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A 60-year-old man underwent low anterior resection for rectal cancer. Histological findings indicated well-differentiated adenocarcinoma(T3[SS]N1M0, ly3, v2, Stage III a). Two years and 1 month later, right adrenalectomy was performed for solitary adrenal metastasis. Three months thereafter, left partial pulmonary resection was performed for a metastatic lung tumor. All resected specimens showed metastatic adenocarcinoma derived from the rectal cancer. The patient is alive and well without recurrence for more than 10years after lung resection. Given that adrenal metastasis is usually found as widespread metastasis, aggressive resection of well-controlled metastatic lesions including those in the adrenal glands is recommended.

The bark of Prunus jamasakura Siebold (Pruni Cortex) has long been used in Japan as a folk remedy and is one of ingredients of the Kampo formula, Jumi-haidoku-to (JHT). JHT is used for treatment of skin diseases such as acne (acne vulgaris). According to Kampo medicinal sources, Quercus Cortex can be used in place of Pruni Cortex. In this study, we found that water extracts of Pruni Cortex, not Quercus Cortex showed a binding effect on estrogen receptor beta (ERbeta). Thus, their chemical analysis was carried out by GC-MS and found that five unique constituents (i.e., sakuranetin, naringenin, genistein, genkwanin and arctigenin) were detected in Pruni Cortex. The ERbeta binding capacity of these constituents was examined using 70 ng/ml 17beta-estradiol, as the positive control with 100% ERbeta binding. Among them, genistein (60% at 10 ng/ml) showed the strongest binding capacity than naringenin (60% at 1000 ng/ml) and sakuranetin (40% at 1000 ng/ml). These results suggested that Pruni Cortex in JHT could play an important role in treatment of acne. In addition, those of Pruni Cortex from different harvest places were also examined in their chemical contents and ERbeta binding capacity. The extracts of Pruni Cortex from Kyushu in Japan and Anhui Province in China showed higher contents of genistein and stronger ERbeta binding capacity than that of Pruni Cortex from Tokushima Prefecture in Japan.

A 12-year-old-man presented with left flank pain after a traffic accident on October 14, 2006. Computed tomography (CT) revealed major left renal hematoma and transection (IIIb). Selectively transarterial embolization (TAE) was performed to control upper transected renal bleeding on the same day, and again to do rebleeding two days later. Because CT revealed left perirenal urinoma caused by upper transected kidney on October 18, TAE was performed for the upper transected kidney not to function. Five months after left renal injury, CT demonstrated the left kidney successfully preserved without hydronephrosis, urinoma and hematoma. The patient was well and could be conservatively treated without hypertension and other complications. In previous reports, only a part of renal injury (III) cases with conservative treatment converted to nephrectomy, whereas approximately half of them with surgical treatment resulted in nephrectomy. Therefore, it is important to treat them as conservatively as possible and to preserve renal function, even in cases of major renal blunt injury.

A 61-year-old man suddenly heard tinnitus and diplopia at night during watchinng television. A few days later he visited at our hospital. Neurologically he exibited marked isolated right superior rectus palsy which was also indicated by the Hess test. No other neurological abnormalities were found such as other ocular muscle paresis, cranial nerve palsies, hemiparesis, sensory impairement or cerebellar ataxia. MRI showed a left medial thalamic infarction extending to a rostral part of the midbrain anterolateral to the cerebral aqueduct at the superior colliculi level. Unilateral superior rectus palsy can rarely be caused by a contralateral midbrain infarction, because fibers from the subnucleus subserving the superior rectus decussate within the oculomoter nerve complex. In this case the crossing fibers toward the contralateral superior rectus may have been selectively involved by a tinny lesion in the area of the oculomotor nucleus. The patient had a slightly narrowed right palpebral fissure. It is indicated that crossing fibers toward the contralateral levator muscle of the eyelid may be also involved. The patient's diplopia completely resolved two months later after the onset.

17 cases of patients with diabetes mellitus who had urinary frequency symptom for which anti-cholinergic agents proved ineffective were given Sarpogrelate Hydrochloride (Anplag), a selective 5-HT 2 receptor antagonist. Efficacy was judged using IPSS and QOL scores after 2 weeks medications, these showed that all cases had improved their urinary frequency during the days as well as the night. This was especially true for the QOL score. One time urinary volume markedly increased, but there was no statistical significance after medication in maximum flow rate and residual urine. At the same time, a separate group of 14 mainly BPH cases did not improve entirely. It is believed that reaction in the detrusor muscle with hyperreflexia of diabetes mellitus patients can reach 5-HT, and its reaction is believed to reach via the 5-HT 2 receptor. This paper is a first clinical report of making use of 5-HT 2 antagonist as hyperactive detrusor on diabetes mellitus patients.

