Cobb syndrome is a rare neurocutaneous angiomatosis characterized by a vascular skin nevus associated with a spinal cord angioma of the same metamere. A 14-year-old girl had an asymptomatic large cutaneous hemangioma distributed from the TI dermatome downward to the L3 dermatome since birth and complained of a gait disturbance and urination difficulty for 1 year. A biopsy specimen in the skin lesion revealed the findings of capillary hemangioma. From C7 downward to L4 posterior epidural hemangioma composed of arteriovenous and cavernous components was diagnosed by radiological examination and surgical exploration. Because of very extensive cord hemangioma, only partial removal of the tumor at T11, T12 and L1 level was performed and postoperatively she was transferred to a special facility for rehabilitative therapy.
Adolescent ; Angiomatosis ; Biopsy ; Female ; Gait ; Hemangioma ; Hemangioma, Capillary ; Humans ; Nevus ; Parturition ; Skin ; Spinal Cord ; Urination

Valproate is widely used because of broad spectrum of action, but it can produce an encephalopathy resulting from hyperammonemia even at the therapeutic range of valproate and is called as valproate-induced encephalopathy (VHE). Delay in recognition of VHE can result in the development of potentially life-threatening complications. Fortunately, it is reversible with discontinuing valproate. A 65-year-old man became progressively lethargic with impaired gait and poor cognitive function while taking valproate as alternative to zonisamide. Routine investigations of admission profiles were performed but revealed no abnormalities. Next, we checked serum ammonia level to identify other possible causes and detected hyperammonemia despite the therapeutic range of valproate in the absence of any abnormalities in liver enzymes. On cessation of valproate, he has achieved dramatic clinical improvement including the reversal of hyperammonemia. We confirmed the diagnosis of VHE. This emphasizes the importance of rapid diagnosis and proper management of VHE in order to prevent the neurological damage and minimize complications.
Aged ; Ammonia ; Gait ; Humans ; Hyperammonemia ; Isoxazoles ; Liver ; Valproic Acid

Traumatic dislocation of the hip presents serious problems that may lead to avascular necrosis, nerve palsy, post-traumatic arthritis even when reduction is promptly and adequately carried out. Among them avascular necrosis is the most important prognostic factor, so the early detection of this complication has critical significance for final result. Bone scan has been considered one of early diagnostic test, but recently MRI replaced its role for imaging in the detection of early AVN, treatment monitoring and prediction of prognosis. Authors prospectively studied the early finding of MRI to detect avascular necrosis of the femoral head and to predict prognosis in traumatic hip dislocation and fracture-dislocation. From December 1990 to November 1994, 12 case of dislocation and fracture-dislocation of the hip were studied . In follow up period ranging from 19 months to 65 months, 10 case had excellent or good result. All cases had abnormal MRI finding such as paraarticular edema, capsular bulging, joint effusion, femoral head fracture, but findings such as femoral head signal changes were dectected in only 3 cases. Among the 3 cases, 1 case had developed AVN and 2 cases were uneventful. Other complications were 3 cases of posttraumatic arthritis, 2 cases of incomplete sciatic nerve palsy, and 1 case of heterotrophic ossification. Although early MRI finding at posttrauma 2weeks gave less specific information, it could give good information for risk group and follow up control with more cases. Although it is expensive, MRI can be one of the most reliable reference in early diagnosis of AVN and prediction of prognosis in traumatic dislocation and fracture- dislocation of the hip with non-invasive method.
Arthritis ; Diagnostic Tests, Routine ; Dislocations ; Early Diagnosis ; Edema ; Follow-Up Studies ; Head* ; Hip Dislocation* ; Hip Joint ; Hip* ; Joints ; Magnetic Resonance Imaging* ; Necrosis ; Paralysis ; Prognosis ; Prospective Studies ; Sciatic Neuropathy

