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Initially, [https://en.wikipedia.org/wiki/Alkannin Alkannin] a gastrostomy was performed to allow for growth of the atretic segments. During the esophagoplasty at age 3 months a TE-fistula of unclassified type was noted and resected (Fig. 2). Delayed primary repair was accomplished. Subsequent to these procedures tracheomalacia became symptomatic, and a tracheostomy was placed at age 4 months. Persistent GE reflux led to a fundoplication at age 4.5 months. A sedated audiogram performed at age 5 months suggested sensorineural hearing loss in the right ear. The patient showed developmental delay with somewhat limited head control at age 5 months. He had persistent swallowing dysfunction. At age 8 months his OFC had increased to 48.7?cm (>95th centile) and his anterior fontanel was enlarged measuring 7?��?8?cm2. A brain MRI revealed mildly enlarged supratentorial extra-axial fluid spaces and suggested the possibility of an ectopic posterior pituitary. Karyotype was 46, XY and comparative genomic hybridization using the SignatureChipOS? oligonucleotide array had [http://www.selleckchem.com/screening/mapk-library.html MAPK Inhibitor Library chemical structure] a normal result. Patient 2 was previously reported by Hall [1997]. This female was born to a mother whose hyperthyroidism was treated with MMI for the first 6 weeks post-conception, and with PTU thereafter. Her birth weight was 2.64?kg, 50th centile for her gestational age of 36�C37 weeks. Findings at age years included a history of bilateral choanal atresia, right iris and retinal coloboma, right sided pelviectasis [http://www.selleckchem.com/products/Metformin-hydrochloride(Glucophage).html click here] and unusual facial features (Fig. 3). Upslanting palpebral fissures, a blunt nasal tip and an inverted V-shaped mouth were previously reported. Her ear length measured 4.1?cm (