Sixty-eight hydrocephalic patients were treated with a shunt system that incorporated a SOPHY programmable valve. This device is very effective for treating disorders of CSF circulation. However, when the valve is placed on the scalp, it produces artifacts on MR imaging due to its small magnetic rotor, and may cause necrosis of the scalp above it. On the other hand, when it is positioned on the chest, MR artifacts decrease and exchanging the valve devices becomes easier. Therefore, we have positioned the device on the chest. However, some complications were encountered, such as the turning over of the valve in the subcutaneous pocket and the twisting of the shunt tube. Moreover, the shunt tube may stretch due to growth of the neck during development in infantile patients or extension of the neck during exercise. These complications are associated only with subcutaneous placement on the chest, so we conclude that such a valve, if placed on the chest, should be placed in a lower position on the chest, and if MR examination will not be performed in the foreseeable future, we recommend that it be placed on the occipital scalp, especially in infantile patients.

Intracranial malignant epidermoid is uncommon. We report a case of a 74-year-old woman with a primary intracranial malignant epidermoid in the parapontine region. The tumor was visualized as two components (a low-density benign prepontine lesion and a malignant enhanced cerebellopontine angle lesion) on CT scan. On MRI, the benign portion demonstrated low-signal intensity on T1 weighted images and markedly increased heterogeneous signal intensity on T2 weighted images, while the malignant portion showed iso-signal intensity on T1 weighted images that was markedly enhanced and heterogeneous signal intensity on T2 weighted images. Lack of contrast enhancement is a typical finding in benign epidermoid, although rim enhancement has been described in a few cases. In our patient, the contrast enhancement on CT scan in the otherwise typical epidermoid cyst suggested malignant degeneration. Using MR imaging, we could distinguish malignant epidermoid from benign epidermoid cyst more precisely.

Immunological effect of recombinant interferon gamma (IFN-gamma) was studied in 18 cases of renal cell carcinoma. Sixteen of them were administered IFN-gamma as post-operative adjuvant therapy. Two of them were administered IFN-gamma for metastatic renal cell carcinoma. All of them were followed for at least 1 year. We divided the patients into two groups. One group subcutaneously received IFN-gamma every other day for 16 days, then 4 weeks later every week for one year (group A), and the other group, received alternate subcutaneous IFN-gamma every other day for 16 days and every week for 4 weeks (group B). For immunological testing, peripheral blood lymphocytes were obtained before treatment on day 1, 2 weeks later, 6 months later and 12 months later. The cells were tested for natural killer activity (NK), antibody dependent cellular cytotoxicity (ADCC), positive lymphocytes of CD3, CD4, CD8 and CD11b. Both ADCC and NK activity were increased after therapy, especially in group B. CD3, CD4 and CD8 positive lymphocytes were increased by means of IFN-gamma. On the other hand, CD11b-positive lymphocytes could not be elicited in group B. Tumor regression was observed in 2 cases of metastatic renal cell carcinomas. When clinical responses were observed, both ADCC and NK were elicited as compared with the pretreatment values. Our findings indicated that ADCC and NK could be elicited in group B.

Recently, we applied inhalation of recombinant interferon (IFN)-gamma, to 4 patients with pulmonary metastatic tumor (3 with renal cell carcinoma and 1 with renal pelvic tumor). Of the patients with renal cell carcinoma 2 had received previous nephrectomy, 1 patient had received embolization alone, 1 subcutaneous IFN-gamma in 1, and 1 systematic chemotherapy. All 4 patients inhaled 1 x 10(6) JR U-2 x 10(6) JR U recombinant IFN-gamma 3 times per day. Additionally, all metastatic renal cell carcinomas received subcutaneous injection of IFN-gamma. One patient with renal cell carcinoma achieved a complete response after inhalation therapy and 2 patients did not respond (NC: 1, PD: 1). A case of metastatic renal pelvic tumor did not respond. Immunohistochemical staining with antibody to Ber-MAC3 (stimulated macrophage) of this case revealed positive cells within the transitional cell carcinoma. According to these results, inhalation of IFN-gamma appears to be applicable as one treatment for pulmonary metastatic tumor of renal cell carcinoma.

