• Journal of Korean Neurosurgical Society
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• v.63 no.4
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• pp.415-426
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• 2020

Arteriovenous malformations (AVMs) are congenital anomalies of the cerebrovascular system. AVM harbors 2.2% annual hemorrhage risk in unruptured cases and 4.5% annual hemorrhage risk of previously ruptured cases. Stereotactic radiosurgery (SRS) have been shown excellent treatment outcomes for patients with small- to moderated sized AVM which can be achieved in 80-90% complete obliteration rate with a 2-3 years latency period. The most important factors are associated with obliteration after SRS is the radiation dose to the AVM. In our institutional clinical practice, now 22 Gy (50% isodose line) dose of radiation has been used for treatment of cerebral AVM in single-session radiosurgery. However, dose-volume relationship can be unfavorable for large AVMs when treated in a single-session radiosurgery, resulting high complication rates for effective dose. Thus, various strategies should be considered to treat large AVM. The role of pre-SRS embolization is permanent volume reduction of the nidus and treat high-risk lesion such as AVM-related aneurysm and high-flow arteriovenous shunt. Various staging technique of radiosurgery including volume-staged radiosurgery, hypofractionated radiotherapy and dose-staged radiosurgery are possible option for large AVM. The incidence of post-radiosurgery complication is varied, the incidence rate of radiological post-radiosurgical complication has been reported 30-40% and symptomatic complication rate was reported from 8.1% to 11.8%. In the future, novel therapy which incorporate endovascular treatment using liquid embolic material and new radiosurgical technique such as gene or cytokine-targeted radio-sensitization should be needed.

• The Journal of Korean Academy of Orthopedic Manual Physical Therapy
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• v.22 no.2
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• pp.35-39
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• 2016

Background: The superficial lymphatic system is divided into areas called lymphatic territories which are separated by watersheds. When the lymphatic system fails to remove its load either due to surgery, radiotherapy or some congenital malformation of it then the fluid and the proteins and wastes contained within it accumulates in that territory. Anastomotic connections exist across the watersheds and while they can work unaided manual lymph drainage (MLD) can significantly help drainage across them into unaffected lymphatic territories. The purpose of the study is to examine the effectiveness of a manual technique in moving fluids and softening hardened tissues using a tape measure and Patient-Specific Functional Scale. Methods: We examined the movement of fluids from the affected limbs of lymphedema patients who underwent a standardized 30-min treatment using the Dr. Vodder method of MLD. We chose a typical cross section of patients with secondary leg or secondary arm lymphedema. The lymphedema patient was also measured after the conclusion of treatment and underwent a follow-up control measurement, within 8 weeks. Both evaluation tools indicated a movement of fluid to different and unblocked lymphatic territories as well as a softening of tissues in some of the affected limbs. Results: MLD is an effective means of fluid clearance when it accumulates as a consequence of a failure of the lymphatic system. It seems likely that MLD has a systemic effect on the lymphatic system and that it can improve flow from otherwise normal tissues. Conclusions: It is hypothesized that a series of treatments would result in even more significant improvements.

• Journal of Veterinary Clinics
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• v.26 no.5
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• pp.467-471
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• 2009

This report describes Neural Tube Defects (NTDs) with Abdominal Wall Defects (AWDs) on the sibling of Yorkshire terriers. The NTDs and AWDs are rare serious congenital defects. The NTDs are neurulation abnormality that results from to failed transformation of the neurual tube by the incomplete closure of the embryonic neural plate. These dysraphic states range form mild to severe according to developmental malformation that include fusion defects of skull (crania bifida; CB) and fusion defects of vertebrae (spina bifida; SB). The AWDs are genetic defects that results from to failed formation of abdominal wall and cavity. These dysraphic states are omphalocele and gastroschisis. The 12-month dam was delivered by caesarian section and 4 littermate had obvious malformations. One male dead stillbirth fetus (L1) was revealed the extruded abdominal viscera, omphalocele. One female fetus (L2) was died within 1 hour after birth with defects of abdominal muscle upper umbilicus, gastroschisis. 3rd fetus (L3) was died within 36 hours after parturition and revealed a copious dermal and vertebral defects on the midline thorax, upper SB asperta. 4th fetus (L4) is still growing well now at 6 months but at the 2 week age, appears hairy nevus on the frontal cranium and dorsal thoracic portion. The radiograph of L1 and L2 are shown decrease bony density of calvarium and L3 was shown defect of spinose processes of the T9-T13. On our knowledge, this is first report of the SB and CB in Yorkshire terrier. And also sibling of NTDs with AWDs that has not previously been reported in the dog.

