• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.23 no.6
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• pp.531-538
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• 2020

Purpose: Our aim was the longitudinal assessment of cyst size in fetuses with biliary cystic malformation (BCM) to explore its etiology and the possibility of antenatal differentiation between biliary atresia (BA) and congenital biliary dilatation (CBD). Methods: We conducted a retrospective review of all patients diagnosed antenatally with BCM from 1994 to 2014 at our institutions. Results: The study cohort comprised of three patients with BA and six with CBD. There were no significant differences in the gestational age and cyst size at the first detection of BCM between the two groups. In fetuses with CBD, the cyst size steadily increased as the gestational age advanced, while it fluctuated around 1.5 cm and remained below 2.1 cm in those with BA. However, the ratio of cystic area to fetal trunk area was approximately constant due to linear fetal growth in fetuses with CBD. Conclusion: Fetuses with BCM <2.1 cm in the late gestation period were more likely to have BA than CBD. Our observation of cyst enlargement with advancing gestational age in the CBD group was attributed solely to fetal growth. Biliary dilatation in fetuses with CBD and BA might be completed at the onset of BCM.

• Journal of Yeungnam Medical Science
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• v.29 no.2
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• pp.136-140
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• 2012

A 61-year-old male who complained of right upper quadrant pain was referred to the authors for evaluation after his computed tomography suggested biliary adenocarcinoma. The lesion consisted of multiple cysts with papillary mass and peri-ampullay mass. The patient underwent an operation due to a clinical suspicion of biliary cystadenocarcinoma, but the pathology confirmed biliary papillomatosis (BP) after diagnosing intrahepatic papillary neoplasm with high-grade dysplasia and invasive adenocarcinoma with papillary neoplasm from the distal common bile duct to the duodenum. BP is a disease characterized by multiple papillary masses. Its cause has yet to be discovered. It commonly manifests as bile duct dilation but rarely as a ductal cystic change. Under computed tomography or magnetic resonance imaging, both the BP and the cystic neoplasm can show bile duct dilation and a papillary mass, which makes their differential diagnosis difficult. A confirmative diagnosis can be made through a pathologic examination. BP is classified as a benign disease that can become malignant and may recur, though rarely. Its treatment of choice is surgical resection. Laser ablation or photodynamic therapy can be used for unresectable lesions. In the case featured in this paper, biliary papillomatosis was difficult to differentiate from cystic adenocarcinoma due to diffusely scattered multiple large cystic lesions in the liver, and it was histologically confirmed to have become malignant with cystic duct dilation after the operation. This case is reported herein with a literature review.

• Advances in pediatric surgery
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• v.15 no.1
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• pp.11-17
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• 2009

Choledochal cyst is a congenital dilatation of the bile duct. Intrahepatic bile duct dilatation of type IVa by Todani's classification at the time of diagnosis resolved spontaneously after cyst excision and hepaticojejunostomy in many cases. It should be distinguished from the true cystic dilatation of the intrahepatic ducts, which tends to persist, albeit after some regression. We therefore studied postoperative intrahepatic duct dilatation changes in choledochal cyst. A total of seventy-six choledochal cysts were managed at the Division of Pediatric Surgery, Department of Surgery, Samsung Medical Center from May 1995 to December 2005. The ratio of males to females was 1:2.8. Preoperative radiologic diagnosis by Todani's classification was Type I (n=52, 68.4 %), II (n=1, 1.3 %), IVa (n=23, 30.3 %). Among fifty-five patients with intrahepatic bile duct dilatation we were able to follow up forty-eight by ultrasonography. Twenty-two patients were type IVa, and twenty-six patients were type I and showed intrahepatic duct dilatation. Mean follow-up duration was 35.3 months (9~105 months). Complete regression of dilated intrahepatic duct was observed in fifteen patients of type IVa and twenty-four patients of type I. Incomplete regression of dilated intrahepatic duct was observed in six patients in type IVa and two patients in type I. Only one patient in type IVa showed no change in ductal dilatation during a follow-up period of 15 months. We conclude that true type IVa is much less frequent than what was diagnosed preoperatively by imaging study. Therefore in type IVa patients who are diagnosed preoperatively the decision to perform liver resection should be carefully considered. Postoperative long term follow up of choledochal cyst with intrahepatic bile duct dilation is needed.

• Journal of Yeungnam Medical Science
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• v.9 no.2
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• pp.427-435
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• 1992

We have experienced a case of infantile nephrotic syndrome confirmed by renal biopsy in a 13-month-old female patient who showed growth and develop mental retardation and persistent proteinuria. She revealed mild eyelid edema, joint laxity, delayed speech development and adrenal cortical calcification on the radiologic study. Renal biopsy showed microcystic tubular change, micro-glomeruli and marked mesangial proliferation.

