Laparoscopic finding of a hepatic subcapsular spider-like telangiectasis sign in biliary atresia

AIM To assess the diagnostic value of a laparoscopic finding of a hepatic subcapsular spider-like telangiectasis (HSST) sign in biliary atresia. METHODS A retrospective study was conducted first and then a validation set was used to investigate the value of an HSST sign in predicting biliary atresia (BA). In the retrospective study, laparoscopic images of the liver surface were reviewed in 126 patients with infantile cholestasis (72 BA patients and 54 non-BA cholestasis patients) and a control group of 38 patients with nonhepatic conditions. Analysis was first made by two observers separately and finally, a consensus conclusion was achieved. Then, the diagnostic value of the HSST sign was validated in an independent cohort including 45 BA and 45 non-BA patients. RESULTS In the retrospective investigation, an ampli.ed HSST sign was found in all BA patients, while we were unable to detect the HSST sign in 98.1% of the 54 non-BA patients. There was no HSST sign in any of the control subjects. In the first review, the sensitivity and specificity from one reviewer were 100% and 98.1%, respectively, and the results from the other reviewer were both 100%. The consensus sensitivity and specificity were 100% and 98.1%, respectively. The HSST sign was defined as being composed of several enlarged tortuous spider-like vascular plexuses with two to eight branches distributed on all over the liver surface, which presented as either a concentrated type or a dispersed type. In the independent validation group, the sensitivity, specificity, positive predictive value and negative predictive value of the HSST sign were 100%, 97.8%, 97.8% and 100%, respectively. CONCLUSION The HSST sign is characteristic in BA, and laparoscopic exploration for the HSST sign is valuable in the diagnosis of BA.

Three-year follow-up of Coats disease treated with conbercept and 532-nm laser photocoagulation

BACKGROUND Coats disease is an idiopathic exudative outer retinopathy caused by abnormalretinal vascular development.AIM To evaluate the long-term outcomes of intravitreal conbercept injection with laserphotocoagulation as a treatment for Coats disease in adults.METHODS This retrospective case series study included patients diagnosed with Coatsdisease and treated with intravitreal conbercept injection and 532-nm laserphotocoagulation at the Ophthalmology Department of Shenzhen People’sHospital between January 2016 and January 2017. Best-corrected visual acuity(BCVA) measurements, noncontact tonometry, ophthalmoscopy, fundusphotography, fundus fluorescein angiography and optical coherence tomographywere performed before treatment and at 1 wk, 1 mo, 3 mo, 6 mo, 9 mo, 12 mo, 24mo and 36 mo after therapy. Best-corrected visual acuity was measured using theearly treatment of diabetic retinopathy study chart.RESULTS The study included eight eyes of 8 patients (7 men) aged 36.10 ± 6.65 years. Theaverage BCVA of the affected eye before treatment was 51.17 ± 15.15 letters(range, 28–70 letters), and the average central macular thickness was 303.30 ±107.87 μm (range, 221–673 μm). Four eyes were injected once, three were injectedtwice, and one was injected three times. Average follow-up duration was 37.33 ±2.26 mo. Average BCVA of the affected eye was 51.17 ± 15.15 letters before treatment and was increased by 13.50 ± 3.20, 16.25 ± 7.73, 18.25 ± 8.96, 18.03 ± 5.27,18.63 ± 3.35, 19.75 ± 6.96, 18.05 ± 5.36 and 17.88 ± 3.45 letters at 1 wk, 1 mo, 3 mo, 6mo, 9 mo, 12 mo, 24 mo and 36 mo after treatment, respectively (P < 0.01). Thepatients showed varying degrees of subretinal fluid resorption after treatment.None of the patients had serious complications such as increased intraocularpressure, development/progression of cataracts, endophthalmitis or retinaldetachment.CONCLUSION Intravitreal injection of conbercept combined with 532-nm laser photocoagulationmay be a feasible treatment for Coats disease in adult patients.

Diagnosis of Fucosidosis through Cutaneous Manifestations

Angiokeratoma corporis diffusum is a dermatological hallmark of several inherited lysosomal storage disorders including Anderson-Fabry disease and other enzyme deficiencies such as fucosidosis. We report a 4-year-old boy with neurodevelopment delay who was diagnosed as having fucosidosis following recognition of dermatological signs, angiokeratoma and telangiectasies. The diagnosis was confirmed by leukocyte oligosaccharide enzyme analysis.

Clinical efficacy of 595-nm pulsed-dye laser in treatment of childhood facial spider nevi: a retrospective study of 110 patients

Background:Spider nevi (SN) are quite common in children. SN are treated via different techniques, and complete removal often requires multiple treatments. However, few studies have evaluated the treatment of SN. The present study aimed to evaluate the therapeutic effect and safety of a 595-nm pulsed-dye laser (PDL) for treating facial SN in children.Methods:A total of 110 children aged 0.2 to 12 years with facial SN were treated with a 595-nm PDL in a single institution from January 2016 to February 2018. In accordance with the treatment method, the patients were retrospectively divided into the small-spot-combined-with-large-spot group (SL-group) and the large-spot group (L-group). Patients with poor therapeutic results were retreated every 6 weeks until the lesions disappeared. The minimum follow-up period was 1 year. The groups were compared using independent-samples t tests, Mann-Whitney U test, Chi-square test, and Fisher exact probability test.Results:The therapeutic efficacy was significantly higher in the SL-group than in the L-group, with clearance rates of 90.9% and 53.0% after the primary treatment, respectively (χ2= 17.937, P < 0.001). For skin lesions with a central spider body diameter ≥1 mm, the once-treatment cure rates were 100% in the SL-group and 34.8% in the L-group (χ2 = 20.780, P < 0.001). For skin lesions with a central spider body diameter <1 mm, the once-treatment cure rates were 82.6% in the SL-group and 62.8% in the L-group (χ2 = 3.961, P = 0.138). The rates of adverse reactions and recurrence did not differ between the two groups (P = 0.141 and P = 1.000, respectively).Conclusions:The 595-nm PDL might be a safe and effective treatment option for facial SN in children. The small-spot-combined-with-large-spot method is especially suitable for SN with a central spider body diameter ≥1 mm.

氩离子凝固术治疗飞行员胃毛细血管扩张症一例

一、临床资料 患者,男性,31岁,初教-6飞行员,飞行时间1300 h.2010年3月15日因间歇性上腹部隐痛5年,加重并黑便20 d入院.患者自2005年反复出现不规律性上腹部不适、隐痛和反酸,服用抗酸剂后症状可缓解.曾于2007年、2009年先后两次因症状较重住院治疗;上消化道钡餐透视和胃镜检查均示:浅表性胃炎,予以抑制胃酸、保护胃黏膜等药物治疗,症状消失后出院.既往无肝炎及鼻衄等疾病史.查体:脉搏92次/min,血压110/70 mm Hg(1 mm Hg=0.133 kPa),上腹部轻度压痛,余无阳性体征.检查:RBC 4.1×1012/L,Hb 112 g/L;肝功能正常,血凝分析正常;大便隐血(++++).胃镜检查示:胃底黏膜皱襞粗大,表面可见网状红斑,范围3 cm×2 cm大小,并见新鲜血液渗出.