Ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome presenting with a large nephrogenic cyst, severe oligohydramnios and hydrops fetalis: a case report and review of the literature

OBJECTIVES: To report a case of EEC syndrome with a large nephrogenic cyst detected by prenatal ultrasonography. METHODS: Prenatal ultrasonographic detection, genetic counselling, termination of pregnancy, radiographic study, autopsy and ultrastructural study of scalp hair. The literature on EEC syndrome with genitourinary anomalies and prenatal diagnosis was also reviewed. RESULTS: A 6-cm cyst in the right side of abdominal cavity was detected in a fetus of 24 weeks' gestational age with severe oligohydramnios and hydrops fetalis. The autopsy revealed bilateral renal dysplasia with a large nephrogenic cyst at the right side and markedly hypoplastic urinary bladder and pulmonary hypoplasia. The fetus also had ectrodactyly and syndactyly of hands and feet and ectodermal dysplasia and left cleft lip and palate. Ultrastructure of hair from scanning electron microscopy revealed no obvious abnormality. CONCLUSION: This is a rare case of fetal malformation partly detected prenatally. In order to detect this syndrome, careful ultrasound search for acral anomalies should be performed in cases with renal malformation or obstructive uropathy.     

OCT-GAN: single step shadow and noise removal from optical coherence tomography images of the human optic nerve head

Speckle noise and retinal shadows within OCT B-scans occlude important edges, fine textures and deep tissues, preventing accurate and robust diagnosis by algorithms and clinicians. We developed a single process that successfully removed both noise and retinal shadows from unseen single-frame B-scans within 10.4ms. Mean average gradient magnitude (AGM) for the proposed algorithm was 57.2% higher than current state-of-the-art, while mean peak signal to noise ratio (PSNR), contrast to noise ratio (CNR), and structural similarity index metric (SSIM) increased by 11.1%, 154% and 187% respectively compared to single-frame B-scans. Mean intralayer contrast (ILC) improvement for the retinal nerve fiber layer (RNFL), photoreceptor layer (PR) and retinal pigment epithelium (RPE) layers decreased from 0.362 ± 0.133 to 0.142 ± 0.102, 0.449 ± 0.116 to 0.0904 ± 0.0769, 0.381 ± 0.100 to 0.0590 ± 0.0451 respectively. The proposed algorithm reduces the necessity for long image acquisition times, minimizes expensive hardware requirements and reduces motion artifacts in OCT images.     

Augmentation rhinoplasty with custom-made S-shape silicone implant in Asians: A 15-year experience

Background:

Asians have low nasal dorsum, thick skin envelope, low defined alar cartilage, low projection of nasal tip and broad alar base. Augmentation rhinoplasty with silicone prosthesis has been performed with predictable results, but unfavourable results and complications still present. This series show techniques and results from single surgeon experience.

Materials and Methods:

We retrospectively reviewed 548 patients chart during January 1995 to December 2009. All patients underwent custom-made S-shape implant silicone augmentation rhinoplasty operated by a single surgeon. There were three major operative steps: (1) Intra-operative S-shape implant carving; (2) pocket dissection through bilateral rim incision and (3) tension adjustment before closure. All the patients were recorded for early surgical complications and satisfaction.

Results:

There were 519 women and 29 men. The mean age is 25.5 years (18-56 years). Mean follow-up period was 6 months (1-60 months). The majority of patient were appointed for esthetic augmentation (86.8%). 515 cases (94.9%) showed well satisfaction following the operation. The total complication rate was 6.5% (4.9% deviation, 0.7% extrusion, 0.5% hematoma and 0.3% infection). All the complications were corrected with uneventful sequelae.

Conclusion:

Augmentation rhinoplasty with custom-made S-shape silicone implant by closed approach provides high satisfaction with acceptable early complication rate.KEY WORDS: Aesthetic surgery, Asians, augmentation rhinoplasty, silicone implant, surgical complication     

Prospective study of Q-switched Nd:YAG laser treatment of hyperpigmented split-thickness skin grafts

Lasers in Medical Science - Hyperpigmentation of split-thickness skin grafts (STSGs) is commonly found among Asians, and it is also challenging to treat. Although the 1064-nm Q-switched Nd:YAG...     

Placental abnormalities associated with human immunodeficiency virus type 1 infection and perinatal transmission in Bangkok, Thailand

The effects of human immunodeficiency virus (HIV) type 1 on the placenta and the role of the placenta in mother-to-child HIV-1 transmission are not well understood. Placentas from 78 HIV-infected and 158 HIV-uninfected women were examined as part of a prospective perinatal HIV transmission study in Bangkok. HIV-infected women were more likely than HIV-uninfected women to have chorioamnionitis (odds ratio [OR], 2.1; P=.03), placental membrane inflammation (PMI; OR, 2. 7; P=.02), and deciduitis (OR, 2.3; P=.03) and less likely to have villitis (OR, 0.3; P=.02). However, among HIV-infected women, fewer women who transmitted infection to their child had chorioamnionitis (relative risk [RR], 0.2; P=.03), funisitis (RR, 0.4; P=.1), or PMI (RR undefined; P=.03). These findings suggest that, in this population, HIV-infected women are at increased risk for placental membrane inflammatory lesions, but that placental inflammatory lesions are not associated with increased perinatal HIV transmission.     

Surgical pathology and etiology of 278 surgically removed mitral valves with pure regurgitation in Thailand.

INTRODUCTION: There are multiple causes of mitral regurgitation. Its etiology includes floppy valve, postinflammatory disease, infective endocarditis, and other disorders. Recently, there has been an increased tendency to remove only portions of the mitral valve, causing difficulty in the determination of etiology. Our objective was to study the pathology and etiology of mitral regurgitation from surgically removed specimens. METHODS: Native mitral valve specimens surgically excised due to mitral insufficiency were examined. Etiology was determined according to macroscopic, microscopic, clinical, and operative findings. RESULTS: Among 278 mitral valve specimens, 43% were classified as floppy valve, 31% as postinflammatory disease (presumably associated with rheumatic fever), 12% as infective endocarditis, and 14% as miscellaneous group. In floppy valves, diffuse myxoid change and chordal rupture were the main findings. In postinflammatory disease, moderate neovascularization and chronic inflammatory cell infiltration were most commonly found. Aschoff bodies were found in two cases. In infective endocarditis, gram-positive cocci were found in 70% of cases. In the miscellaneous group, three cases were related to Marfan syndrome and one case was related to papillary muscle necrosis. In comparison with postinflammatory disease, the posterior leaflet in the floppy valve had a significantly longer basal free-edge length, a more frequent chordal rupture, and an higher mean age of patients. Among completely and partially excised specimens with postinflammatory disease, there were no significant differences in microscopic findings. CONCLUSION: The three most common etiologies in mitral regurgitation were floppy valve, postinflammatory disease, and infective endocarditis. Macroscopic, microscopic, clinical, and operative findings are important in the evaluation of etiology, especially in partially excised specimens.