• Archives of Plastic Surgery
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• v.38 no.5
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• pp.616-620
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• 2011

Purpose: Mayer-Rokitansky-Kuster syndrome (MRK) is second common cause of primary amenorrhea. It is a syndrome of vaginal aplasia and Mullerian duct anomaly. Vaginal aplasia varies from agenesis of whole vagina to aplasia of upper 2/3. For reconstructing vagina, various methods are introduced. Gracilis myocutaneous flap was the first attempt in that the flap is used in vaginal reconstruction. Various flap-based vaginal reconstruction methods have been introduced. Modified Singapore flap (pedicled neurovascular pudendal thigh fasciocutaneous flap) is one of those methods that used posterior labial artery as pedicle, and pudendal nerve branch as sensory root. As its donor lies on inguinal crease that is easily hidden and there are benefits on sexual intercourse by early sensory recovery, authors think that modified Singapore flap is effective for young MRK syndrome patients. Methods: Eight patients underwent surgery between 2008 and 2010. The flap was designed on both groin area with external pudendal artery branch as a pedicle. All flaps were fixated in pelvic cavity with absorbable suture, and additional compression on neovaginal wall was supplied by polyvinyl alcohol sponge ($Merocel^{(R)}$). Results: All patients were successfully reconstructed without flap related complications such as congestion or partial flap loss. The average size of the flap (each side) was 69.34 $cm^2$. Polyvinyl alcohol sponge ($Merocel^{(R)}$) was inserted into neovagina for 5 days on every patient. One case of rectal laceration was occurred while making pelvic pocket by OBGY team. Other complications such as lumen narrowing, wound contracture or vaginal prolapsed were not reported during 8 months of follow up. Conclusion: Modified Singapore flap is a sensate fasciocutaneous flap that is thinner than other myocutaneous flap such as VRAM, and more durable over skin graft. Therefore this is a good choice for vaginal reconstruction in MRK syndrome. And known complications of Modified Singapore flap could be reduced with careful procedure and mild compression techniques.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.15 no.1
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• pp.65-69
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• 2019

Wolf-Hirschhorn syndrome(WHS) is a congenital disorder caused by deletions of the short arm of chromosome 4. The most common characteristics are mental and growth retardation, dietary disorder and craniofacial features with a characteristic 'Greek warrior helmet' appearance. The dental characteristic of WHS includes delayed development, tooth agenesis, clefts, microdontia, taurodontism, and severely worn dentition. The purpose of this case report is to describe the dental treatment of a patient with WHS. 3-year-old boy with WHS visited the Seoul National University Dental Hospital for dental treatment. He had difficulty with nasotracheal intubation because of craniofacial anomalies and also had poor oral hygiene due to a limitation of mouth opening and dietary disorder. Due to his airway problem, behavior management and severity of dental conditions, dental treatment was performed under general anesthesia. This case suggests general anesthesia can be chosen with WolfHirschhorn syndrome patients to safely care for their dental problems.

• The Journal of Korean Academy of Prosthodontics
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• v.57 no.1
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• pp.81-87
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• 2019

Patients with Down's syndrome have several dental complications such as small teeth caused by underdevelopment of dentin and enamel, periodontitis, agenesis of teeth, prolonged retention of primary teeth and malocclusion due to narrow palate. Removable denture with maxillary double crowns would be a good treatment option to solve the problems of the patient with Down's syndrome. Double crowns compensate the insufficient support and retention of denture and easily solve the cross bite problem. Double crowns also allow easy repair of denture in case of abutment teeth extraction. In this case, 26-year-old female patient with Down's syndrome and dental phobia had small number of teeth with enamel hypoplasia, prolonged retention of primary teeth and dental cross bite. Prosthetic treatment was done using removable denture with double crowns in the maxilla. In the mandible, teeth preparation was done on enamel margin without anesthesia. Anterior laminate and posterior complete zirconia crown restorations were performed. As a result, the cross bite was effectively corrected by denture with double crowns. Pronunciation and appearance were also improved without extraction of teeth and dental anesthesia.

