ABSTRACT
We present a case of a 41-day-old girl with diffuse hemangiomatosis on her chin, right thigh, occipital area, and liver. She also had hepatomegaly, heart failure, and hypothyroidism. Hemangiomatosis did not respond to 2 weeks of oral administration of prednisolone, but was successfully treated with propranolol. After 2 years of propranolol treatment, the lesions of hemangiomatosis nearly disappeared. Hypothyroidism was controlled by levothyroxine replacement. As hemangiomatosis regressed, thyroid function was normalized. Propranolol may have adverse effects including hypotension, hypoglycemia, bronchoconstriction, and restlessness, but she did not experience such adverse effects. Propranolol could be the first choice for diffuse neonatal hemangiomatosis (DNH). Our case also suggests that thyroid function test is needed in patient with DNH.
Subject(s)
Female , Humans , Administration, Oral , Bronchoconstriction , Chin , Heart Failure , Hepatomegaly , Hypoglycemia , Hypotension , Hypothyroidism , Liver , Prednisolone , Propranolol , Psychomotor Agitation , Thigh , Thyroid Function Tests , Thyroid Gland , ThyroxineABSTRACT
We present a case of a 41-day-old girl with diffuse hemangiomatosis on her chin, right thigh, occipital area, and liver. She also had hepatomegaly, heart failure, and hypothyroidism. Hemangiomatosis did not respond to 2 weeks of oral administration of prednisolone, but was successfully treated with propranolol. After 2 years of propranolol treatment, the lesions of hemangiomatosis nearly disappeared. Hypothyroidism was controlled by levothyroxine replacement. As hemangiomatosis regressed, thyroid function was normalized. Propranolol may have adverse effects including hypotension, hypoglycemia, bronchoconstriction, and restlessness, but she did not experience such adverse effects. Propranolol could be the first choice for diffuse neonatal hemangiomatosis (DNH). Our case also suggests that thyroid function test is needed in patient with DNH.
Subject(s)
Female , Humans , Administration, Oral , Bronchoconstriction , Chin , Heart Failure , Hepatomegaly , Hypoglycemia , Hypotension , Hypothyroidism , Liver , Prednisolone , Propranolol , Psychomotor Agitation , Thigh , Thyroid Function Tests , Thyroid Gland , ThyroxineABSTRACT
El síndrome de Beckwith-Wiedemann (SBW) es un desorden esporádico o heredado, infrecuente, que se caracteriza por peso elevado al nacimiento, macroglosia, defectos de la pared abdominal y menos frecuentemente hipoglucemia, hemihipertrofia y visceromegalia. Se presenta un paciente de sexo femenino de un mes de vida con antecedentes de nefromegalia evidenciada por ecografía prenatal con múltiples hemangiomas en tronco y labio superior. Al examen físico se evidenció notable macroglosia, hemihipertrofia con compromiso de genitales externos, onfalocele y percentilo de peso mayor a 90. El laboratorio demostró alfa fetoproteína de 608ng/ml. Se realizó diagnóstico de síndrome de Beckwith Wiedemann. El paciente evolucionó con aumento del número y tamaño de las lesiones hemangiomatosas, descenso de los niveles de alfa fetoproteína y su maduración psicomotriz fue adecuada a su edad. Presentamos un síndrome infrecuente en un paciente con hemangiomatosis neonatal benigna (HNB), asociación no descripta previamente en la literatura. Destacamos la importancia del examen físico en la consulta dermatológica como oportunidad diagnóstica.
Beckwith-Wiedemanns syndrome is a sporadic or hereditary rare disor-der characterized by macroglosia, omphalocele, visceromegalia, hypo-glycemia and hemihypertrophy.We report the case of a 1 month-old infant with a history of nephromegalia detected by prenatal ultrasound scan, who presented various generalized hemangiomas.On examination she had macroglosia, hemihypertrophy, omphalocele and high body weight. She also had alpha feto protein 608 ng/ml withno further abnormalities, leading us to diagnose Beckwith-Wiedemann ́s syndrome.The interest of this case is to report an infrecuent syndrome in a patient with diagnosis of neonatal hemangiomatosis. This association has not been previously reported in the literature. We wish to emphasize the importance of a thorough physical exam as part of the dermatologic consultation leading to the correct diagnosis.
Subject(s)
Humans , Female , Infant , Beckwith-Wiedemann Syndrome/diagnosis , Beckwith-Wiedemann Syndrome/genetics , Urogenital Abnormalities/genetics , Skin Diseases/pathology , Hyperplasia , Hemangioma/genetics , Macroglossia , Practice Guidelines as Topic , alpha-Fetoproteins/analysisABSTRACT
Hemangiomas are the most common benign tumors of infancy. Fifteen to 30% of these patients have multiple hemangiomas. Diffuse neonatal hemangiomatosis (DNH) is a disease that often has a fatal outcome if left untreated, and is characterized by multiple cutaneous and visceral hemangiomas. Corticosteroids are the commonly accepted first line treatment, but if no effect is seen, further treatment is required such as interferon, surgical excision, embolization and radiotherapy. Interferon is effective, but the neurologic sequela including spastic diplegia can be a complication. We experienced a case of DHN in a neonate. In this case, the baby presented with multiple cutaneous and visceral hemangiomas with Kasabach-Merritt syndrome (KMS) that included thrombocytopenia and consumptive coagulophthy. The baby was successfully treated with vincristine after the failure of steroid therapy.
Subject(s)
Humans , Infant, Newborn , Adrenal Cortex Hormones , Cerebral Palsy , Fatal Outcome , Hemangioma , Interferons , Kasabach-Merritt Syndrome , Prednisolone , Radiotherapy , Thrombocytopenia , VincristineABSTRACT
Diffuse neonatal hemangiomatosis (DNH) is a rare entity with the distinctive features of multiple hemangiomas of the skin and internal organs, which may result in a fatal outcome if wide-spread involvement of the internal organs. We report a case of DNH with cutaneous and hepatic involvement. The significant regression in the vascular lesions was achieved with systemic corticosteroid therapy.
Subject(s)
Fatal Outcome , Hemangioma , SkinABSTRACT
Diffuse neonatal hemangiomatosis is rare and a life-threatening disorder characterized by multiple cutaneous and visceral hemangiomas. The organs most commonly affected are the liver, lung. brain, GI tract. The complications are high output cardiac failure, hemorrhage, Kassabach-Meritt syndrome, etc. A 30-day-old girl was diagnosed to have diffuse neonatal hemangiomatosis by her multiple cutaneous and hepatic hemangiomas. She was treated with corticosteroid and interferon alfa-2a. During the treatment the number and size of hemangiomas were markedly reduced.
Subject(s)
Female , Humans , Brain , Gastrointestinal Tract , Heart Failure , Hemangioma , Hemorrhage , Interferons , Liver , LungABSTRACT
Most neonatal cutaneous hemangiomatosis are complicated by visceral involvement and. are associated with a high mortality rate in the first month of life. Some neonates with multiple cutaneous hemangiomas, however, may follow a benign course of spontaneous resolution without visceral involvement. Such cases are called benign neonatal hemangiomatosis (BNH). BNH is characterized by a lack of mucosal or symptomatic visceral involvement. rapid spontaneous regression of cutaneous hemangimas, and an excellent prognosis. We report herein a case of BNH which is atypical compared to previously reported BNFI cases. Our patient had a more prolonged course than those of usual cases with BNH and showed a conjunctival involvement.