We report the coexistence of multiple arteriovenous malformations (AVMs) and hemangioma of the skull in a 38-year-old man with associated varicose veins in his scalp. The patient also had hypotrophy of all right limb tissues with skin pigmentation and syndactyly of both feet. CT scans revealed intraventricular hemorrhage and a hemangioma of the skull at the posterior fossa. Because of the location of the arteriovenous malformations, surgical evacuation and resection were not attempted. The hemangioma was totally removed to decompress the posterior fossa. A few cases of regional phacomatosis with intracranial AVMs have been reported, but we could not find a report of the coexistence of intracranial multiple AVMs and hemangioma of the skull with associated varicose veins of the scalp and skeletal hypotrophy. We discuss on the etiology of the coexistence of regional phacomatosis and hemangioma and on the difference between our case and typical regional phacomatosis.

Transiently evoked otoacoustic emissions (TEOAE) should be used clinically as an objective and noninvasive screening test for auditory dysfunction in children. The features of TEOAE measured by "ILO88" with non linear click stimuli are discussed. The following results were obtained: 1) The normal range of a power spectrum was determined using 42 adults with normal hearing and compared with data for sensorineural hearing disturbance. The power spectrum of TEOAE in adults with normal hearing sloped downward at high frequency and was the same for right and left ears in both males and females. For comparison with sensorineural hearing disturbance, a significant correlation between the audiogram and power spectrum of TEOAE was sought. 2) Patients with otitis media with effusion (109 ears in 67 children) were examined by audiometry, tympanometry and TEOAE. The hearing disturbance threshold of 45 cases with TEOAE was lower than that in cases with no TEOAE. In tympanometry, low intratympanic pressure was noted in the absence of TEOAE. 3) TEOAE and spontaneous otoacoustic emission were examined in 42 adults with normal hearing and 27 neonates using the same probe and level of stimuli to clarify differences in TEOAE according to age. The amplitude of TEOAE and the highest peak of the frequency component at 4kHz in neonates exceeded those of adults. The peak stimuli recorded with the intracanal probe in neonates was also larger than that of adults. Three out of 15 neonates had spontaneous otoacoustic emission and essentially the same proportion was noted in young adults.(ABSTRACT TRUNCATED AT 250 WORDS)

The immunological effect of interferon-gamma (IFN-gamma) was investigated in 18 cases of renal cell carcinoma before and after the operation. In 6 patients, IFN-gamma was administered preoperatively for 21 days (administration group), while 12 patients underwent nephrectomy alone without preoperative treatment (control group). The peripheral immunological effects were measured at before and 12 days after the operation in the administration group and at 11 days in the control group. In the administration group, a marked increase was noted in the test of antibody dependent cell-mediated cytotoxicity (ADCC) activity and natural killer (NK) activity, and slight increase in CD4/8 and CD11b. In low stage cases, no appreciable effect was obtained by the administration of IFN-gamma. However, in high stage cases, IFN-gamma tended to increase the value of ADCC activity and NK activity. Immunohistochemical studies of tumor infiltrating lymphocytes in renal cell carcinoma showed a high incidence of CD8 and CD11b in the administration group. Moreover, the presence of CD8 was higher than that of CD4 in the administration group in contrast to the results of the peripheral blood analysis.

This study was conducted to assess the usefulness and limitation of high-resolution CT for evaluating the condition of ossicular chain in the middle ear. Preoperative CT findings of the ossicular chain were compared with the operative findings of ossicles in 26 patients with chronic otitis media or congenital ossicular anomaly who underwent tympanoplasty. Total defect of head of the malleus, body of the incus and long process of the incus were completely detected by high-resolution CT. But the reliability in detecting the defect of handle of the malleus and superstructure of the stapes were 33.3% and 60%, respectively. Defect of the I-S joint (1 case) and partial defect of stapes crus (2 cases) could not be diagnosed correctly in the preoperative estimation. Although these findings demonstrate the limitations of high-resolution CT in the diagnosis of ossicular chain, it will be diminished by the advanced space resolution of CT in the future.

