PURPOSE: Joubert syndrome presents neonatal respiratory abnormalities and other clinical manifestations. Pathologically the patients show hypoplasia or agenesis of cerebellar vermis and other intracranial anomalies. Our purpose is to evaluate the clinical manifestations and MR findings of Joubert syndrome. MATERIALS AND METHODS: Among the patients presenting with clinical stigmata of Joubert syndrome and agenesis of vermis on MR imaging, eight patients who did not satisfied the criteria of Dandy-Walker malformation, tectocerebellar dysraphia and rhombencephalosynapsis were selected. MR findings and clinical manifestation were analyzed. RESULTS: On MR imaging, agenesis of the cerebellar vermis (all cases), hypoplasia of the cerebellar peduncle (6cases), fourth ventricular contour deformity(6cases), tentorial elevation (4cases), deformity of the lateral ventricles (4cases), dysgenesis of the straight sinus (3cases) were demonstrated. Other findings were abnormalities of corpus callosum (3cases), falx anomalies (3 cases), occipital encephalomeningocele (2 cases) and fluid collection in posterior cranial fossa (2cases). Clinical manifestations were developmental delay (5cases), abnormal eyeball movement (3cases), hypotonia (2 cases), neonatal rerspiratoy abnormality (2cases), etc. CONCLUSION: Joubert syndrome showed various clinical manifestations and intracranial anomalies. MR imaging is an useful modality in detection of the cerebellar vermian agenesis and other anomalies of the patients.
Christianity ; Congenital Abnormalities ; Corpus Callosum ; Cranial Fossa, Posterior ; Dandy-Walker Syndrome ; Humans ; Lateral Ventricles ; Magnetic Resonance Imaging* ; Muscle Hypotonia

Bottoming out is the term used to describe the inferior displacement of a breast implant after breast augmentation that results in increased distance between the nipple areolar complex and the inframammary fold. Conventional techniques for correcting bottoming out involve capsulectomy and capsulorrhaphy via an inframammary fold incision that is prone to cause large scar and increases the patient's burden. However, using an endoscopic approach via the axilla, we are able to correct bottoming out, resulting in a smaller scar and shorter recovery time. In this article, we present a novel and simple method to correct bottoming out using endoscopy and electrocauterization.
Axilla ; Breast Implants ; Breast* ; Cicatrix ; Endoscopy ; Nipples

No abstract available.
Agranulocytosis* ; Granulocytes* ; Humans

Although neuroendocrine differentiation is a characteristic feature of tumors of the adrenal medulla, cortical tumors may also rarely be differentiated into medullary element. Recently we experienced such a case of adrenal cortical tumor having features of both cortical and medullary tumor. The patient was an 11-year-old girl who was incidentally found to have a left adrenal mass. Laboratory results showed elevated serum cortisol, aldosterone, renin, and epinephrine with high excretion of urinary metanephrine. Urine vanillyl mandelic acid and 17-ketosteroid remained within normal limits. Histologic featuresof a 6 cm round yellowish tumor were ambiguous to decide the orgin of this neoplasm. Cortical element predominated in the tumor with minor areas of pheochromocytomatous feature. Immunohistochemically, the tumor cells were positive for vimentin, neuron specific enolase, and epithelial membrane antigen. Ultrastructural examination revealed scattered membrane bound dense core granules in the tumor cells of medullary differentiation, measuring 150~500 nm in average diameter. Cortical tumor element showed corresponding ultrastructural features. These results indicate that this is a case of adrenal cortical tumor with features of neuroendocrine differentiation.
Child ; Male ; Female ; Humans

