

PURPOSE: To study the diagnostic and therapeutic aspects of hemangioma of the tongue. OBSERVATION: A 65 year old man admitted to ENT for a swelling of the tongue that appeared two years ago and progressively increased in volume leading to permanent protrusion. The swelling took up the entire anterior third of the tongue. It had a reddish appearance. On palpation, it was a rounded, firm, well-limited, slightly sensitive mass, measuring 5 cm in diameter. The rest of the ENT examination was unremarkable. Lingual CT scan revealed a very limited mass with hyperechogenic content that did not increase in size after injection of the contrast agent. Surgical excision was performed and the postoperative follow-up was simple. Histology concluded that there was a hemangioma of the tongue. CONCLUSION: Hemangioma of the tongue is a rare pathology. It must be considered in front of any lingual mass in adults. Its positive diagnosis is clinical and histological.

BUT: Etudier les aspects diagnostiques et thérapeutiques de l'hémangiome de la langue. OBSERVATION: Un homme de 65 ans admis en ORL pour une tuméfaction de la langue apparue depuis deux ans ayant progressivement augmenté de volume entrainant sa protrusion permanente. La tuméfaction prenait toute le tiers antérieur de la langue. Elle était d'aspect rougeâtre. A la palpation, il s'agissait d'une masse arrondie, ferme, bien limitée, légèrement sensible, mesurant 5 cm de grand diamètre. Le reste de l'examen ORL était sans particularité. La TDM linguale a objectivé une masse bien limitée à contenu hyperechogène ne se rehaussant pas après injection du produit de contraste. L'exérèse chirurgicale a été effectuée et les suites opératoires ont été simples. L'histologie a conclu à un hémangiome de la langue. CONCLUSION: L'hémangiome de la langue est une pathologie rare. Il faut y penser devant toute masse linguale chez l'adulte. Son diagnostic positif est clinique et histologique.

[Radiation therapy of hepatic haemangiomas: Review from a case report]. / Radiothérapie externe des angiomes hépatiques : revue de la littérature, à partir d'un cas.

Haemangioma is the most frequent benign hepatic tumour. Haemangioma is generally asymptomatic but it can sometimes cause disabling symptoms depending on its size and location. Surgery and interventional radiology are the cornerstone of the treatment in this situation. Radiation therapy, already used with good efficacy and safety to treat hepatic malignant lesions as hepatocarcinoma and metastases, is a relevant option in case of contraindication to surgery because of multiple or very large lesions. In this context, we report the case of a patient presenting with multiple symptomatic hepatic haemangiomas, successfully treated by radiation therapy in our department. These good results justified a review of the literature to report series of patients treated in this indication and to describe the main treatment regimens used.

Flexor digitorum profundus tendon infiltration by post-traumatic hemangioma. A pediatric case.

Pediatric cases of post-traumatic hemangioma of the palm are rare. Clinical evaluation and imaging are sometimes ambiguous and surgical exploration may show more conclusive results. This case report discusses the diagnosis and treatment of a teenager with post-traumatic hemangioma infiltrating the flexor digitorum profundus tendon of the index finger. Pathologic examination revealed a lobular capillary hemangioma known as pyogenic granuloma.

Cavernous venous malformation of the lacrimal gland.

BACKGROUND: Cavernous venous malformation (CVM) is a common benign vascular lesion of the orbit. It was previously known as "orbital cavernous hemangioma". Localization within the lacrimal gland is extremely rare. CASE PRESENTATION: We describe the case of a 76-year-old man with an asymptomatic CVM of the left lacrimal gland incidentally discovered on a routine MRI. A curative and diagnostic en bloc surgical resection was performed, allowing for histological diagnosis. CONCLUSIONS: CVM of the lacrimal gland is extremely rare and usually asymptomatic. Proptosis is the main symptom. On MRI, the lesion appears hypointense with heterogeneous enhancement after Gadolinium injection on T1-weighted imaging and hyperintense on T2 STIR-weighted imaging. Histological examination is mandatory for the diagnosis. Surgical resection is usually performed.

