A typical bilateral cleft lip nose shows a short columella, depressed nasal tip, bilateral dislocation and flaring of alar cartilage, wide alar base and almost no nasolabial angle in profile. Among them, the most conspicuous residual stigmata of the repaired bilateral cleft lip nose deformity is apparently a short columella. The optimal columellar lengthening procedure should be selected according to the particular nasolabial deformity and the age of patient. However, whatever methods are used, it is extremely difficult to produce the natural columellar contour. Among the numerous techniques employed for columellar lengthening. Cronin's method and forked flaps have been widely used. The operation for lengthening the columella can be classified into three groups on the basis of source of material:lip,nose, or ear. Cronin's method is an advancement of skin medially and forward from the floor of the nose and alae on two bipedicle flaps, based medially on the columella and laterally on the alae. But, Cronin's method is rarely sufficient in achieving good nasal projection. Forked flap is a method using vertical scar flaps. It is effective in ways, but the method disturbs the well formed arhitectures of the Cupid's bow and philtral width attained at the primary lip repair, and creates new upper lip scar with adverse effect on upper lip tightness. Columellar composite graft from the helical rim has been used quite effectively, but the curvature and consistency of the conventionally used ear cartilage graft are often inadequate. The author peformed and reviewed the results of the above various techniques of columellar lengthening and found that the various techniques had its own unique disadvantages. So, the author developed a new composite graft that the chondrocutaneous composite graft is harvested from the concha rather than the rim and is folded into a file-folder design. This method allows symmetrical and straight columellar formation with firm and stable support. The author performed this chondrocutaneous composite graft of a file-folder design in our series of 19 patients and the postoperative results were satisfactory in a follow-up period of 12months. This study included 46 cases of bilateral cleft lip nose deformity for the past 10 years from February of 1987 to September of 1997. They consist of 3 cases of Cronin's method, 3 cases of primary forked flap, 17 cases of forked flap, 4 cases of bilateral reverse W-plasty and 19 cases of composite graft of file folder design by author's method. The follow-up period was variable from 1 month to 13 years (mean:13 months) and the results of long term follow-up more than 2 years are shown.
Cartilage ; Christianity ; Cicatrix ; Cleft Lip* ; Congenital Abnormalities* ; Dislocations ; Ear ; Ear Cartilage ; Follow-Up Studies ; Humans ; Lip ; Nose* ; Skin ; Transplants

No abstract available.
Axis, Cervical Vertebra* ; Estradiol* ; Testosterone*

No abstract available

No abstract available.
Adrenocorticotropic Hormone* ; Animals ; Plasma* ; Rats*

The nose has number of characteristics that make it unique when planning or reconstruction of surgical defects. The topography of nose is complex with multiple adjacent convex and concave surfaces that must be preserved. The free margins of the alar rims are mobile and easily distorted in case of inadequate planning. The skin over the lower one third of the nose id liss mobile and, therefore, cannot be easily recruited for closure of small defects. In addition, the texture and color of this skin are so unique that neither distant nor nearby skin can always provide a good match. Finally, the function of the nose must always be considered by preserving or replacing the bony and cartilaginous framework, mucosal linig, and never compromising a patent airway. The bilobed flap is particularly suited for reconstruction of small sized or medium sized nasal defects up to 1.5 cm in diameter. By definition, it is a double transposition flap and it is designed to move more skin over a longer distance than would be possible with a single transposition flap in the same location. On the lower one third of the nose where the skin is the least mobile, the bilobed flap allows the surgical site to be covered with nearby skin matched for color and texture and then allows for repair of that secondary defect with another well-matched flap whose donor site can finally be closed primarily. This flap also results in little or no distortion of the nose since the flap efficiently recruits skin from distant and more lax sites. While the standard design often results in tissue protrusions or pincushioning effect, improvements in the design are outlined herein to achieve the best results for defects of the nose.
Humans ; Nose ; Skin ; Tissue Donors

Congenital hypohidrotic ectodermal dysplasia is a rare genodermatosis. It is characterized by hypohidrosis hypotrichosis, dental hypoplasia and characterstic facial features, which reflect a wide constellation of developmental defec of tissue from the ectoderm. We have encountered three cases of congenital hypohidrotic ectodermal dysplasia in a 28-year-old female, her new-born baby, and a 10-month-old boy with a family history. All of the three patients had hypohidrosis, hypotrichosis, defective dentition, and characterstic facial features, which were characterstic features of this disorder. In addition, they showed dry skin, sparse and thin hairs. Histopathologic findings of previous cases revealed no eccrine gland structure in the dermis with routine and immunohistochemical stainning such cytokeratin and filaggrin. We report three typical cases of hypohidrotic ectodermal dysplasia with the review of literature.
Adult ; Dentition ; Dermis ; Eccrine Glands ; Ectoderm ; Ectodermal Dysplasia 1, Anhidrotic* ; Female ; Hair ; Humans ; Hypohidrosis ; Hypotrichosis ; Infant ; Keratins ; Male ; Skin

It has been widely accepted that complete surgical resection of hepatoblastoma is essential for long-term survival. But unfortunately less that 50% of hepatic tumors in children can be totally removed at the time of diagnosis. This report is to present the experience of successful resection of hepatoblastoma after concurrent radiotherapy with transarterial chemoinfusion in a child. We believe this modality of treatment enables complete resection of unresectable hepatoblastoma, which is resistant to the systemic chemotherapy.
Child ; Diagnosis ; Drug Therapy ; Hepatoblastoma* ; Humans ; Radiotherapy*

A collodion baby is born with a tough, inelastic parchment-like membrane covering the whole body surface. As the meinbrane fissures and peels, a more characteristic ichthyosiform change is evident beneath the collodion membrane. Uncommonly, normal skin is found under it. We herein present two cases af collodion baby, which were confirmed as a sporadic case of lamellar ichthyosis and a case of lamellar exfoliation of the newborn, respectively, after long-term clinical observation. It is necessary that detailed genetical and molecular biological studies should be perforrned in order to elucidate the fur:damental, molecular changes that cause these dramatic cutaneous changes.
Collodion* ; Humans ; Ichthyosis, Lamellar ; Infant, Newborn ; Membranes ; Skin

Some sexual differentiation disorders are associated with gonadal neoplasia and increased incidence of testicular tumors has been discribed in the patients with, XY gonadal dysgenesis. The incidence of testicular tumors in infants and children are rare, representing only 1% of all pediatric solid tumors. In general, gonadal stromal tumors are one of the most characteristic endocrine tumors of the testis, endocrine activity occurs in at least 10-20%, among them Leydig cell tumors and Sertoli cell tumors are clinically important. Although the exact pathogenesis is unknown, endocrine activity due to estrogen secretion can be manifested clinically with gynecomastia or precocious puberty. We experienced and reported a child who visited for sexual precocity and had XY gonadal dysgenesis with Sertoli cell tumor.
Child ; Disorders of Sex Development ; Estrogens ; Gonadal Dysgenesis ; Gonads ; Gynecomastia ; Humans ; Incidence ; Infant ; Leydig Cell Tumor ; Male ; Puberty, Precocious ; Sertoli Cell Tumor* ; Testicular Neoplasms ; Testis

No abstract available.
Cell Line* ; Gonadotropin-Releasing Hormone* ; Medroxyprogesterone Acetate* ; Medroxyprogesterone* ; Tamoxifen*