Objective To observe the cl inical outcome of treating blepharophimosis-ptosis-epicanthus inversus syndrom (BPES) by means of primary and complex recection of levator palpebrae superioris musculus. Methods From May 2001 to May 2007, 12 patients with BPES were treated, including 6 males and 6 females aged 4-15 years old (average 7 years old).All patiens marked signs of BPES— typical ptosis of the upper eyel ids, epicanthus inversus, palpebral fissure, and increased distance between inner canthus.The eye fissure width was (2.8 ± 1.8) mm, the eye fissure length was (19.8 ± 4.7) mm, and the inner canthic diameter was (41.6 ± 6.5) mm.The muscular strength of levator palpebrae superioris was deficient in 4 cases, the muscular strength of levator palpebrae superioris was (2.0 ± 0.6) mm in 8 cases.All patients were associated with visual function congenital defects of varying degrees.The surgical technique included shortening of the internal canthal l igaments, recection of the tarsus and levator muscle, and skin plasty. Results All the incisions healed by first intension.Twelve patients were followed up for 12-48 months (average 30 months).Amel ioration of ptosis and epicanthus was achieved.At 18 months after operation, the eye fissure width of 10 petients was (9.0 ± 2.1) mm, the eye fissure length was (26.5 ± 3.5) mm, and inner canthic diameter was (30.2 ± 2.7) mm, the muscular strength of levator palpebrae superioris increased to (5.6 ± 1.9) mm, showing significant difference when compared with preoperation (P lt; 0.05). Conclusion The primary and complex recection of levator palpebrae superioris musculus can provide relating good cosmetic and functional results for the correction of BPES. Patients with BPES should receive surgery as early as possible.
Objective To investigate the anatomical features of congenital chordee without hypospadias in children and to discuss the diagnosis and treatment.Methods From August 1984 to December 2004, 94 children with chordee withouthypospadias treated in the West China Hospital of Sichuan University were classified and analyzed for anatomical alterations. Their ages ranged from 18 months to 13 years (mean 6.9 years). Ninety-four patients were divided into four groups. With intraoperation artificial erection, the patients with penis straightened after degloving were classified as type Ⅰ patients (skin-tethering), those with peins straightened after fibrotic tissue in Buck’s fascia released as type Ⅱ patients (dysgenetic fascia), those with normal urethra and orthoplasty failed after degloving and removing fibrotic tissue as type Ⅲ patients (corporal disproportion), and those with dysgenetic urethra tethering the corpora cavernosa as type Ⅳ patients (short urethra).Results In type Ⅰ (n=31, 32.9%) patients, the ventral skin and dartos fascia were contracted while Buck’s fascia and the urethra was normal, in some (7 cases) scrotal skin extended to the ventral portion of penis (webbed penis). In type Ⅱ (n=45, 47.9%), contracture of Buck’s fascia was evident and the thickening fibrotic tissue constituted the chief obstacle to orthoplasty, though in some skin was shortened. In type Ⅲ (n=6), the dorsal and ventral sides of the corpora cavernosa were disproportionated, and the morphologically normal urethra tightly adhered to the ventral aspect of corpora cavernosa. In some cases ventral skin and fascia were contracted, but orthoplasty could notbe achieved through releasing these layers. In type Ⅳ (n=12, 12.8%), the distal urethra was paper-thin and lacking corpus spongiosum, or dense fibrotic bandswere found to be deep to the urethra. The urethra tethered the corpora cavernosaand formed a bow-to-string relation. The overlying skin and fascia were contracted in varying degrees while none had significance in straightening the penis. After operation, the length of penis increased to 6.9 cm from 5.2 cm on average and the chordee was corrected to 1.6° from 42.6° before operation on average. The patients were followed up 1 months to 15 years. The results were satisfactory.Chordee remained in 2 cases, fistula and urethral stricture occurred in 2 casesrespectively, fistula in association with urethral stricture and diverticulum in 1 case; the operation was given again and the results was satisfactory. Conclusion Patients with chordee without hypospadias may be divided into four types depending on which layer of the ventral penis constitutes thechief contribution to chordee. A systematic approach with repeated artificial erection tests is needed in determining the classification and surgical correction.