A child dural-based occipital cavernoma with an accessory venous sinus.

BACKGROUND: The diagnosis of extra-axial cavernoma and surgical decision-making can be difficult on children. REPORTED CASE: In this report we present the case of a 33-month-old child for whom the work up done for asthenia, anorexia and psychomotor regression reveals a well-defined occipital extra-axial lesion. A follow up MRI was performed two months later, revealing an increase in the size of the lesion associated with hemorrhagic changes. The surgical decision is performed after an arteriography; it allows the total removal of the lesion contained in a duplication of the dura mater, by placing a vascular clip on an accessory venous sinus draining the lesion, respecting the integrity of the lateral sinus. Histology confirms a cavernoma. No complication resulted from the surgical procedure. This atypical case of pediatric dural cavernoma associated with a well-defined accessory venous sinus in arteriography is to our knowledge the first description in the literature. CONCLUSION: Extra-axial cavernomas have a misleading presentation. The management of these lesions is of twofold interests: to avoid a repercussion on the development of these children, and to obtain histological confirmation.

Hémangiome vertébral neuro-agressif de révélation tardive: cas cliniqueA clinical case of late-onset aggressive vertebral hemangioma with neurological signs.

Résumé Dans la majorité des cas asymptomatiques, les hémangiomes vertébraux peuvent être, dans de rares cas, symptomatiques avec des manifestations cliniques purement neurologiques. S´ils sont fréquemment observés chez un sujet adulte jeune, ils peuvent exceptionnellement être observés chez un sujet âgé. Nous rapportons un cas d´hémangiome vertébral neuro-agressif de révélation tardive traité par une chirurgie décompressive, une sclérothérapie, une cimentoplastie et suivi d´une évolution favorable. English abstract In the majority of asymptomatic cases, vertebral hemangiomas can be, in rare cases, symptomatic with purely neurological clinical manifestations. They commonly occur in young adults, exceptionally in elderly subjects. We here report a case of late onset aggressive vertebral hemangioma with neurological signs treated with decompressive surgery, sclerotherapy and cementoplasty, with favorable outcome.

[Sclerosing pneumocytoma: A rare and benign tumor]. / Le pneumocytome sclérosant : une tumeur rare et apparemment bénigne.

INTRODUCTION: Sclerosing pneumocytoma is a benign and rare lung tumor affecting epithelial cells. In most cases, patients are asymptomatic and the diagnosis is made on an X ray or a CT scan performed for other enquiry. Sex ratio favors women. Epidemiological studies report that middle-aged Asian women are more frequently affected. Radiological investigations find a solitary nodule or a mass with peripheric localization. When performed, histological analysis shows a tumor composed of at least two of the four following architectures: papillary, sclerosing, hemangiomatous and solid, with two types of cells that can be round or cubic cells. CASES REPORT: We report two cases of multiple sclerosing pneumocytoma in two caucasien men. The first patient was asymptomatic, the second complain from moderate dyspnea. A wedge resection was performed in both, allowing diagnosis. Anatomopathology revealed respectively a predominant sclerosing and solid architecture and a sclerosing and papillary architecture. There was no progression of the other concomitant nodules after three years follow-up. CONCLUSION: Pneumocytoma is a benign, slow-growing tumor with good prognosis.

[A case of multifocal cavernous haemangiomas of the lung, spleen and bone]. / Hémangiomatose multifocale bronchique, ganglionnaire, splénique et osseuse.

INTRODUCTION: Haemangiomas are vascular malformations, frequently cutaneous, hepatic and splenic. Respiratory involvement and multiple localisations are uncommon. CASE REPORT: We present a rare case of multiple cavernous haemangiomas in a 35 year old woman presenting with repeated haemoptysis. Thoracic CT scanning showed a mass in the left lower lobe associated with lymph node enlargement above and below the diaphragm, heterogeneous splenomegaly and a single spinal lesion without hypermetabolism on PET scanning. Enbronchial ultrasound-guided trans-bronchial needle aspiration was not contributory. Histopathological diagnostic was made firstly by splenectomy with lumbar-aortic curettage and then by lobectomy for haemostasis. A final diagnosis of multiple cavernous haemangiomas involving lung, lymph nodes, spleen and bone was made. CONCLUSIONS: Bronchopulmonary cavernous haemangiomas associated with extra-thoracic lesions are exceptionally rare and their presentation, suggesting, a malignant cause, often leads to surgical resection for diagnostic and, eventually, therapeutic management. We report an original case of cavernous haemangiomas involving lung, lymph nodes, spleen and bone.

