• Korean Journal of Head & Neck Oncology
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• v.32 no.2
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• pp.79-83
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• 2016

Chyle fistula is one of the complications of neck dissections. Although immediate surgical repair seems to be the best choice when chyle leakage is observed during the operation, some operators can be embarrassed when chyle leakage is heavy and not controlled during surgery. In this case, chyle leakage was occurred after extensive resection of lymph nodes in left level IV, and was not controlled in any way. The clavicular head of sternocleidomastoid muscle was dissected and inferior-based muscular flap was rotated to cover the suspected region of fistula orifice. Amount of drainage was checked less than 20 ml per day in the following days, and drain tube was taken out on the 3rd postoperative days. We present the technique using the inferior based sternocleidomastoid muscle flap for intraoperative management of chyle leakage not easily controlled.

• Journal of Chest Surgery
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• v.29 no.6
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• pp.672-674
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• 1996

Chylothorax is a rare, but well-recognized complication of cardiothoracic surgery and operations in the region of the esophageal or aortic diaphragmatic hiatus. Especially, in nutritionally depleted patients requiring esophagectomy for benign or malignant diseases, it is a potentially life-threatening disorder that has profound respiratory, nutritional and immunologic co sequences . We have experienced a case of chylothorax after transhiatal esophagectomy in benign esophageal stricture. The diagnosis of chylothorax was confirmed after feeding through the jejunostomy tube by the change of the character of pleural effusion and high triglyceride level on the 5th postoperative day. On the thirteenth postoperative day, supradiaphragmatic thoracic duct ligation was performed through right thoracotomy. We could remove the chest tube on the 22th postoperative day, and the patient is being followed-up at out patient clinic without complications.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.21 no.1
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• pp.15-21
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• 2021

Purpose: Urea cycle disorder (UCD) is an inherited inborn error of metabolism, acting on each step of urea cycle that cause various phenotypes. The purpose of the study was to investigate the long-term clinical consequences in different groups of UCD to characterize it. Methods: Twenty-two patients with UCD genetically confirmed were enrolled at Pusan National University Children's hospital and reviewed clinical features, biochemical and genetic features retrospectively. Results: UCD diagnosed in the present study included ornithine transcarbamylase deficiency (OTCD) (n=10, 45.5%), argininosuccinate synthase 1 deficiency (ASSD) (n=6, 27.3%), carbamoyl-phosphate synthetase 1 deficiency (CPS1D) (n=3, 13.6%), hyperornithinemia-hyperammonemia-homocitrullinuria syndrome (HHHS) (n=2, 9.1%), and arginase-1 deficiency (ARG1D) (n=1, 4.5%). The age at the diagnosis was 32.7±66.2 months old (range 0.1 to 228.0 months). Eight (36.4%) patients with UCD displayed short stature. Neurologic sequelae were observed in eleven (50%) patients with UCD. Molecular analysis identified 37 different mutation types (14 missense, 6 nonsense, 6 deletion, 6 splicing, 3 delins, 1 insertion, and 1 duplication) including 14 novel variants. Progressive growth impairment and poor neurological outcomes were associated with plasma isoleucine and leucine concentrations, respectively. Conclusion: Although combinations of treatments such as nutritional restriction of proteins and use of alternative pathways for discarding excessive nitrogen are extensively employed, the prognosis of UCD remains unsatisfactory. Prospective clinical trials are necessary to evaluate whether supplementation with BCAAs might improve growth or neurological outcomes and decrease metabolic crisis episodes in patients with UCD.