• Clinical and Experimental Pediatrics
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• v.51 no.10
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• pp.1085-1089
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• 2008

Purpose : This study aims to examine and compare the features of rolandic epilepsy. Methods : Of 158 patients selected retrospectively, 116 had typical (group A) and 42 had atypical (group B) rolandic epilepsy, as defined by Worrall's criteria. Results : The age at onset of the seizures in group A was $8.6{\pm}2.0y$ and $6.2{\pm}1.7y$ in group B (P>0.05). Among the 40 patients who underwent neuroimaging studies (25 patients in group A and 15 patients in group B), abnormal findings in group B included ventricular dilatation, mild cortical atrophy, and partial agenesis of corpus callosum. group A had no abnormal findings. The frequency of seizures was $2.0{\pm}1.0$ and $2.3{\pm}1.2$ per month in groups A and B respectively. Seizure control from the initial anticonvulsant treatment was achieved within 3 months in group A, and 3 to 12 months in group B. A 2-year remission rate was noted in 105 patients in group A and in 38 patients in group B. Of these, the recurrence rate after 2 y was 13 in group A and 12 in group B. Conclusion : Age of onset of seizures, gender, frequency of seizures before therapy, and 2-y remission rate were not significantly different in the 2 groups. However, neuroimaging abnormalities, the time to achieving seizure control from the initial anticonvulsant treatment, and the recurrence rate after being seizure-free for 2 y were significantly different in the 2 groups.

• Clinical and Experimental Pediatrics
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• v.52 no.1
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• pp.99-104
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• 2009

Purpose : Magnetic resonance diffusion tensor imaging-based three-dimensional fiber tractography (DTI-FT) is a new method which demonstrates the orientation and integrity of white matter fibers in vivo. However, clinical application on children with cerebral palsy is still under investigation. We present various abnormal patterns of DTI-FT findings and accordance rate with clinical findings in children with hemiplegic cerebral palsy, to recognize the use fulness of DTI-FT. Methods : The thirteen children with hemiplegic cerebral palsy evaluated at Yeungnam University hospital from March, 2003 to August, 2007 were enrolled in this study and underwent magnetic resonance DTI-FT of the corticospinal tracts. Two regions of interest (ROI) were applied and the termination criteria were fractional anisotropy ${\geq}0.3$, angle ${\leq}70^{\circ}$. Results : The patterns and distribution of abnormal DTI-based corticospinal tractographic findings were interruption(10 cases, 76.9%), reduction of fiber volume (8 cases, 61.5%), agenesis of corticospinal tract (3 cases, 23.1%), transcallosal fiber (2 cases, 15.4%) and, aberrant corticospinal tracts (4 cases, 30.8%). Abnormal DTI-based corticospinal tractographic findings were in accordance with the clinical findings of cerebral palsy in 84.6% of the enrolled patients. Conclusion : Our results suggest that DTI-FT would be a use ful modality in the assessment of the corticospinal tract abnormalities in children with hemiplegic cerebral palsy.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.227-236
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• 2002

Purpose : A study was done to assess the incidence and classification of congenital urinary tract anomalies detected by antenatal ultrasonogram. Methods : We reviewed 558 cases of urinary tract anomaly which were detected by antenatal ultrasonogram and postnatally confirmed between June 1989 and May 2002. We investigated the incidence and classified congenital urinary tract anomalies by review of medical records, antenatal and postnatal radiologic studies retrospectively. Results : In 558 cases of congenital urinary tract anomalies, 292 cases of hydronephrosis were found and the most common. Another anomalies were composed of 65 cases of multicystic dysplastic kidney, 32 cases of hydroureteronephrosis, 31 cases of duplication of kidney, 25 cases of renal agenesis, 21 cases of simple renal cyst, 20 cases of polycystic disease, 13 cases of ureterocele, 11 cases of renal hypoplasia, 10 cases of horseshoe kidney, 9 cases of vesicoureteral reflux, 8 cases of posterior urethral valve, 7 cases of bladder diverticulum, 6 casts of megaureter, 5 cases of ectopia, 2 cases of megacystis, and 1 case of medullary cystic disease. In 82 of the 558 cases, there were two or more combined urinary tract anomalies. Associated diseases other than urinary tract were observed in 13 cases, of which the congenital heart disease was the most common. Conclusion : The congenital urinary tract anomaly is frequently found and diverse during the antenatal ultrasonography. The multicenter study is needed to investigate precise incidence and distribution of each anomalies in general population.