Intramuscular hemangioma of the hypothenar eminence. A case report.

Intramuscular hemangiomas are benign vascular tumors. They develop in striated muscles. Various sites have been reported in the literature but only rarely in the hand. We report on a case of an intramuscular hemangioma located within the hypothenar eminence, diagnosed by a biopsy. Treatment options are limited in the hand as embolization may lead to a risk of digital ischemia and sclerotherapy may lead to recurrence. This type of infiltrating tumor is poorly structured within the muscle, thus simple removal is not an option. Surgical excision of the entire affected muscle, in our case the abductor digiti minimi, was performed without any consequences on the hand's function and no signs of recurrence at the latest follow-up of 6months.

[The efficacy of multimodal treatment for symptomatic vertebral hemangiomas: A report of 27 cases and a review of the literature]. / Intérêt d'une prise en charge multimodale des hémangiomes vertébraux symptomatiques : à propos de 27 cas et revue de la littérature.

INTRODUCTION: Vertebral hemangiomas (VH) represent the most common primary bone tumor of the spine and are rarely symptomatic. Currently, there is no consensus for treatment and many therapeutic options are available, alone or in combination including cementoplasty, sclerotherapy, surgery, embolization and/or radiotherapy. OBJECTIVE: To evaluate the clinical and radiological outcome of a multimodal management for symptomatic VH. METHODS: A consecutive prospective and retrospective multicenter study was conducted to review cases of symptomatic VHs between 2005 and 2015. Clinical and radiological aspects, treatment modalities and complications were evaluated preoperatively; postoperatively and at last follow-up. We also reviewed the literature of studies concerning case series of VH, published after 1990 and involving more than 10 patients. RESULTS: Twenty-seven VHs were included in our series (mean age at diagnosis: 47.9 years), out of which 26 were symptomatic. Ten presented with neurologic deficit (37%). An epidural extension was noted in 13 patients (48%). Eleven patients (41%) underwent multimodal treatments. In the multimodal group, eradication was observed in 6 patients (54%), stable residue in 5 cases (46%) with no recurrence versus 3 eradication (23%), 9 stable residue (69%) and no recurrence in the monomodal group, (P>0.05). The literature comprised 14 studies including 458 patients. Only 4 studies were focused on multimodal treatments. CONCLUSION: Based on this study, the multimodal management of symptomatic VHs appeared safe and effective. Finally, we propose an algorithm for symptomatic VHS management based on the severity of epidural extension and fracture risk.

[Lombosacral epidural capillary hemangioma mimicking a dumbbell-shaped neurinoma: A case report and review of the literature]. / Hémangiome capillaire épidural lombosacré mimant un neurinome en sablier : à propos d'un cas et revue de la littérature.

INTRODUCTION: Capillary hemangiomas are benign endothelial cell neoplasms that are believed to be hamartomatous proliferations of vascular endothelial cells. The occurrence of spinal epidural capillary hemangiomas is exceedingly rare. Only 8 epidurally located cases of capillary hemangiomas in the spinal canal have been reported in the literature. CASE REPORT: We report for the first time, to our knowledge, a case of lumbosacral epidural capillary hemangioma revealed by S1 back pain and radicular pain in a 60-year-old patient, caused by an L5-S1 epidural capillary hemangioma. The neurological symptoms of the patient improved after surgery. CONCLUSION: Spinal epidural capillary hemangioma is exceedingly rare. These lesions are benign and can mimic dumbbell-shaped neurinoma. Total removal by surgery is curative.