Metwalley Kotb Abbass, Farghaly Hekma Saad
Pediatric Endocrinology Unit, Department of Pediatrics, Faculty of Medicine, Assiut University, Assiut, Egypt.
J Med Case Rep. 2013 Feb 18;7:48. doi: 10.1186/1752-1947-7-48.
Benign neonatal hemangiomatosis is a condition in which multiple cutaneous hemangiomas appear at birth or shortly thereafter; visceral complications are absent. Here, we report a case of a consumption hypothyroidism in an Egyptian baby with benign neonatal hemangiomatosis.
An 8-month-old Egyptian boy with benign neonatal hemangiomatosis was referred to our institution for evaluation of developmental delay. Initial examination revealed a quiet baby who was able to sit only with support. He had hypotonia, a large anterior fontanelle, puffy eyes, cold extremities, hypothermia, bradycardia, and abdominal distension. An examination of his skin revealed more than 100 dome-shaped red-purple cutaneous hemangiomas that varied in size from 5 to 10mm on the back, the abdomen and the extremities without mucus membrane involvement. He had low serum free thyroxine concentration and triiodothyronine levels and high thyroid-stimulating hormone and reverse-triiodothyronine levels. A work-up that involved appropriate imaging ruled out visceral involvement. Based on the above mentioned data, a diagnosis of consumptive hypothyroidism due to benign neonatal hemangiomatosis was made. He was started on oral thyroid medication which was gradually increased to 90μg L-thyroxine daily (15μg/kg/day). After three months of treatment, he was able to sit alone without support and he had normal levels of thyroid-stimulating hormone and serum free thyroxine.
Thyroid function should be assessed periodically in babies with benign neonatal hemangiomatosis, especially if symptoms of hypothyroidism appear or the size and number of hemangiomatosis increase rapidly. Moreover, high doses of L-thyroxine may be needed to achieve euthyroidism during the infancy.
良性新生儿血管瘤病是一种出生时或出生后不久出现多个皮肤血管瘤的疾病,无内脏并发症。在此,我们报告一例患有良性新生儿血管瘤病的埃及婴儿出现消耗性甲状腺功能减退症的病例。
一名患有良性新生儿血管瘤病的8个月大埃及男孩因发育迟缓被转诊至我院评估。初步检查发现该婴儿安静,仅在支撑下才能坐立。他肌张力低下、前囟大、眼睛浮肿、四肢冰冷、体温过低、心动过缓且腹胀。对其皮肤检查发现背部、腹部和四肢有100多个大小从5至10毫米不等的圆顶状红紫色皮肤血管瘤,未累及黏膜。他的血清游离甲状腺素浓度和三碘甲状腺原氨酸水平较低,促甲状腺激素和反三碘甲状腺原氨酸水平较高。包括适当影像学检查在内的检查排除了内脏受累。基于上述数据,诊断为良性新生儿血管瘤病所致的消耗性甲状腺功能减退症。开始给他口服甲状腺药物,逐渐增加至每日90μg左甲状腺素(15μg/kg/天)。治疗三个月后,他能够独自坐立且促甲状腺激素和血清游离甲状腺素水平正常。
对于患有良性新生儿血管瘤病的婴儿,尤其是出现甲状腺功能减退症状或血管瘤大小和数量迅速增加时,应定期评估甲状腺功能。此外,婴儿期可能需要高剂量左甲状腺素来实现甲状腺功能正常。