Seidel nail provides more advantages for treatment of humeral shaft fracture than other treatment modalities. It enables patients to get high bone union rate, makes surgical technigne less invasive, and allows early mobilization mobilization so that the patients is subjected to more comfortable treatment. But, Seidel nail is apt to injure the rotator cuff and has relatively weak holding power of distal fragment of fracture so that it may result in distraction and rotatory instability, and make nonunion and impaired shoulder function. Thus, in order to the functional and radiological results, complications and technical problems, we evaluated the efficacy of Seidel nail in treatment of 20 cases of hurneral shaft fractures from March l994 to March 1996, retrospectively. The results were as follows. 1. 18 cases(90%) achieved radiological union. 2. The time of union was 12.5 weeks in average. 3. 17 cases achieved satisfactory results according to Neers functional score. 4. The complications included proximal protrusion of nail from the entry site(10%), fracture of greater tuberosity of proximal humerus(5%), loosening of distal spreading screw(10%), and nonunion( l 0%). In conclusion, Seidel nailing is one of good treatment modalities for humeral shaft fracture, but skillful operative technique is needed to obtain the satisfactory functional result. And also, it is necessory to modify the distal locking system of Seidel nail to reinforce the rotational stahility of the fracture site.
Early Ambulation ; Humans ; Humerus ; Retrospective Studies ; Rotator Cuff ; Shoulder

Conventional laparoscopic surgery requires definite leaming curve, and must be converted to an open procedure in the event of a large vessel injury. The usage of carbon dioxide gas for securing operative field is generally accepted, but such field rapidly disappears when air pressure decreases. To overcome these shortcomings without sacrificing advantages of excellent cosmetic results and reduced wound pain of laparoscopy without using the gas insufflation, various urologic operations utilizing incisions 2 to 5 cm long were made without cutting abdominal musculature with the help of specially designed retractors and video systems used in conventional laparoscopic surgery. It can be performed in familiar anatomical settings and all the surgical skills of open procedures can be utilized. We have performed 3 living donor nephrectomies, 6 simple nephrectomies, 2 partial nephrectomies, 9 pyeloplasties, 16 ureterolithotomies, 1 ureteroureterostomy, and 2 renal cyst marsupializations. Mean operative time and hospital stay were 185 minutes and 7 days for living donor nephrectomies, 150 minutes and 6.7 days for simple nephrectomies, 255 minutes and 7.5 days for partial nephrectomies, 130 minutes and 4.8 days for pyeloplasties, 85 minutes and 4.1 days for ureterolithotomies, 90 minutes and 5 days for ureteroureterostomy, and 75 minutes and 5.5 days for renal cyst marsupializations, respectively. Immediate wound pain was severe probably due to intense traction of abdominal musculature, but the recovery of such pain was remarkably rapid. No postoperative wound paresthesia was seen and early discharge was possible. In conclusion, the gasless retroperitoneoscopically assisted surgery can take advantage of both conventional open and laparoscopic surgery, and it can be a valid option to conventional open surgery for any surgical procedures in retroperitoneum, including kidney, ureter, and bladder.
Air Pressure ; Carbon Dioxide ; Humans ; Insufflation ; Kidney ; Laparoscopy ; Length of Stay ; Living Donors ; Nephrectomy ; Operative Time ; Paresthesia ; Traction ; Ureter ; Urinary Bladder ; Wounds and Injuries

Purpose: We present a case of internal astigmatism correction using a toric scleral contact lens in a patient with high myopia after implantable collamer lens (ICL) surgery.Case summary: A 45-year-old man presented with a significant decrease in visual acuity despite previous ICL surgery. Initial attempts to fit a corneal rigid gas permeable contact lens to the left eye were unsuccessful due to fitting challenges. Subsequently, the use of a spherical scleral contact lens effectively corrected corneal astigmatism but failed to address internal astigmatism, thereby limiting improvement in visual acuity. Postoperative corneal ectasia and ICL rotation were suspected to be the underlying causes of this astigmatism. The introduction of a front toric scleral lens resulted in a significant improvement in visual acuity and sustained comfort throughout the 3-month follow-up period. Conclusions The use of toric scleral lenses can significantly improve visual acuity and provide exceptional comfort in cases where corneal morphology and lens position change after ICL surgery. This modality presents a promising alternative for future consideration.

