脉冲染料激光治疗婴幼儿体表溃烂型血管瘤疗效研究

目的:观察波长为585 nm脉冲染料激光治疗婴幼儿体表溃烂型血管瘤的临床疗效。方法:采用波长为585 nm的脉冲染料激光,对76例(男26例,女50例)体表溃烂型血管瘤(平均瘤体表面积22.1 cm2)患者进行照射治疗,激光能量为5.8 J/cm2~8 J/cm2(平均6.6 J/cm2),间隔4周治疗一次,治疗二次后瘤体无消退者予口服类固醇辅助治疗,类固醇辅助治疗4周无效者再联合干扰素治疗,直至创面愈合和瘤体消失。结果:68例患儿在治疗二次后创面完全愈合,瘤体明显缩小,经一至八次(平均四次)激光治疗后瘤体完全消退,8例患儿辅以类固醇治疗,6例治愈,2例无效后予干扰素联合治疗后瘤体消退,总治愈率100%;经过平均12个月随访,所有患儿无溃疡复发和血管瘤再发。结论:脉冲染料激光治疗婴幼儿体表溃烂型血管瘤,具有良好疗效,是一种行之有效的治疗方法。     

Blow-in fractures of the orbit

A blow-in fracture is an inwardly displaced fracture of the orbital rim or wall resulting in decreased orbital volume. The purpose of this study is to classify orbital blow-in fractures, describe the distinguishing clinical and radiologic features, and review the result of treatment. The series consists of 41 patients with blow-in fractures (34 males and 7 females). The mean age of the patients was 36 years. All were treated between 1979 and December of 1986 at Sunnybrook Medical Centre in Toronto. Clinical features of blow-in fractures were primarily related to the decrease in volume of the orbital cavity. Proptosis was a consistent finding, and in 27 percent of patients, the globe was further displaced in a coronal plane. Restricted ocular motility and diplopia were documented in 24 and 32 percent of patients, respectively. Fracture fragments displaced into the orbit resulted in globe rupture in 12 percent of patients, superior orbital fissure syndrome in 10 percent, and optic nerve injury in 1 patient. Blow-in orbital injuries were classified as pure fractures, consisting of an isolated blow-in of a segment of the roof, floor, or walls, or impure fractures, where the orbital rim itself was disrupted. In all cases, early decompression of the orbit and open reduction of fractures was necessary. Late sequelae of blow-in fractures were primarily related to injuries of intraorbital contents. Twelve percent of patients underwent enucleation and 8 percent reported persistent diplopia. Despite the presence of superior orbital fissure syndrome and complete ophthalmoplegia in 10 percent of patients, early orbital decompression resulted in resolution of nerve palsies in all but one patient.     

Surgical reconstruction of the contracted orbit

Anophthalmic patients and patients afflicted with retinoblastoma incur severe deformity of the orbit. Treatment of the severely contracted orbit is very difficult, and patient satisfaction is often poor. Since 1988, we have performed temporalis muscle transfer and surgical expansion of the contracted bony orbit in 26 patients. Satisfactory results were obtained. Gradual expansion of the orbit was performed in case of congenital anophthalmic patients. The treatment should be established in multiplicity, among many methods available for contracted eye sockets, according to the degree of orbital deformity and the amount of residual conjunctiva. In case of severe deformity, volume expansion surgery and temporalis muscle transfer are necessary. If augmentation is required in the periorbital region, rib bone onlay graft must be performed. We were able to shorten the operative time by modifying the three-wall orbital expansion technique of Tessier and Wolfe to a more simplified method. Our observations show that our procedures achieved symmetry in both eyes in all patients, and there have been no remarkable complications.     

Intraosseous vascular malformations of the orbit

Intraosseous vascular malformations are rare benign tumors involving the bones of the orbit. The diagnosis should be considered when a patient presents with an enlarging mass fixed to bone in the upper face, and the characteristic x-ray appearance should be looked for on plain films. Treatment is local excision of the bone containing the tumor and immediate reconstruction with autogenous bone.     

Rosai dorfman disease of the orbit

Objectives

We have analyzed t(12;21)(p13:q22) in an attempt to evaluate the frequency and prognostic significance of TEL-AML1 fusion gene in patients with childhood CD 10 positive B-ALL by fluorescence in situ hybridization (FISH). Also, we have monitored the prognostic value of this gene as a minimal residual disease (MRD).

Methods

All bone marrow samples of eighty patients diagnosed as CD 10 positive B-ALL in South Egypt Cancer Institute were evaluated by fluorescence in situ hybridization (FISH) for t(12;21) in newly diagnosed cases and after morphological complete remission as a minimal residual disease (MRD). We determined the prognostic significance of TEL-AML1 fusion represented by disease course and survival.

Results

TEL-AML1 fusion gene was positive in (37.5%) in newly diagnosed patients. There was a significant correlation between TEL-AML1 fusion gene both at diagnosis (r = 0.5, P = 0.003) and as a MRD (r = 0.4, P = 0.01) with favorable course. Kaplan-Meier curve for the presence of TEL-AML1 fusion at the diagnosis was associated with a better probability of overall survival (OS); mean survival time was 47 ± 1 month, in contrast to 28 ± 5 month in its absence (P = 0.006). Also, the persistence at TEL-AML1 fusion as a MRD was not significantly associated with a better probability of OS; the mean survival time was 42 ± 2 months in the presence of MRD and it was 40 ± 1 months in its absence. So, persistence of TEL-AML1 fusion as a MRD had no additive prognostic value over its measurement at diagnosis in terms of predicting the probability of OS.

Conclusion

For most patients, the presence of TEL-AML1 fusion gene at diagnosis suggests a favorable prognosis. The present study suggests that persistence of TEL-AML1 fusion as MRD has no additive prognostic value.     

Renal cell carcinoma metastatic to the orbit

Metastatic lesions to the orbit, although rare, appear to be increasing in frequency. In a significant number of these patients, ocular signs and symptoms are the first indicators of their disease. Optometrists need to be aware of the clinical presentation, differential diagnosis, and indicated medical work-up in order to properly diagnose and manage this challenging group of patients.     

Infection of the human orbit by Aspergillus stromatoides

We report a case of human infection due to Aspergillus stromatoides. To our knowledge this organism has not previously been described as causing human infections. The infection of sino-orbital tissues was of 3 1/2 years duration and the patient died following cerebral involvement despite seven months of hospitalisation and therapy including amphotericin B, 5-fluorocytosine and rifampicin.     

Trapdoor fracture of the orbit in a pediatric population

Orbital-floor blowout fractures of the trapdoor variety, first described by Soll and Poley, have also appeared in the French and Japanese literature. The trapdoor fracture is described as a pure orbital-floor fracture, linear in form and hinged medially, which allows herniation of orbital contents through the fracture and then entraps these herniated contents. A review of the orbital-floor fracture literature reveals a high incidence of persistent diplopia associated with ocular-muscle entrapment, which may later necessitate corrective surgery of extraocular muscles. Recent publications in the ophthalmologic literature have stressed the importance of early surgical intervention. This article reports a retrospective series of 19 pediatric patients (age range, 5 to 16 years) who presented to two institutions. All patients had radiographic confirmation of a trapdoor fracture. Physical examination demonstrated a high association between these fractures and restricted ocular motility (17 of the 19 patients). In the cases with trapdoor fracture and restricted ocular movement, early intervention was associated with better postoperative function. It is thus recommended that the symptomatic trapdoor orbit fracture be considered an urgent indication for surgical intervention. Practitioners therefore must have a high index of suspicion for these injuries. Prompt diagnosis is critical to maximize clinical outcome.