经未显影的岩下窦途径栓塞海绵窦区硬脑膜动静脉瘘

目的探讨经未显影的岩下窦途径栓塞海绵窦区硬脑膜动静脉瘘(CSDAF)的治疗方法。方法回顾性分析7例CSDAF病人的临床资料,均经未显影的岩下窦插管,以微弹簧圈栓塞海绵窦和瘘口。结果5例插管成功并达到完全致密栓塞;2例导管虽插入海绵窦,但海绵窦分隔致导管位置与瘘口不相通,而改行面静脉-眼静脉途径插管行栓塞治疗。7例病人均治愈,随访5～20个月,均无复发。结论经岩下窦途径栓塞是CSDAF最常用的治疗方法,即使岩下窦未显影,也有相当部分病人可插管和治疗成功。     

The dysmorphic lung: imaging findings

Congenital lung malformations are not infrequent and can be discovered in adults. It is, therefore, necessary to know their radiological manifestations in order to avoid diagnostic errors. We classify the congenital lung malformations in two main groups: dysmorphic lung and focal pulmonary malformations. We review the radiological spectrum of dysmorphic lung, based on a classification that emphasises the pulmonary abnormality, adding variants when diaphragmatic or venous abnormalities are present. In our opinion this approach allows for a rational use of advanced imaging techniques (CT, MRI). Correspondence to: Josep M. Mata.     

硬脑膜动脉海绵窦瘘的眼眶CT表现,DSA检查及栓塞治疗

笔者分析了５例硬脑膜动脉海绵窦瘘伴突眼，结膜充血的病例，全部经ＣＴ及ＤＳＡ检查，同时进行了栓塞治疗，经随访７－９个月未见复发，主要ＣＴ表现为：（１）眼球突出，结膜充血；（２）眼上静脉扩张；（３）海绵窦增大；（４）眼外肌增厚：（５）眶尖软组织肿胀。并讨论了诊断鉴别诊断，ＤＳＡ检查及有关治疗问题。     

Catheter embolization of pulmonary arteriovenous fistulas in an infant

Summary An infant with pulmonary arteriovenous fistulas is described. Surgical ligation of the fistulas and limited resection of paraenchyma from the right lung was followed at 11 months of age, by successful steel coil embolization of residual fistulas.     

Experimental model of chronic tonsillar herniation associated with early stage syringomyelia

This report describes an experimental model of chronic tonsillar herniation and its effects on the spinal cord. In ten rats, a small piece of chemically induced mammary cancer was transplanted to the supraoccipital bone. In all cases, the transplanted cancers grew into the posterior fossa, destroying the supraoccipital bone and compressing the cerebellum extradurally. In six of the ten rats, tonsillar herniation was observed at 8–14 weeks after transplantation. Transdural infiltration of the tumor cells was not apparent in any animal. In those rats with tonsillar herniation (n=6), the spinal cord from the C5 to the T8 segments showed enlargement of the central canal without exception. Histological examination revealed the following changes: stretching and thinning of the ependymal cells; swelling of the astrocytic processes; and extracellular edema, predominantly in the dorsal gray matter, but also in the ventral inner portion of the dorsal column. In the control group (n=4) and those rats without tonsillar herniation (n=4), such histological changes of the spinal cord were not observed. Although the lesions can not be regarded as representing mature syringomyelia, they most likely constitute an earlier evolutionary stage.     

Continuous arteriovenous haemofiltration in the treatment of tumour lysis syndrome

The management of tumour lysis syndrome remains problematic despite the rigorous use of preventative measures. Continuous arteriovenous haemofiltration (CAVH) is well suited to its use in both the prevention and treatment of metabolic abnormalities and renal insufficiency associated with tumour lysis. We report the successful use of CAVH in the treatment of a patient with tumour lysis syndrome.     

ONE-STAGE ARTERIOVENOUS REVERSAL FOR REVASCULARIZATION OF SEVERELY ISCHEMIC LIMBS

Staged arteriovenous reversal has been successfully established forrevascularization of severely ischemic limbs since 1984. The authors tried to constrict thecentral venous limb immediately after the arteriovenous fistula formation to make the pres-sure distal to the anastomosis raising to its maximum to shorten the time required for thedistal valves to become incompetent. Thus, the revascularization of the severely ischemiclimbs may be established much sooner than the staged arteriovenous reversal, and more di-seased limbs will be saved. Forty-two patients with a total of 60 upper and lower limbshave been operated on with good results.     

Tentorial sinus

Summary The authors report a case of tentorial sinus draining the telencephalic and diencephalic tributaries of the basal vein. Dorsally the sinus joined the straight sinus and later the confluence of the sinuses. Attention should be paid to this normal variation whenever a transtentorial surgical approach is considered.

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Sinus de la tenteAspect radiologique et anatomique, à propos d'un cas

Résumé Les auteurs rapportent un cas de sinus de la tente du cervelet drainant les affluents télencéphaliques et diencéphaliques de la veine basale. En arrière, le sinus rejoint le sinus droit puis le torcular. Une attention particulière pré-opératoire devrait être accordée à la mise en évidence de cette variété lorsqu'une approche transtentorielle est discutée.

     

Trisomy 17p due to a t(8; 17) (p23; p11.2)pat translocation. Case report and review of the literature

We describe a female newborn girl with partial trisomy 17p, which was not detected at the initial cytogenetic investigation, but which later proved to be an unbalanced product of a paternal translocation t(8; 17)(p23;pl 1.2). Comparison with the three previously reported patients suggests a clinically distinct "trisomy 17p syndrome", i.e. pre- and postnatal growth retardation, microcephaly, antimongoloid slanting of palpebral fissures, hypertelorism, long philtrum with thin upper lip, micrognathia and high-arched palate. Two of the four patients had a heart defect, and psychomotor developmental delay was evident in all four cases. In the present patient, the chromosomal anomaly was only detected after the finding of the autosomal reciprocal translocation in the father. The importance of cytogenetic investigations in parents of a MCA/MR child with apparently normal chromosomes is emphasized.     

Interstitial deletion of band q12 of chromosome 5

A six-months-old girl is presented with psychomotor retardation and multiple congenital malformations. The karyotype done on peripheral blood lymphocytes and skin fibroblasts was found to be 46,XX del(5)(411q13). The parents are consanguineous. Their karyotypes were normal.
The genes for Arylsulphatase B and Hexosaminidase B are not located in band 5q12.