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Volume 23(1); June 2017

Case Reports

[English]
A Case of Extramedullary Hematopoiesis Associated with Congenital Dyserythropoietic Anemia
Won-Ho Han, Chaeyoun Oh, Ji-Won Han, Joong-Kee Youn, Hyun-Young Kim, Sung-Eun Jung
J Korean Assoc Pediatr Surg 2017;23(1):1-4.   Published online June 26, 2017
DOI: https://doi.org/10.13029/jkaps.2017.23.1.1

It has been known that extramedullary hematopoiesis occurring after birth can be developed in various diseases, and it is often found in hematologic diseases. Among these, congenital dyserythropoietic anemia is a rare disease characterized with increase of ineffective hematopoiesis and morphological abnormalities of erythroblasts. In congenital dyserythropoietic anemia, extramedullary hematopoiesis is very rare and only a few cases have been reported. Although treatment is not required if there is no symptom in extramedullary hematopoiesis, surgery or radiation therapy is effective in case that there is symptom or unresponsive anemia despite blood transfusion. This case report is about surgical treatment for extramedullary hematopoiesis in 23-year-old patients diagnosed of congenital dyserythropoietic anemia.

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[English]
Esophageal Atresia with Bronchogenic Cyst
Youngmin Kim, Chaeyoun Oh, Joong-Kee Youn, Ji-Won Han, Hyun-Young Kim, Sung-Eun Jung
J Korean Assoc Pediatr Surg 2017;23(1):5-8.   Published online June 26, 2017
DOI: https://doi.org/10.13029/jkaps.2017.23.1.5

A baby was diagnosed with esophageal atresia (EA) with tracheoesophageal fistula (TEF) on the next day after birth, and end-to-end anastomosis of esophagus with TEF ligation was performed. The distance between proximal and distal esophageal pouch was checked as 3 vertebral body lengths and a 1 cm-sized bronchogenic cyst (BC) was identified near carina on the right side, just below the proximal esophageal pouch. This case report described the baby who have a BC was located between the both esophageal pouch and a longer esophageal gap than usual EA with distal TEF.

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[English]
Hernia of Umbilical Cord with Segmental Dilatation of Ileum: Cause or Effect?
Shalini Hegde, Monika Bawa
J Korean Assoc Pediatr Surg 2017;23(1):9-11.   Published online June 26, 2017
DOI: https://doi.org/10.13029/jkaps.2017.23.1.9

Hernia of the umbilical cord associated with segmental dilatation of the ileum (SDI) is a rare entity with only 17 cases reported in literature. We hypothesize that the prominent mesenteric vessels or folds that splay out towards the ends of the SDI signify the presence of an antecedent duplication cyst which eventually got resorbed to form the dilated segment.

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[English]
Familial Isolated Anorectal Malformation: A Case Report
Jinyoung Park
J Korean Assoc Pediatr Surg 2017;23(1):12-14.   Published online June 26, 2017
DOI: https://doi.org/10.13029/jkaps.2017.23.1.12

There have been a few reports of familial anorectal malformations extending over more than one generation. We experienced a case of a family with 3 members spanning 2 generations affected with isolated low type anorectal malformations. They had same low type of anorectal malformations. In all 3 patients, a perianal anoplasty was performed.

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[English]
“Intraluminal” Pyloric Duplication: A Case Report
Kyeong Deok Lee, Yoshifumi Kato, Geoffrey J. Lane, Atsuyuki Yamataka
J Korean Assoc Pediatr Surg 2017;23(1):15-17.   Published online June 26, 2017
DOI: https://doi.org/10.13029/jkaps.2017.23.1.15

We report a neonatal case of “intraluminal” pyloric duplication cyst, causing gastric obstruction after birth. Endoscopy revealed a submucosal cystic lesion approximately 15 mm in size arising from the anterior and inferior surfaces of the pylorus obliterating the pyloric canal. After laparotomy, intraoperative cholangiography was performed, which documented no communication between the cyst and the bilio-pancreatic duct. Gastrotomy was performed transversally over the antrum, and the cyst delivered through the incision. The cyst was incised, the upper part of the cyst wall removed, and a mucosectomy performed on the inner cyst wall of the lower part. The mucosa and muscle of the margin of the cyst were approximated. At follow up of 10 months, the patient is well without any sign of gastric obstruction.

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