Periodontal disease is a predictor of stroke and cognitive impairment. The association between the number of lost teeth (an indicator of periodontal disease) and silent infarcts and cerebral white matter changes on brain CT was investigated in community-dwelling adults without dementia or stroke. Dental examination and CT were performed in 438 stroke- and dementia-free subjects older than 50 yr (mean age, 63 +/- 7.9 yr), who were recruited for an early health check-up program as part of the Prevention of Stroke and Dementia (PRESENT) project between 2009 and 2010. In unadjusted analyses, the odds ratio (OR) for silent cerebral infarcts and cerebral white matter changes for subjects with 6-10 and > 10 lost teeth was 2.3 (95% CI, 1.38-4.39; P = 0.006) and 4.2 (95% CI, 1.57-5.64; P < 0.001), respectively, as compared to subjects with 0-5 lost teeth. After adjustment for age, education, hypertension, diabetes mellitus, hyperlipidemia, and smoking, the ORs were 1.7 (95% CI, 1.08-3.69; P = 0.12) and 3.9 (95% CI, 1.27-5.02; P < 0.001), respectively. These findings suggest that severe tooth loss may be a predictor of silent cerebral infarcts and cerebral white matter changes in community-dwelling, stroke- and dementia-free adults.
Age Factors ; Aged ; Alzheimer Disease/diagnosis ; Brain/*radiography ; Cross-Sectional Studies ; Dementia/pathology/prevention & control ; Diabetes Complications/diagnosis ; Female ; Humans ; Hyperlipidemias/complications ; Hypertension/complications ; Interviews as Topic ; Male ; Middle Aged ; Odds Ratio ; Periodontal Diseases/complications/*diagnosis ; Predictive Value of Tests ; Risk Factors ; Stroke/pathology/prevention & control ; Tomography, X-Ray Computed ; Tooth Loss

PURPOSE: To report a case of retinal detachment surgery in a patient with oculocutaneous albinism. CASE SUMMARY: A 44-year-old man visited our clinic complaining of decreased visual acuity in his left eye. His best corrected visual acuity was hand movement in his left eye, and rhegmatogenous retinal detachment involving the macula at the superior temporal site was found. We performed pars plana vitrectomy and attempted to reattach the retina using endolaser photocoagulation; however, the laser burn was not made, and we failed to reattach the retina. At that point, we carried out cryopexy around the retinal tear, and injected silicone oil into the vitreous cavity. Ten months after surgery, his best corrected visual acuity was 0.06, and there was no recurrent retinal detachment or proliferative vitreoretinopathy. CONCLUSIONS: In patients with albinism with melanin deficiency, cryopexy is more useful than laser photocoagulation for retinal detachment surgery.
Adult ; Albinism ; Albinism, Oculocutaneous ; Burns ; Eye ; Hand ; Humans ; Light Coagulation ; Melanins ; Retina ; Retinal Detachment ; Retinal Perforations ; Retinaldehyde ; Silicone Oils ; Visual Acuity ; Vitrectomy ; Vitreoretinopathy, Proliferative

Purpose: To report successful graft reattachment using atropine after Descemet’s membrane stripping automated endothelial keratoplasty (DSAEK) in iris deformity.Case summary: A 76-year-old male was referred for decreased visual acuity due to bullous keratopathy in his right eye. He had previously undergone several eye surgeries, including vitrectomy and intraocular lens fixation, because of retinal detachment, which caused the iris deformity. DSAEK was performed, but the graft detached because of an air bubble in the posterior chamber. Atropine was used to move the air into the anterior chamber. The graft was attached the next day, and maintained during 3 months of follow-up. Conclusions Atropine is an effective treatment option for cases with air bubble migration to the posterior chamber, leading to graft detachment after DSAEK in iris deformity.

Leiomyosarcoma of the penis is an extremely rare disease. We have encountered a case of leiomyosarcoma of the penis in a 45 year old man. On staging work up. local invasion to corpus cavernosum. but no evidence of lymph node metastasis was evident. After the confirmation of leiomyosarcoma by excisional biopsy, the patient underwent partial penectemy. Herein we report this case with the review of literatures.
Biopsy ; Humans ; Leiomyosarcoma* ; Lymph Nodes ; Male ; Middle Aged ; Neoplasm Metastasis ; Penis* ; Rare Diseases