• Childhood Kidney Diseases
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• v.18 no.2
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• pp.116-122
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• 2014

Renal and perinephric abscesses are a rare but potentially fatal complication of urinary tract infection (UTI). Diagnosing renal and perinephric abscesses has been known to be difficult. These abscesses may occur when the appropriate antibiotic treatment for a UTI is delayed, or in cases with a congenital malformation of the urinary system, especially in children. In the present report, we describe 2 cases of renal abscesses with extra-capsular invasion in children with febrile UTI. A 4-month-old male infant with vesicoureteral reflux developed a renal abscess that infiltrated the perinephric area and the left psoas muscle, despite early antibiotic treatment. A 9-year-old boy with prolonged fever also showed a multi-loculated renal abscess that infiltrated the spleen and diaphragm. Both patients were successfully treated with appropriate antibiotics and percutaneous drainage.

• Journal of Chest Surgery
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• v.19 no.4
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• pp.618-626
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• 1986

Univentricular heart is a rare congenital cardiac anomaly in which the atrial chambers are connected to only one ventricular chamber and it consists of a diverse group of cardiac malformation characterized by both AV valves or a common AV valve opening into the same ventricle, or the presence of only a solitary AV valve. In spite of recent development in cardiac surgery, corrective operations for univentricular heart still have high mortality and complication rate. Twenty eight patients underwent corrective operation for univentricular heart at Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital from February 1979 to July 1986. Of the 28 patients, 7 patients were operated on by ventricular septation and 21 patients by modified Fontan operation. Of the 28 patients, 19 patients were male and 9 patients female and ages ranged from 5 months to 18 years old with the average age of 7.3 years. There were 2 mortalities in 7 patients operated on by septation with the mortality rate of 28.6% and 5 complications, 3 complete AV block, 1 low cardiac output and 1 arrhythmia. All survived patients are being followed up without specific problem till now. There were 10 mortalities in 21 patients operated on by modified Fontan operation with the mortality rate of 47.6% and 10 complications, 2 low cardiac output, 2 respiratory failure necessitating tracheostomy, 2 persistent cyanosis, 2 arrhythmia, 1 missing of left AV valve in situs inversus patient due to misdiagnosis and one rupture of closed right AV valve. Incremental risk factors for operative mortality are young age less than 5 years old, anomalous pulmonary and systemic venous drainage and atrial septation procedure. In 11 survived patients, 9 patients show good follow-up results but one patient complains of persistent cyanosis and another one patient is suffered from CHF. In our series, results of corrective operation for univentricular heart shows continuing improvement but still high mortality and complication rate. So there must be continuing improvement in surgical result by selection of patient, by adequate decision making for timing and method of operation and by improving operative methods.

• Korean Journal of Head & Neck Oncology
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• v.27 no.1
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• pp.84-87
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• 2011

Branchio-otic syndrome(BOS) is a relatively uncommon genetic malformation associated with dysmorphogenesis of the first and second branchial arches and is characterized by branchial fistulae, congenital preauricular fistulae, and anomalies of the pinnae, external, middle, and inner ears, accompanied by hearing loss. Recently, we experienced a case of BOS in a 10 years old female patient and report this case with a review of literature. 10-year-old girl presented with hearing impairment, bilateral preauricular fistula and cervical fistula. The pure tone audiometry revealed that she had 60dB sensorineural hearing loss on right side and 90dB mixed hearing loss on left. Bilateral branchial fistula was found on the neck CT scan and bilateral ossicular and cochlear abnormality combined with enlarged internal auditory canal was noted on the temporal bone CT scan. To investigate the association with EYA1 gene, we performed DNA sequncing with peripheral white blood cell and found the point mutations on Exon 7, 12 and 16 of EYA1 gene. The preauricular fistula and branchial fistula was excised surgically and hearing aid was applied on her left side. There was no sign of fistula recurrence for seven years after the surgery.

• Childhood Kidney Diseases
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• v.12 no.2
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• pp.262-266
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• 2008

Crossed renal ectopia is a congenital malformation in which both kidneys lie on the same side of the spine, usually side by side longitudinally. More often on the right side. Fusion of the two renal units is eight times more common than nonfusion. Although crossed renal ectopia is uncommon, this unusual entity must be considered in an infant when cystic mass in the abdomen or pelvis paticularly if no kidney can be found on the opposite side. In many cases of crossed fused ectopia with multicystic dysplastic kidney(MCDK), the diagnosis can be strongly suspected from the sonogram, and no other studies may be necessary. However, both intravenous urography and isotope renography is useful to assess the function of the crossed kidney. Crossed renal ectopia and MCDKs are associated with a greater incidence of ureteropelvic junction obstruction and reflux. So, screening voiding cystourethrography should be performed. Very few studies of MCDK in the setting of crossed fused ectopia have been reported. We have experienced a 3-year-old boy with crossed fused renal ectopia with multicystic dysplasia.