This clinical research has been conducted to extend the understanding of pathophysiological mechanism involved in the tethered cord syndrome. In normal individuals, the filum terminale has elasticity similar to the rubber band. This allows cephalad or caudad movement of the lumbosacrococcygeal spinal cord while the spine is flexed or extended. This is based on the observation that the lowest pair of dentate ligaments spans from the T12 and L1 junction of cord segments to the dura between the dural exit of T12 and L1 nerve roots, and the spinal cord below T12 can freely ascends on flexion and descends in the spinal canal on extension of the spine. In 50 healthy human subjects, x-ray films of the thoracolumbosacral spine were studied. Lateral films of the spine were taken in the neutral, hyperextended and hyperflexed positions. The first group included 10 individuals whose ages ranged from 27 to 42 years. The distance between the middle of the T10 vertebra and L1-L2 interspace was measured. This distance was considered to represent the length of the lumbosacral cord segment (L1 through coccygeal cord segments). The second group included 40 individuals whose ages ranged from 17 to 50 years. The distance between the L1-L2 interspace and S1-S2 interspace was measured. The distance signified the distance between the caudal end of the spinal cord and attachment of filum terminale to the sacrum. There was a 3.5% (4.6mm by X-ray films) increase during hyperflexion in the distance measured in the first study.(ABSTRACT TRUNCATED AT 250 WORDS)

Expression of major heat shock and stress-induced protein, HSP70, is known to be under complex regulation in tumor cells. In this study, we investigated the alternations of cytokinetics and HSP70 expression by hyperthermia in the in vitro experimental systems, using two rat glioma cell lines, two human glioblastoma cell lines and rat glioblast cells. For hyperthermal treatment the flasks were placed in water baths warmed up at 41 -45 degrees C for 15 min. To determine the effect of hyperthermia on the cell cycle progression, the changes in the DNA distribution of the cell population were studied by flow cytometry (FCM). The levels of HSP70 protein were determined by immunoblot analysis. The relationship between cell cycle and HSP70 expression was investigated by FCM using PI and FITC-labelled HSP70 double staining technique. These results were as follows: 1) Compared with the control, hyperthermic treatment at 42 degrees C or 44 degrees C caused both 354A and T98G cells to accumulate in S phase 18 hours after treatment and G2/M phase after 6-18 hours. 2) Hyperthermic treatment at 42 degrees C caused C6 cells to accumulate in S phase 6 hours after treatment, whereas heat treatment at 44 degrees C caused C6 cells to accumulate in S phase after 18 hours and G2/M phase after 6 hours. 3) A172 cells were accumulated only in G2/M phase by hyperthermia. 4) Glioblast cells did not show the alterations of cytokinetics by heat treatment remarkably. 5) HSP70 protein synthesis were enhanced under hyperthermic conditions in all type of cells, whether primary glioblast or permanent glioma cell lines.(ABSTRACT TRUNCATED AT 250 WORDS)

Using a high definition television (HDTV) technique, we have designed and developed the real time microscopic image transmission system. The department of laboratory and pathology, 500 m apart, are connected by this system on a light fiber line. It consists of an electrically controlled microscope, a HDTV camera, a HDTV color monitor, and a video transmitter on the laboratory side, while on the pathology a remote microscope controller, a video receiver, and a HDTV color monitor of 22.5 (H)-39.5(W) cm in CRT size. By monitoring real time noiseless picture, pathologists can select the objective lenses, move the stage, and adjust the focus of microscope at their own office. Three-month trial revealed that this prototype system was quite useful for prompt histo-pathological diagnosis during surgical operations. So far as we know, this microscopic image transmission system with Hi-vision TV is the first one in practical use in this field.

Vasoactive intestinal polypeptide (VIP) containing nerves have been considered as one of the inhibitory nerve of non-adrenergic, non-cholinergic action at the bladder neck. To clarify the action of VIP, isovolumetric pressure at the bladder neck and intravesical pressure were examined in the unstimulated control condition as well as after electrical stimulation of the sacral nerves in male dogs. For the purpose of immunohistochemical studies, the bladder neck was extirpated immediately after the continuous electrical stimulation, and the VIP and acetylcholine esterase (AchE) were stained by Avidin Biotin Complex (ABC) method. The bladder neck pressure showed a marked decrease following unilateral sacral nerve stimulation, while intravesical pressure remained unchanged. Immunohistochemical studies revealed a lot of VIP positive deposits at the bladder neck after stimulation, which were absent in unstimulated control. However, AchE was well stained in both tissues, and no remarkable differences could be found in AchE staining between control and electrically stimulated tissues. In the present study, a plenty of VIP containing nerves and parasympathetic nerves were demonstrated in the bladder neck of canine. Thus, VIP is supposed to participate in the bladder neck opening by the strong smooth muscle relaxation under the influence of parasympathetic nerves.

