The cystic disease of the kidney include a heterogeneous group of developmental, hereditary, and acquired disorders. Based on extensive microdissection studies, Potter concluded all renal cystic diseases could be categorized into four types. We have experienced 5 cases of cystic kidney disease which were confirmed by aoutopsy and classified as Type I, Type II, Type III, Boderline between types II and... |
Sacrococcygeal teratoma is a rare tumor, it occurs once in every 40,000 live births. Most of the reported cases appear in female infant (M:F=1:4). They deserve clinical attention because they are potenially malignant and are curable if diagnosed and treated early. The patients may have associated congenital anomalies. This patient has spina bifida cystica in lower sacrum. Brief review of related literature is included... |
Bile peritonitis due to spontaneous perforation of choledochal cyst is a rare disease and the etiology of spontaneous perforation is unknown in most of infant cases. Recently, we experienced a case of bile peritonitis caused by spontaneous perforation of choledochal cyst in a 6 month-old female infant. She had progressive abdominal distention with ascites, mild jaundice and intermittent passage of acholic... |
Acute hemorrhagic cystitis characterized by gross hematuria and symptoms of bladder irritation occurs in children as a self-limited disease that must be differentiated from serious renal disease. We analyzed 22 cases of hemorrhagic cystitis which were managed in the Dept. of pediatrics, Kyung Hee university hospital and present the availability of bladder sonography for diagnosis of hemorrhagic cystitis and for follow-up. The... |
We experienced two cases of porencephaly in a 1 year old male and an 8 years old femalo were suffered from right hemiparesis and convulsion. Brain CAT confirmed the diagnosis of porecephalic cyst. We report these cases with brief review of some related literatures. |
Cystic hygroma is a true, benign, congenital multi-cystic tumor. It is thought to be arised from failure of the lymphatic system to communicate with the venous system. Frequently it occurs at birth, and it is found most commonly in the neck as painless and fluctuant mass. For many years, surgical excision has been considered as the treatment of choice. However, excision... |
The authors evaluated 19 childrenwith chronic renal failure patients diagnosed and followed up at the Department of Pediatrics, Seoul National University Hospital for the detection of acquired renal cystic disease (ARCD) with two radiologists by the means of ultrasonography. The results were as follows: 1) Of the 19 patients, male to femals ratio was 14:5 and of them, hemodialysis and peritoneal dialysis... |
Pararenal pseudocyst is an encapsulated cystic collection of chronically extravasated urine or serous fluid, usually occurring in the perirenal space by renal trauma, operative procedures or obstructive uropathy such as posterior urethral valve, or calculi, tumors of genitourinary tract and often associated with hydronephrosis and displacement of the kidney. We experienced a case of pararenal pseudocyst, right and congenital hydronephrosis, left... |
A 3-year-old female patient was admitted due to marked abdomainal distension. Her mother has been noticed the abdominal distension since birth, and the abdomen was enlarged progressively, but there were no subjective symptoms such as abdominal pain, vomiting nor indigestion. The radiographic findings showed huge intraabdomainal mass anterior to intestine which had septated fluid collection in it. Omental cyst was... |
Congenital cystic diseases of the lung (CCDL) is a clinical group of disorders characterized by a cystic appearance of pulmonary tissue. It can be classified into four distinct categories: 1) Congenital lobar emphysema (CLE), 2) Congenital cystic adenomatoid malformation (CCAM), 3) Pulmonary sequeestration (PS), and ^Bronchogenic cyst (BC). 24 patients with CCDL admitted to the Seoul National University Children’s Hospital from January 1984 to June... |
A clinical study was performed on 5 patients of neurocysticercosis in childhood who admitted to the Seoul National University Hospital for the last two years. The conclusions were as follows: 1) Boys were more than girls with the ratio of 4:1. The average age was 9.5. 2) Three out of five lived in Jeju Island. Three had familial history of taeniasis. 3) Three out of five were... |
A case of congenital bile duct cyst in a 15-year-old female patient was diagnosed by clinical symptoms, laboratory data, ultrasonography, endoscopic retrograde cholangiopancreatography. Excision with Roux-en-Y choledochocystojejunostomy was performed with good result. We report one case of congenital bile duct cyst type IV-A (by Todani classification) with brief review of all 277 cases reported so far in Korean literature and present comparisons with Flanigan’ s... |
Homocystinuria caused by cystathionine B-synthetase deficiency is characterized biochemically by increased concentration of homocystine and methionine, decreased concentration of cystine in plasma and urine. The manifestation of this rare disorder include skeletal abnormalities, a high incidence of thromboembolism, ectopia lentis, and a high frequency of mental retardation. We experienced a case of homocystinuria in a 10-year-old female patient who had mental retarda- tion, ectopia lentis,... |
A rare form of congenital cystic lung disease, characterized by the presence of one or usually multiple interconnecting cyst, is called congenital cystic adenomatoid malformation of the lung. This disease almost invariably presents either in live premature or stillborn infants, and death in those infants bom alive usually occurs within a few hours of birth. Infants with congenital cystic adenomatoid malformation usually have tachypnea,... |
The authors experienced a case of neuroepithelial cyst which was found deep inside the brain, involving the ventricular septum. A 2 months old female patient was presented with convulsion. Characteristic CT findings showing multiloculated cystic structures were seen. Operation was carried out to confirm multiple intraventricular cysts containing yellowish fluid. Histologically the cyst wall consisted of simple cuboidal epithelium and fibrous connective tissue. A brief... |