• Childhood Kidney Diseases
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• v.3 no.1
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• pp.88-94
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• 1999

Purpose : Malformation of urinary tract is among the most common of all congenital anomalies and can progress to irreversible renal damage before diagnosis due to difficulty of early diagnosis. Present study was undertaken to determine the clinical characteristics of urinary tract anomaly and to find out the most appropriate diagnostic and therapeutic measures for children with these anomalies. Methods : During the past 10 years from 1987 to 1998, review of medical records revealed 65 children with congenital anomaly of urinary tract and the following results were obtained. Results : The most common anomalies were ureteropelvic junction obstruction occuring in 26 cases ($36\%$), followed by ureteral duplication in 11 cases, renal agenesis in 10 cases and ureterovesical function obstruction in 7 cases. Complex anomaly of urinary tract was found in 8 cases and anomaly of other systems such as congenital heart disease was detected in 11 cases. The most frequent age group at the time of diagnosis was below 1 year of age constituting 39 cases ($60\%$) and male preponderance was noted as male to female ratio being 2.25:1. Presenting symptoms were urinary tract infection in 25 cases, followed by hematuria, abdominal mass, abdominal pain and voiding difficulty, etc, and in 11 cases, the anomaly was picked up by routine prenatal ultrasonography. Azotemia was noted in 9 cases and the underlying anomaly was obstructive uropathy in 4 out of these 9 cases. Surgical correction was undertaken in 38 cases (most frequently in cases of obstructive uropathy) and in 2 out off cases with obstructive uropathy in whom surgical correction was done, azotemia disappeared during follow up period of 1-5years. No new cases of deteriorating renal function appeared during follow-up period. Conclusion : In spite of high incidence of congenital malformation of urinary tract, early diagnosis is still hampered by nonspecific symptoms and signs. Therefore, in patients with symptoms such as urinary tract infection, abdominal pain and voiding problems, etc, it Is advisable to take various diagnostic tests promptly to pick up any urinary tract anomaly and to apply proper therapy in order to avoid progression to irreversible renal damage. In this regard, prenatal ultrasonography should be utilized more widely as a routine procedure to detect any urinary tract anomalies before birth.

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.31-37
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• 1997

The DMSA scan is a useful radiologic study in diagnosis of morphologic and functional diseases of kidney. We evaluated the distribution of sex and age, clinical manifestations, diagnosis, combined diseases, treatment and prognosis of the 61 patients with non-functioning kidney(no isotope uptake or uptake below 5% in DMSA scan) who admitted in our hospital from 1980 to 1995. The proportion of patients under 1 year old age was 46%. Sex ratio was 1.4:1 with male predominance. Most diagnosis of non-functioning kidneys were congenital such as multicystic dysplastic kidney, hydronephrosis due to ureteropelvic junction obstruction, renal agenesis and renal hypoplasia. In order of frequency thirty one percent of them were previously detected on antenatal ultrasonogram. Treatment consisted of operation in 47.5%, mostly nephrectomy and 32.8% of patients were followed up at OPD base without definite treatment. The most common combined diseases was hydronephrosis, in 4patients who had both kidneys inveloved progressed to chronic renal failure, but the prognosis in most cases were good. It is important to evaluate renal diseases in perinatal periods, and we believe that highly sensitive diagnostic study contribute to early treatment plan and thus to good prognosis.

• Childhood Kidney Diseases
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• v.14 no.1
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• pp.42-50
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• 2010

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.