Bond strength of luting cements to dentin is a critical consideration for success of complete cast crowns. This study was performed to evaluate the relationship between surface characteristics of teeth prepared for complete cast crowns and retention of cemented restorations. Eighty artificial crowns were cast for standardized complete crown tooth preparations accomplished with the use of a special device on recently extracted human teeth. Coarse diamond(#102R, Shofu) and superfine finishing diamond(#SF102R, Shofu) burs of similar shape were used. Crowns in each group were randomly subdivided into four subgroups of 10 for luting cements selected for this study: zinc phosphate cement (FLECK'S), polycarboxylate cement (Poly-F), reinforced glass ionomer cement (Fuji PLUS), and adhesive resin cement (Panavia 21). Retention was evaluated by measuring the tensile load required to dislodge the artificial crown from tooth preparations with an Instron testing machine, and analysed by one-way ANOVA and Student's t-test. The obtained results were as follows ; 1. When tooth preparation was done with coarse diamond bur, retentive force was diminished in order of Panavia 21, Fuji PLUS, FLECK'S, and Poly-F. Retentive forces showed the significant difference between Fuji PLUS group and FLECK'S group(p<0.001). 2. When tooth preparation was done with superfine diamond bur, retentive force was diminished in order of Fuji PLUS, Panavia 21, FLECK'S and Poly-F. Retentive forces showed the significant difference between Panavia 21 group and FLECK'S group(p<0.001). 3. Retentive force in coarse tooth surfaces was significantly higher than that in superfine tooth surface with all luting cements(p<0.001), and cement residues were almost retained with in the cast crown in all groups.
Adhesives ; Crowns* ; Dental Instruments ; Dentin ; Glass Ionomer Cements ; Humans ; Polycarboxylate Cement ; Resin Cements ; Tooth Preparation ; Tooth* ; Zinc Phosphate Cement

Fibrous hamartoma of infancy (FHI) is an uncommon, benign, solitary intradermal or subcutaneous tumor. It occurs typically in the axillary or shoulder region. The histopathologic examination of affected lesion shows the characteristic elements: dense fibrous tissue, adipose tissue, and primitive mesenchymal tissues. A 15 month-old girl had the multiple, asymptomatic, discrete, and skin-colored tumors that scattered on the scalp. These were present at birth. We report a rare case of FHI occurred on the scalp with multiple and congenital characteristics.
Adipose Tissue ; Female ; Hamartoma* ; Humans ; Parturition ; Scalp ; Shoulder

No abstract available.
Echocardiography* ; Heart Defects, Congenital* ; Prenatal Diagnosis*

Verrucous carcinoma is a slow growing, low grade squamous cell carcinoma, which usually occurs on oropharynx, genitalia and soles. When it occurs on feet, it is known as epithelioma cuniculatum. Epithelioma cuniculatum is characterized by its histologic sinuses and cystic formations in a cuniculate(rabbit burrow-like) pattern. A 59-year-old man was consulted to our department for a well circumscribed, 5 × 5 cm sized, round, hyperkeratotic, verrucous plaque on the right great toe. He suffered from diabetes mellitus for 16 years and was managed by peritoneal dialysis three times a week due to chronic renal failure for last two years. The plaque had gradually increased in size. The biopsy specimen taken from the tumor mass showed chronic ulcerative inflammation with atypical squamous cell proliferation. We herein report a case of verrucous carcinoma occurring in a patient with diabetes melli-t tus and chronic renal failure.
Biopsy ; Carcinoma ; Carcinoma, Squamous Cell ; Carcinoma, Verrucous* ; Cell Proliferation ; Diabetes Mellitus* ; Foot ; Genitalia ; Humans ; Inflammation ; Kidney Failure, Chronic* ; Middle Aged ; Oropharynx ; Peritoneal Dialysis ; Toes ; Ulcer

No abstract available.
Histiocytoma*

Larval nematodes belonging to genera Ancylostoma, Strongyloides, Rhabditis, Gnathostoma or microfilariae (Onchocerca volvulus)/adult filaria (Loa loa) can invade human skin, each making different lesion. In a 71-year old immunocompetent Koreanman with 23% eosinophilia and elevated serum IgG (2530 mg/dl), a skin biopsy was undertaken for a nodular lesion at abdominal wall. A longitudinal section of a larval nematode was recognized in mid-dermal tunnel surrounded by pseudolymphomatous diffuse lymphoreticular cell infiltration. The larval nematode, 15 um in maximum diameter, had outermost cuticular layer and pale intestinal cells. The nematode section was identified as a filariform larva of Strongyloides stercoralis mainly based on its maximum diameter. In Korea, this is the first description of a cutaneous larval nematode infection. This case did not show quick migration of lesion which is a typical manifestation of classical cutaneous larva migrans.
Adult ; Male ; Female ; Humans ; Biopsy