Ten patients, 8 with hydrocephalus and 2 with a subdural fluid collection, were operated using a pressure-adjustable shunt system (SOPHY). The valve-pressure of this shunt device can be adjusted percutaneously by means of a specially designed magnet. In the postoperative course, the valve-pressure was reset in 7 patients. In four patients, valve-resetting was necessitated because of inappropriate valve pressure. In the others, resetting was performed in order to confirm shunt-independence. Especially, in the case with disproportional large communicating fourth ventricle, we could adjust valve-pressure three times without surgical procedure. Although the number of cases in this study is small, this shunt device seems to be efficient in treating both the disorders of CSF circulation and subdural fluid collections. However, this device must make an artifact in the MR imaging because of its small pieces of magnet. So we recommend the device is placed on the chest if the follow-up MR imaging is necessitated. And as it is larger than other kinds of shunt devices, it might cause the necrosis of the skin on the device, especially in the newborn. But we have never seen the necrotic change of the skin since our first operation using it.

A 59-year-old male had a fluctuating large mass on the sacral region existed after birth. Sagittal MRI (T1-weighted image) disclosed a large fluid space in the mass and also revealed that sacral nerves extended caudally in the cyst. Enlargement of the sac may result in stretching of the spinal cord.

Magnetic resonance imaging (MRI) was conducted on infants with lumbosacral lipomas. Based on Chapman's classification (1982), these MRIs were classified into three representative types: caudal, dorsal and transitional. As confirmed by intraoperative observations, the relation between the lipomas and the spinal cord was clearly disclosed in the preoperative MRI. However, in the dorsal type, the intraoperative view of the margin between the lipomas and spinal cord was different from the MRI findings. Postoperative MRI disclosed the existence of residual lipomas and the change in the low conus medullaris. Moreover, preoperative and postoperative changes in the hydromyelia could be observed.

The clinical effectiveness of KL 007 (oxybutynin hydrochloride) was studied on patients with urinary disturbance, mostly complaints of urinary frequency, who were suffering from nervous pollakisuria, irritable bladder or neurogenic bladder. Fifty two patients were administered KL 007 three times daily at the dose of 6 mg, 9 mg, or 12 mg per day for one or two weeks. An overall clinical effectiveness of KL 007 was seen in the cases of hypertonic bladder. As to the clinical effectiveness of subjective symptoms, KL 007 improved the symptoms of urinary frequency, urinary incontinence, discomfort upon micturition, urinary urgency and sense of residual urine. Transient side effects were observed in 23 cases (41.4%), and dry mouth was most observed. No severe side effects were noticed.

The mechanism of impact head injuries has generally been discussed on the basis of the cranium is a rigid box completely filled with incompressible contents. In this paper, two cases of head injuries in patients with severe craniectomized skull defects are reported and their conditions are discussed. The 1st case was a 46 year-old female with a large frontoparietal skull defect following surgery on a brain tumor. While hospitalized, she fell down from her bed (50 cm in height) and suffered strong impact on the craniectomized area. From immediately after the injury, there was flaccid hemiparesis, but CT scans showed no morphological changes. The 2nd case was a 12 year-old female with a major frontal craniectomized skull defect following surgery. She fell down in a corridor at school and suffered an impact injury in the defect area. For several days after the fall, she had a headache and nausea, but there were no other neurological deficits. In CT scans, there were findings of a cortical contusional hemorrhage. Wounds of the skull with a partial defect, i.e. "a semiclosed box" in the narrow sense, cause conditions which are difficult to explain by conventional dynamic traumatology. In this paper, these rare head injuries are discussed on the basis of these two cases experienced by the authors and the literature.

VIP-containing nerves have been considered as an inhibitory nerve of non-cholinergic, non-adrenergic action in the bladder neck. Using immunohistochemistry, VIP was stained in the bladder neck in 14 cases which involved 5 cases of bladder neck contracture in addition to 9 cases of benign prostatic hypertrophy (BPH). Consequently, the VIP immunoreactive nerve was not stained in the 4 cases of bladder neck contracture with a large quantity of residual urine which had the fibrosis of bladder neck, but in one case was stained without residual urine. In the BPH, 8 out of 9 cases were stained and they had small residual urine. It was fully stained in the cases without residual urine or with a small volume if they had it. The possible role of VIP in the bladder neck was supposed to be to maintain the bladder neck opening by the strong smooth muscle relaxation.