Cystic lymphangioma, or cystic hygroma, is a true, benign, congenital multic-ystic tumor arising from sequestration of embryonic lymphatic tissue. Most often it occurs at birth or early in life, and it was found most commonly in the neck, but rarely in the retroperitoneum and its distribution coincides with that of the primitive lymph sac. We have experienced a case of retroperitoneal cystic lymphangioma in... |
We report cystinuria in siblings. Renal colic, hematuria, dysuria and stone passage were developed in younger brother (4 year 6 month old boy). But the elder sister (6 year old girl) had no specific symptoms nor signs. The identification of the disease was proved by cyanide nitroprusside test and amino acid analysis of urine. In our patients the chromatographic amino acid patterns of urine showed... |
Congenital cystic adenomatoid malformation is a rare variant of pulmonary cystic disease char- acterized by a mass of cysts lined by proliferating bronchial or cuboidal epithelium. The onset of symptoms, which are cyanosis, and tachypnea, usually occurs during the first week of life. We have experienced a case of congenital cystic adenomatoid malformation in a 9 month-old female. The diagnosis was mady by chest... |
In childhood, cancer of the ovary is highly unusual. It is responsible for only 1—2% of cancers found in patients under the age of 17. The frequency with which the various histologic types of ovarian neoplasm occur in childhood differs from that in adults. Epithelial tumors account for 70% to 80% of ovarian neoplasms in adults but only 20% in patients less than... |
A case of congenital esophageal duplication cyst has been studied in a 10-month old male Korean infant. He had suffered from cough, fever and dyspnea since 4 months of age, and he had been admitted 4 times to other hospitals before he visited severance hospital due to aggravated cough and dyspnea. An esophagogram and computed tomogram of chest were done and a post-tracheal mass... |
The following results were obtained from eight cases of hereditary spherocytosis (HS) who were admitted at the Department of Pediatrics, Kyung-pook National University Hospital from July 1984 to January 1989. The percentage of HS among the pediatric hematology patients during the same period was 2.6%. Male amd female were one and seven respectively. The mean age at the diagnosis and at onset of symptom was... |
We studied the cyclophosphamide-induced hemorrhagic cystitis in 72 cases of acute lymphocytic leukemia or Non-Hodgkin lymphoma, which had been treated at Seoul National University Children' s Hospital from June, 1985 to May, 1988 1) Among 72 cases treated with cyclophosphamide, 6 cases(12.8%) developed hemorrhagic cystitis in 47 cases of acute lymphocytic leukemia and 2 cases(8.0%) in 25 cases of Non-Hodgkin lymphoma. 2) The development of... |
We experienced and case of huge ovarian cyst in a 13 year-old gril, which is rare in chilbhood and adolescence. The cyst was about 6 kg in weight, 29 x 20 x 17 cm in size and occupied the entire abdome and pelvis. Its pathologic diagnosis was mucinous cystadenoma. Rt. hydronephrosis due to compression by a huge ovarian cyst was found. A brief review... |
Multilocular renal cyst is an uncommon renal disease. Since the first description of the disease by Edmund in 1892, about 100 cases have been reported in the world. Recently, authors experienced an unilateral multilocular cyst of the right kidney in 12 months-old male patient, and reported with review of related Iiteratures. |
Serial neurosonographic examinations were performed in 34 low birth weight infants who were admitted to the neonatal intensive care unit of Yong-Dong Severance Hospital. Among them, 14 (41 %)were found to have periventicular-intraventricular hemorrhage. The initial hemorrhage occured in the first three days of life among 71% infants. After initial hemorrhage, hemorrhage was decreased in size and thereafter absorbed. Fourteen (44%) infants demonsrated cysts... |
A clinical study of 24 patients with congenital choledochal cyst who had been admitted to Severance Hospital, Yonsei University College of Medicine during the 16 year peroid from January, 1971 to December, 1986 was done. The results were as follows; 1) Of the 24 cases studied, the highest incidence of this cyst, 15 cases (62.5%), was found in children younger than 10 years of age. The... |
Among heterogenous group of developmental anomalies known as enteric duplication, mediastinal gastroenteric cysts are rare and of outstanding diagnostic importance, because without definitive treatment they can be fatal. Unlike other mediastinal cysts they are almost never asymtomatic. The authors experienced a case of mediastinal gastroenteric cyst in 1 year 7 month-old female patient with chief complaint of abnormal mass density in chest X-ray. The... |
Arachnoid cysts are benign cysts occuring in the cerebrospinal axis in relation to the arachnoid membrane. From Jan, 1974 unitl June, 1986. we evaluated the clinical features and outcome of the arachnoid cyst by the review of the medical records and followup study of 20 cases in pediatric age group, and the results are as follows: 1) The age distribution of the patients was from 2... |
The authors experienced a case of benign cystic sacrococcygeal teratoma in a 2 month-old female infant. The cystic mass was located at presacral area without external presentation, causing left renal hydronephrosis and ureterectasis. Surgical excision of the mass was performed successfully. The literature regarding the subject was reviewed. |
In 1946, Potter described a series of 20 cases of infants in whom bilateral absence of the kidneys was associated with hypoplasia of the lungs and characteristic faces. Afterwards it has been known that any condition that causes significant reduction in urine formation or excretion will result in oligohydramnios accounts for many of the nonrenal features of Potter syndorme. We report a case... |