Cas cliniquePELVIS/SACRAL syndrome avec hémangiome livédoïde et bride amniotiquePELVIS/SACRAL syndrome with livedoid haemangioma and amniotic band
Section snippets
Observation
Un nouveau-né de sexe masculin, premier enfant né à terme d’une grossesse normale, subissait le jour de sa naissance une colostomie gauche dans un contexte d’imperforation anale et d’anomalies des organes génitaux externes avec ambiguïté sexuelle : micro-pénis avec verge de 15 millimètres, méat apical avec absence d’hypospadias, scrotum bifide, anomalie d’insertion scrotale et raphé médian. Le sillon interfessier et le sacrum étaient déviés vers la gauche avec une asymétrie des masses
Discussion
L’hémangiome infantile est la plus fréquente des tumeurs vasculaires de l’enfant, avec une prévalence chez 10 % des nourrissons de moins d’un an. Le sexe féminin, la prématurité et les traumatismes placentaires sont des facteurs prédisposants. Le diagnostic est le plus souvent clinique sur la notion d’intervalle libre après la naissance, le profil évolutif biphasique et l’aspect sémiologique caractéristique. L’indication d’une biopsie [1] est limitée aux aspects cliniques inhabituels (formes
Conclusion
Un PELVIS/SACRAL syndrome doit être recherché systématiquement devant un hémangiome segmentaire concernant tout ou une partie du territoire recouvert par les langes. De même, un tableau de dysraphie caudale associant de façon variable anomalies osseuses et médullaires lombosacrées, anales et urogénitales doit évoquer un PELVIS/SACRAL syndrome, en sachant que l’hémangiome peut être de diagnostic retardé ou difficile du fait d’un aspect réticulé atypique. La dysraphie périnéale (organes génitaux
Références (17)
- et al.
GLUT1: a newly discovered immunohistochemical marker for juvenile hemangiomas
Hum Pathol
(2000) - et al.
Extensive perineal infantile hemangioma with associated congenital anomalies: An example of the PELVIS Syndrome
J Pediatr Adolesc Gynecol
(2007) - et al.
Cutis marmorata telangiectatica congenita in unique association with imperforate anus and myelodysplasia
J Am Acad Dermatol
(2005) - et al.
Not all hamangiomas look like strawberries: uncommon presentations of the most common tumor of infancy
Pediatr Dermatol
(1995) - et al.
PELVIS syndrome
Arch Dermatol
(2006) - et al.
PHACE syndrome. The association of posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities
Arch Dermatol
(1996) - et al.
SACRAL hemangiomas and multiple congenital abnormalities
Arch Dermatol
(1986) - Stockman A, Boralevi F, Taïeb A, Léauté-Labrèze C. Perineal hemangioma: retrospective analysis of 49 cases regarding...
Cited by (10)
Role of Magnetic Resonance Image in Children with Lumbosacral and Perineal Hemangiomas: Case Reports and Review of the Literature
2019, Actas Dermo-SifiliograficasCitation Excerpt :Some authors who have published reports that associate hemangiomas with internal malformations suggest that patients with large hemangiomas of the perineal area, even if neurologically asymptomatic, should have a kidney, abdominal and medullar US performed to rule out a malformation18; others propose a spine MRI to detect spinal dysraphism14 and a pelvic MRI to detect other internal anomalies,16,19 without clarifying the most appropriate moment of these studies. Other authors also suggest that every infant with lumbosacral segmental hemangiomas should undergo a systematic research of OSD and should have performed a medullar US before the 4 months of age, ideally in the neonatal period, and a lumbosacral MRI when the infant is older than 4 months17,20 in order to avoid late neurologic symptoms that can be hardly treated.21,22 In 1989, Albright et al.3 described 6 patients with vast perineal hemangiomas studied with MRI and 1 with myelogram.
Reticular infantile hemangiomas with minimal or arrested growth associated with lipoatrophy
2015, Journal of the American Academy of DermatologyCitation Excerpt :There is confusion in the literature about the use of the term “reticular infantile hemangioma” because, over the years, it has been considered as a synonym for IH-MAG3 or described as either “superficial telangiectatic/thin”4 or “abortive or minimal-growth” IH5-7 to take into account the flat telangiectatic surface and the absence of, or very limited, proliferative growth. The term “livedoid” IH8 has also been proposed. When clinical and immunohistochemical evidence suggests an abortive or MAG IH,5 the term “reticular” should be retained to designate this particular morphologic subtype.
PELVIS or LUMBAR syndrome: The same entity. Two case reports
2012, Archives de PediatrieNormal and abnormal prepuce
2020, Normal and Abnormal PrepuceInfantile hemangiomas: Our current understanding and treatment options
2018, Dermatology Online JournalVascular tumors and anomalies
2016, Moss and Adams' Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adult: Ninth Edition