Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis is a rare congenital anomaly. Excision of the obstructed vaginal septum is the treatment of choice for symptom relief and the preservation of reproductive capability. A 14-yr-old girl complained of persistent vaginal spotting following each menstruation. Pelvic magnetic resonance imaging revealed a uterus didelphys with left hematocolpos and ipsilateral renal agenesis. Instead of conventional transvaginal excision of the vaginal septum, we used hysteroscopic excision under transabdominal ultrasonographic guidance to preserve the integrity of the hymen. The postoperative course was uneventful, and clinical symptoms were completely resolved after this intervention. Resectoscopic excision of the vaginal septum was found to be easy, safe, effective, and appropriate for young women as it preserved hymen integrity. We believe that this is the first Korean report on the use of a hysteroscopy for vaginal septum resection in a patient with uterus didelphys with obstructed hemivagina.
Adolescent ; Female ; Humans ; Hysteroscopy/*methods ; Kidney/abnormalities ; Urogenital Abnormalities/surgery ; Uterus/*abnormalities ; Vagina/*abnormalities/*surgery/ultrasonography

Thanatophoric dysplasia (TD) is a lethal inherited skeletal disorder characterized by extremely short limbs, narrow chest, skull deformity and underdeveloped lungs. TD is divided into two types, depending primarily upon whether the bone in the upper leg (the femur) is curved or straight. We experienced two case of TD type I that were confirmed by clinical and radiological features after birth. Unlike previously reported cases of TD in our country, the multiple anomalies of CNS, kidney and cardiovascular system were identified in one of these cases.
Cardiovascular System ; Congenital Abnormalities ; Extremities ; Kidney ; Leg ; Lung ; Parturition ; Skull ; Thanatophoric Dysplasia ; Thorax ; Ultrasonography, Prenatal

Meckel-Gruber syndrome is a congenital disorder characterized by occipital encephalocele, bilateral multicystic kidneys with fibrotic changes of the liver and postaxial polydactyly. Other anomalies are frequently associated. This syndrome is a rare autosomal recessive disorder often resulting in neonatal death within a few hours of life and has the high recurrence(one in four) in future pregnancies. We present the case of Meckel-Gruber syndrome diagnosed by ultrasonography at 18 weeks` gestation with a brief literature review.
Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Encephalocele ; Liver ; Multicystic Dysplastic Kidney ; Polydactyly ; Pregnancy* ; Prenatal Diagnosis ; Ultrasonography*

Choristoma ; diagnosis ; Female ; Humans ; Kidney ; abnormalities ; diagnostic imaging ; Middle Aged ; Radiography ; Ultrasonography

Adult ; Amniotic Fluid ; physiology ; Female ; Humans ; Kidney ; abnormalities ; diagnostic imaging ; Pregnancy ; Retrospective Studies ; Ultrasonography, Prenatal

OBJECTIVETo investigate the proportion of children with congenital abnormalities of the kidney and urinary tract (CAKUT) among those who received screening, analyze the rate, diagnosis and treatment of each abnormality, and evaluate the value of ultrasonography in the CAKUT diagnosis.

METHODTotally 489 of 26 989 children who received screening for urinary tract abnormalities were found to have CAKUT and were followed up by telephone interviews and were asked whether they had defined diagnosis of CAKUT, had clinical symptoms and received treatment. Children who had been operated on were also asked about the operation time, surgical procedure, postoperative diagnosis, and outcome. Children who had never been reviewed or still had no diagnosis were arranged to Beijing Children's Hospital for ultrasound follow-up. The final data were summed up for a retrospective analysis.

RESULT1) The proportion of children with CAKUT was 1.67%. Most children with CAKUT had no clinical manifestations. 2) Of the 489 cases, 320 were followed up, and 169 were lost. Of the cases who were followed up, 16 out of 295 cases who had clear diagnosis showed inconsistent results in ultrasound screening. 3) Hydronephrosis was identified in 137 cases (0.78%), among whom 111 were males and 26 were females, in 98 cases the hydronephrosis was on the left, in 28 on the right and in 11 were bilateral. Ureterovesical junction obstruction occurred in 33 cases (0.19%), 25 males and 8 females, in 22 cases on the left, in 10 on right and bilateral in 1 case. Renal dysplasia was shown in 30 cases (0.17%), 22 males and 8 females; renal cysts in 26 cases (0.15%); duplex kidney in 24 cases (0.14%); solitary kidney in 22 cases (0.12%); ureterocele in 16 cases (0.09%); fused kidney in 10 cases (0.06%); renal hypoplasia in 9 cases (0.05%); ectopic kidney in 5 cases (0.03%); polycystic kidney in 3 cases (0.02%). Primary vesicoureteral reflux, posterior urethral valves, renal malrotation, branch renal pelvis, extrarenal pelvis in 1 case each. 4) Totally 114 of the 295 cases (38.6%) who were followed up for CAKUT in screening test required surgical treatment. Only one case of ureteropelvic junction obstruction (UPJO) failed in surgery.

CONCLUSIONThe proportion of children with CAKUT was high. CAKUT is difficult to detect since there is no significant clinical manifestations in early ages. More than one third of patients with CAKUT needed surgical treatment. Ultrasonography showed obvious advantages in detecting children's CAKUT, proven to be the most preferred method of screening, thus could be recommended as a routine of children's physical examination.

