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Chylothorax is a rare cause of pleural effusion in children, and it is usually difficult to identify the location of chyle leakage due to the small size of the thoracic duct in children. Herein we report an infant case with chylothorax whose leakage of the thoracic duct was successfully located by magnetic resonance lymphangiography (MRL) using pre-contrast MR cholangiopancreatography (MRCP) and gadodiamideenhanced spectral presaturation inversion recovery (SPIR) T 1 -weighted imaging, which demonstrate the imaging method is easy and effective for detecting the focal disruption of the thoracic duct in children with chylothorax and younger than 8 months old.Chin Med Sci J 2017; 32(4): 265-268.

Background Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is mainly characterized by congenital aplasia of the uterus and the upper two-thirds of the vagina in females with normal secondary sex characteristics and female karyotype (46,XX). MRKH syndrome is typically diagnosed due to primary amenorrhea in adolescence and is very difficult to diagnose in childhood. MRKH syndrome combined with central precocious puberty (CPP) is extremely rare. In this article, we report a case of MRKH syndrome with idiopathic CPP (ICPP). Case Description A 7-year-old girl was presented with development of bilateral breasts for 1 year and relatively low body height. Based on her age, clinical signs, and laboratory findings, she was initially diagnosed with ICPP and treated with sustained-release gonadotropin-releasing hormone analog (GnRHa) therapy, and recombinant human growth hormone (rhGH) therapy from the 6 th month onwards. During the follow-up, ultrasound and magnetic resonance imaging showed no uterus or uterine neck, an unclear vaginal structure, and normal ovaries. Her chromosome karyotype was 46,XX. A pediatric gynecological examination showed colpatresia. She was finally diagnosed with MRKH syndrome combined with CPP. After the GnRHa and rhGH treatment, her height became normal compared to her peers, and her bone age development was delayed. Conclusions The present case suggests the possibility of concomitant CPP in patients with MRKH syndrome. The gonads and sexual organs of children with precocious puberty should be carefully monitored and assessed to exclude any sexual organ disorders.