• Advances in pediatric surgery
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• v.10 no.2
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• pp.136-141
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• 2004

Biliary atresia (BA) with extrahepatic biliary cysts (EHBC) is a rare disease. It has been generally recognized as type I (correctable with cystic dilatation), which means a good prognosis. From a total of 73 patients with BA who underwent operation from September 1988 to September 2003 at our institute, 7 (9.6 %) cases of type III BA with EHBC (uncorrectable with cystic dilatation) are reviewed. Clinical findings, laboratory data, radiologic findings, treatment methods and outcomes were reviewed. Female was more prevalent (male to female ratio; 2:5). All cases were type III with EHBC according to the intraoperative cholangiography, and underwent Kasai' s portoenterostomy. The mean age was 57 days at operation. Three patients(42.9 %) are long term survivors. Further evaluation is needed to determine the correlation between prognostic factors and outcome for.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.17 no.3
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• pp.170-177
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• 2014

Purpose: Choledochal cyst is a cystic dilatation of common bile duct. Although the etiology is presently uncertain, anomalous pancreaticobiliary ductal union (APBDU) is thought to be a major etiology of choledochal cyst. In this study, we analyzed the clinical and anatomical characteristics and pathologies of patients diagnosed with choledochal cyst in a single institute for 25 years. Methods: A total of 113 patients, diagnosed with choledochal cyst and who received an operation in Severance Children's Hospital from January 1988 to May 2013, were included. Medical records were reviewed, including clinical and demographic data, surgical procedures. Abdominal ultrasonography, magnetic resonance cholangiopancreatography, and intraoperative cholangiography were used as diagnostic tools for evaluation and classification of choledochal cyst and the presence of anomalous pancreaticobiliary ductal union. Todani's classification, and relationship between APBDU and surgical pathology. Results: Among 113 patients, 77 patients (68.1%) presented symptoms such as hepatitis, pancreatitis and/or cholecystitis. Eighty three patients (73.5%) had APBDU, and 94 patients (83.2%) showed inflammatory pathologic changes. APBDU, pathologic inflammation, and serological abnormalities such as hepatitis or pancreatitis showed a statistically significant correlation to one another. Conclusion: APBDU is thought to be one of the etiologic factors of choledochal cyst. It is related to the inflammatory changes in bile duct that can lead to the cystic dilatation.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.26 no.5
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• pp.284-289
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• 2023

A 14-year-old girl was admitted to the emergency department for excessive bile-containing vomiting and severe abdominal pain. She had been healthy until she intentionally lost 25 kg over a 6-month period. Thick, bloody bile-mixed food particles were drained from the stomach through a nasogastric tube. Abdominal computed tomography revealed huge stomach dilatation with extensive gastric pneumatosis, possible near rupture, acute pancreatitis, and a very narrow third of the duodenum, indicating superior mesenteric syndrome. Gastrofibroscopy revealed multiple hemorrhagic ulcers and numerous beadlike cystic lesions in the stomach. Laboratory examination results were notable for severe deficiencies in critical nutrients, including iron, zinc, proteins, and prealbumin, as well as undernutrition-associated endocrine complications such as hypothyroidism and hypogonadotropic hypogonadism. Excessive vomiting ceased after the endoscopic removal of stagnant gastric contents. Gastric pneumatosis improved after 3 days of supportive care.

• Childhood Kidney Diseases
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• v.3 no.2
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• pp.221-226
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• 1999

Glomerulocystic kidney disease(GCKD) is a rare form of renal cystic disease defined histopathologically by containing dilated Bowman's space with variable atrophy of glomerular tufts, which may occur as sporadically or as familial cases and can be presented as a major component of heritable syndromes. It has not been recognized in Korean children but only one report of adult case has been reported having GCKD. We experienced a case of GCKD in a 10-year-10-month-old boy, who was admitted for hypertension. Abdominal ultrasonography and computed tomography revealed clustered numerous small cysts in left kidney and renal biopsy findings was consistent with the GCKD showing cystic dilatation of Bowman's space with intact glomerular structure.

• Journal of Chest Surgery
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• v.19 no.2
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• pp.335-340
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• 1986

Pulmonary arteriovenous fistula is unusually congenital malformation consisting of an abnormal connection between a pulmonary artery and vein. It may appear as an isolated anomaly or with hereditary hemorrhagic telangiectasis. In the classic form, symptoms and signs are caused by the abnormal right to left shunt. since severe clinical symptoms such as paradoxical embolism, infection, and spontaneous rupture with massive bleeding may occur, surgical treatment is often indicated. Recently we have experienced a surgical treatment of pulmonary arteriovenous fistula in 54 years old housewife. She was admitted in May 1986 with recurrent hemoptysis for 1 month. On admission, the PaO2 was 65.2 mmHg without specific findings. Pulmonary arteriovenous fistula was confirmed by preoperative pulmonary arteriography. A well circumscribed cystic mass was noted in visceral subpleural and inferior lingular segment of left upper lobe. Left upper lobectomy was performed with good results. Histologically angiomatous dilatation of abnormal vessels embedded in lung parenchyma was noted. Postoperative PaO2 was 90 mmHg. Postoperative results were good.

• Clinical and Experimental Pediatrics
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• v.59 no.5
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• pp.239-241
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• 2016

Choledochal cyst is a dilation that encloses the intrahepatic or both extra- and intrahepatic portions of the biliary ducts. Postnatally, ultrasonography is the initial diagnostic modality of choice, allowing for precise measurements of intra- or extrahepatic duct dilatation and identification of stones and sludge. Symptoms depend on the age at presentation. Common bile duct malformations should be considered as a differential diagnosis of a cystic mass regardless of the cyst's size or patient's age, especially in children presenting with abdominal pain, jaundice, and palpable mass. To the best of our knowledge, we report the largest choledochal cyst in infancy.