Adolescent ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Kidney ; abnormalities ; Male ; Retrospective Studies ; Ultrasonography ; Urinary Tract ; abnormalities ; Urogenital Abnormalities ; diagnostic imaging ; epidemiology

Herlyn-Werner-Wunderlich syndrome (HWWS) is a very rare congenital anomaly of the urogenital tract involving Mullerian ducts and Wolffian ducts, and is characterized by the triad of uterine didelphys, unilateral obstructed hemivagina and ipsilateral renal agenesis. Generally it is diagnosed at puberty after menarche due to recurrent pelvic pain or abdominal mass. We report 2 cases of female newborns whose fetal ultrasonography (USG) showed unilateral renal agenesis and were diagnosed with HWWS by postnatal evaluation. Both cases were female newborns who were born at term by vaginal delivery. They had no perinatal problems except suspicious findings of unilateral renal agenesis from fetal USG. Abdominal ultrasonography and pelvic MRI were performed after birth, and they were diagnosed with HWWS. The potential complications of this syndrome such as pyosalpinx, pelvic adhesions and increased risk of abortion or infertility can occur, but without complication, the prognosis is very good with simple surgical treatment. If renal agenesis is detected in a fetus or a newborn, possible anomalies of urogenital tract such as HWWS should be considered; and postnatal evaluation should be performed, as a simple surgical treatment before menarche can prevent unnecessary complications of disease.
Congenital Abnormalities ; Female ; Fetus ; Humans ; Infant, Newborn ; Infertility ; Kidney ; Kidney Diseases ; Menarche ; Mullerian Ducts ; Parturition ; Pelvic Pain ; Prognosis ; Puberty ; Ultrasonography, Prenatal ; Urogenital Abnormalities ; Wolffian Ducts

A 34-yr-old female was diagnosed as being brain dead. Preoperative ultrasound revealed no abnormal focal lesions. However, the horseshoe kidney was identified during organ harvest. En bloc nephrectomy was performed. The kidney was divided at the midline of isthmus. The divided right kidney was discarded due to numerous arteries and veins. The divided left kidney was transplanted. After declamping, the kidney was well perfused and started clearing. Resistive index was 0.72. Glomerular filtration ratio was 84.69 mL/min on postoperative day 14. The horseshoe kidney can be successfully transplanted and could be a good solution for the shortage of organ donors.
Adult ; Cadaver ; Female ; Humans ; Kidney/*abnormalities/ultrasonography ; Kidney Failure, Chronic/diagnosis/*therapy ; Kidney Transplantation/*methods ; *Tissue Donors ; Tissue and Organ Harvesting/*methods ; Treatment Outcome

A 34-yr-old female was diagnosed as being brain dead. Preoperative ultrasound revealed no abnormal focal lesions. However, the horseshoe kidney was identified during organ harvest. En bloc nephrectomy was performed. The kidney was divided at the midline of isthmus. The divided right kidney was discarded due to numerous arteries and veins. The divided left kidney was transplanted. After declamping, the kidney was well perfused and started clearing. Resistive index was 0.72. Glomerular filtration ratio was 84.69 mL/min on postoperative day 14. The horseshoe kidney can be successfully transplanted and could be a good solution for the shortage of organ donors.
Adult ; Cadaver ; Female ; Humans ; Kidney/*abnormalities/ultrasonography ; Kidney Failure, Chronic/diagnosis/*therapy ; Kidney Transplantation/*methods ; *Tissue Donors ; Tissue and Organ Harvesting/*methods ; Treatment Outcome

PURPOSE: The clinical characteristics and associated anomalies in children with solitary kidney (SK) were analyzed retrospectively. METHODS: Total 38 children diagnosed to have SK at our hospital between December 1989 and December 2009 were recruited, and the clinical records including imaging studies were retrospectively reviewed. SK was defined as unilateral renal agenesis by imaging studies only, and patients with regression of unilateral dysplastic kidney were excluded. RESULTS: Among total 38 patients, 12 were male. The median age at the diagnosis of SK was 6.5 months (at birth-13 years). SK was detected by prenatal ultrasonography in 14 patients and during work-up for renal or urinary tract diseases in 13 (including urinary tract infection in 7). In 10 patients, SK was detected incidentally. Anomalies in the SK were noted in 17 patients including vesicoureteral reflux in 11. Other anomalies in the genitourinary tract were present in 16 patients, and multi-organ-involving syndromes or chromosomal anomalies were detected in 9. The mean duration of follow-up was 9 years (9 months-20 years). Two patients developed chronic renal failure during follow-up, and the median serum creatinine concentration of the remaining 36 at their last follow-up was 0.6 mg/dL. CONCLUSION: SK may be isolated and clinically asymptomatic; it is frequently accompanied by other anomalies in genitourinary tract and other organs, some of which can induce progressive renal dysfunction. Early recognition of associated anomalies with SK and regular follow-up is recommended to reduce long-term risk.
Child ; Congenital Abnormalities ; Creatinine ; Follow-Up Studies ; Humans ; Kidney ; Kidney Diseases ; Kidney Failure, Chronic ; Male ; Retrospective Studies ; Ultrasonography, Prenatal ; Urinary Tract Infections ; Urogenital Abnormalities ; Urologic Diseases ; Vesico-Ureteral Reflux