• Journal of Chest Surgery
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• v.16 no.2
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• pp.157-163
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• 1983

Ebstein's anomaly is a rare congenital cardiac malformation oand the ideal surgical correction seems controversial at present, and some problems are left unsolved in the surgical correction of this anomaly. Between June 1978 and June 1982, 12 patients with Ebsteins' anomaly underwent corrective open heart surgery at Seoul National University Hospital. Except for one patient, who had no ASD, all had a huge right atrium, secundum type ASD, and definite atrialized right ventricle. Typically, displaced tricuspid valve leaflets were found in all cases, but the degree of displacement and deformity were variable. In the point of NYHA functional classification, five were in class II, six were in class III, and one was in class IV. Ten patients were operated on by tricuspid valve replacement and pication. Two patients were operated on only by plication and annuloplasty techniques, and in all cases, ASD was closed. Postoperatively, four patients suffered from complete A-V block, and two of them died immediately. The remaining two patients took pacemaker generator implantation with good results. The other eight patients were in good condition. Tricuspid valve replacement using tissue valve and plication of the atrialized rght ventricle seems to be a good method of surgical correction for Ebstein's anomaly.

• Korean Journal of Cleft Lip And Palate
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• v.15 no.2
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• pp.61-68
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• 2012

Cleft lip and palate is the most common congenital facial malformation and has a significant developmental, physical, and psychological impact on those with the deformity and their families. When treating the patients with unilateral cleft lip, many surgeons adopt the rotation advancement flap method originally developed by Millard, or the triangular flap technique developed by Tennison, Randall or the modifications of these techniques. Among these, Millard's rotation advancement flap method has its advantage in designing the flap using the patient's anatomic landmarks. For performing this rotation advancement technique, skillful operation is needed to obtain esthetically satisfactory results. Vomer flap sometimes is used to repair anterior hard palate in complete cleft lip and palate patients. Vomerine tissue is readily available in the vicinity of the palatal defect and elevation of the vomerine flap is relatively simple procedure. In this article, we will introduce the comprehensive vomer flap technique conjunction with primary lip closure and review the comparative studies of the outcome of simultaneous repair of cleft lip and cleft hard palate with Millard's rotation advancement method and vomer flap.

• The Journal of Korean Physical Therapy
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• v.12 no.2
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• pp.69-82
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• 2000

Physical therapists are exposured to radio-and microwave-frequency electromagnetic radiation by operating electrotherapy units. So there is few protection system in physical therapy room. Clinical pathology room and so on where various kins of electromagnetic instruments is used in hospital while protection failities like protection wall or protection glass is being used only in radiological room to reduce the damage of radiation. Acoording to Larsen's survey on female physical therapist in denmark. it was said that the percentage of congenital malfornation was $3.6\%$ and cadiac malformation made up $0.7\%$. It is likely that effect of electromagnetic fields on the result cannot be ruled out. Rita ouellet-Hellstron and Walter F. Steward insisted that the danger of abortion increase in the case of pregnant femeal physical therapist exposured to microwave diathermy. The intention of our study is arousing the necessity of microwave protection in P.T room and finding the proper method for physical therapist safe. The results of this study were as follows: 1. Each electrotherapy units are occurrenced the electromagnetic fields, and specially amply occurrenced in H.P,I.C.T 2 unit operating, M.W.D unit head on parallel, S.W.D unit head on parallel. all electrotherapy units are operating. 2. There were electric fields mount are consideration to species of electrotherapy units(p<.05). 3. There were magnetic fields mount are consideration to species of electrotherapy units(p<.05). 4. There were electric fields mount are consideration to distance of electrotherapy units(p<.05). 7. There were magnetic fields mount are consideration nut to distance of electrotherapy units(p>.05). 8. Before and after protection on magnetic fields mount are consideration to all distance(0m, 0.3m, 1m, 3m, 5m)(p<.05) 9. Before and after protection on electric fields mount are consideration to 0m, 1m, 3m distance(p<.05), and consideration not to 0.3m, 5m distance(p>.05) 10. After protection fellow the each electrotherapy units. distance, intencity to electromagnetic fields are reduced(p<.05).