E-643, a newly developed alpha 1-adrenergic blocker with a postsynaptic action, was tried on 17 subjects, 10 patients with neurogenic bladder and 7 patients with lower urethral obstruction. The results of treatment were excellent in 5, good in 7, and poor in 5 of them, the rate of effectiveness therefore being 70.6%. The inhibitory effect of E-643 on the contractility of the detrusor was no stronger than that of other alpha-blockers. The rate of effectiveness for neurogenic bladders was as high as 80% whereas that for lower urethral obstruction was much lower: 57%. Concerning benign prostatic hyperplasia (B.P.H.) alone, this agent was markedly beneficial. In the case of B.P.H the urethra pressure in the prostatic urethra tends to drop and urination is facilitated even without a change in maximum urethral closure pressure, which reduces the amount of residual urine, this sequence of events taking place on condition that the contractility of the detrusor is adequate. According to urodynamic criteria, the underlying causes of the so-called bladder neck contracture can be classified into organic and functional obstruction, but it is mistaken to use this term for functional disturbance of the bladder neck. It seems worthwhile to determine the clinical effect of alpha-blockers on functional urethral obstruction. E-643 produced few if any appreciable signs or symptoms of side effects either subjectively or objectively.

A 39-year-old male experienced unilateral right hearing loss and tinnitus for 7 years and was hospitalized after he suddenly developed severe headache, vertigo, and right facial paralysis. Computed tomography (CT) showed a round, high-density are in the right cerebellopontine angle. Magnetic resonance (MR) imaging demonstrated a crescent-shaped region of high signal intensity, representing hemorrhage, in the superior aspect of the tumor, surrounded by edema. The right internal auditory canal was enlarged. Four-vessel angiography disclosed neither an aneurysm nor an arteriovenous malformation. A right suboccipital craniectomy revealed an encapsulated mass 3 cm in diameter in the right cerebellopontine angle. The tumor was totally removed. Histological examination revealed a typical neurinoma composed of Antoni type A and B cells. After undergoing anastomosis of the right hypoglossal and facial nerves, the patient was discharged in good condition. In this case MR imaging demonstrated intratumoral hemorrhage (which is rare in cases of acoustic neurinoma) and the surrounding tissue more clearly than did CT scanning.

The inside of an epidermoid is formed by masses of epithelial debris, desquamative keratin and cells derived from the epithelial capsule which accumulate very slowly within the capsule. As a result, the growth of the tumor is very slow, and the clinical signs are very diverse and bizarre because of the situation and extension of the epidermoids throughout the basal cisterns. A 52-year-old housewife was admitted to our clinic with complaints of dizziness and gait disturbance. The abnormal neurological findings were truncal ataxia, lt-trigeminal disturbance, lt-facial palsy, lt-hearing disturbance, lt-cerebellar signs (dysmetria, dysdiadochokinesia) and nystagmus. CT scan revealed low density area in the prepontine cistern and lt-cerebello-pontine angle, which was not enhanced following intravenous injection of the contrast medium. Metrizamide CT cisternography showed staining of the tumor heterogeneously revealing a honeycomb appearance. MRI showed the tumor clearly as well as the severe atrophy of the lt-cerebellar hemisphere and the pontine basal area. In this case atrophy of the cerebellar hemisphere and pons may have resulted from being compressed by the tumor. Because of this, we think it necessary to stress that early diagnosis and operation of the epidermoid are very important.

Klippel-Trenaunay Syndrome with hemicranial hypertrophy and macrodactyly is reported. The patient was a 3-year-old Japanese woman with an extensive nevus flammeus which extended over the right side of the neck, axilla, upper arm, and leg. Her right extremities were longer and thicker than left, and showed apparent macrodactyly in the right hand. Her skin of right side body was irregular, rough surface and was pigmented prominently. Hyperthermia was present in the hypertrophic limbs, but hyperhidrosis was none. She had moderate mental retardation, delayed speech. Her hemicranial hypertrophy (same side as hypertrophic limbs) was examined by CT scan. Her right lateral ventricle was dilatated and cortical sulci was well demonstrated. Her right hemicranial volume was enlarged but rt-cerebral hemisphere was atrophic, and her infratentorial structures were symmetrical. Presented case, Klippel-Trenaunay Syndrome with hemicranial hypertrophy, suggested the embryological theory affecting on trilaminar disc in embryonal stage as the genesis of this rare syndrome.