婴儿主动脉缩窄合并心内畸形的一期矫治

目的总结婴儿主动脉缩窄（CoA）合并心内畸形一期矫治的手术方法和临床经验。方法2001年1月至2006年1月，对28例CoA合并心内畸形患者行一期手术矫治。CoA为导管前型18例，邻近或正对导管处10例；伴主动脉弓发育不良6例，动脉导管未闭22例。合并的心内畸形包括：室间隔缺损16例、室间隔缺损＋房间隔缺损5例、完全性房室间隔缺损3例，完全型大动脉错位伴室间隔缺损（D—TGA／VSD）2例，右心室双出口2例。采用左胸后外侧联合胸骨正中切口（双切口）径路手术12例，胸骨正中切口径路（单一切口）手术16例。采用缩窄段切除端端吻合术20例，Gore—Tex补片扩大成形术4例，左锁骨下动脉翻转扩大成形术4例。结果全组无手术死亡，术后呼吸机辅助呼吸时间7h～13d，住ICU时间3～18d。因术后心脏扩大延迟48～72h关胸4例，术后发生急性肾功能衰竭行腹膜透析3例，吸入一氧化氮（NO）治疗重度肺动脉高压3例。术后下肢收缩压高于上肢10～20mmHg18例（64．3％），收缩压上、下肢相差不大8例（28．6％），上肢分别高于下肢15mmHg和20mmHg2例（7．1％）。所有患者均得到随访，随访时间3～50个月，1例D—TGA／VSD患者因重度三尖瓣反流、肺部严重感染和呼吸功能衰竭于术后3个月死亡；其余27例患者恢复顺利。心脏超声心动图提示：1例采用端端吻合法、1例采用Gore～Tex补片扩大成形术的患者在CoA纠治处仍存在压差，分别为25mmHg、28mmHg，均未再次手术。结论一期手术矫治CoA合并心内畸形可以缩短疗程，有利于患者术后心、肺功能的恢复，避免二次手术的痛苦，降低治疗费用。可采用单一胸骨正中切口或胸骨正中联合左胸后外侧切口完成手术。     

350例脊髓血管畸形介入治疗的护理分析

目的探讨护理工作在脊髓血管畸形介入治疗中的价值。方法通过对我院介入中心350例脊髓血管畸形患者介入治疗,针对脊髓血管畸形患者的临床特点和介入治疗方法的适应证、操作过程和预后等特点,从护理学的角度对术前、术中、术后等阶段进行讨论分析。结果术前进行有效的心理护理和健康教育;术中与介入医生及技术操作人员进行有机的配合、熟练使用输液微量泵、准确使用相关药物及认真观察病情变化;术后对患者进行严密的护理观察,可减少并发症的发生,将有助于该手术的成功。结论介入护理工作对脊髓血管畸形介入治疗手术的成败起着至关重要的作用。     

白消安诱导构建胎鼠肢体畸形模型的研究

目的用易获得的化学药物建立大鼠四肢畸形发生率稳定、畸形类型特异的动物模型。方法采用抗肿瘤类致畸药物白消安作为受试物,观察不同剂量和不同给药时间的胎仔畸形率、畸形类型及特征。结果在大鼠受孕第12天(GD12),一次经口给予白消安25mg/kg时,胎仔畸形类型主要为肢体畸形。肢体畸形率以活胎计为37.9%(33/87),以窝计为61.5%(8/13)。畸形类型常见于多指(趾)和缺指(趾),掌跖骨缺失和骨化不全发生率也较高。此外,还发生胫骨缺失和骨化不全,在观察大体形态时所见的短肢是由胫腓骨缺失和发育不全所致。四肢畸形的发生率和严重程度存在着不对称性,后肢较前肢出现率高,缺指(趾)畸形较其他畸形出现率高。结论成功建立大鼠肢体畸形动物模型,为进一步分析研究肢体发育畸形的分子机制和潜在原因奠定了基础。     

Extended Lateral Supramalleolar Flap for Very Distal Foot Coverage: A Case With Arteriovenous Malformation

Arteriovenous malformation of the foot is very uncommon, and surgical closure after its treatment with embolization and total excision may be challenging for the foot surgeon, particularly in distally localized lesions. A popular method to cover these difficult wounds is free-tissue transfer, which is a highly demanding procedure. Alternatively, distally based regional flaps have been occasionally reported for clinical use in such distant foot defects. Herein, we present a 36-year-old female patient with a diagnosis of arteriovenous malformation arising in the distal medial plantar and dorsal surfaces of the right foot. After surgical resection of the vascular lesion preceded by a misapplied embolization procedure, an extended lateral supramalleolar flap was successfully transferred to the defect area, covering it completely. Functional and aesthetic outcome was satisfactory after 6 months follow-up. Extended lateral supramalleolar flap is a useful and reliable choice for distal foot reconstructions.     

Lateral transmaxillosphenoidal approach to the lateral compartment of the cavernous sinus: technical case report.

This article outlines the surgical technique and the indications for the lateral transmaxillosphenoidal approach, which is illustrated by an index case. A 27-year-old woman presented with a trigeminal sensory deficit caused by a dermoid tumor occupying the lateral compartment of her right cavernous sinus. A lateral transmaxillosphenoidal approach was performed, and the tumor was removed lateral to the intracavernous carotid artery (ICA) and medial to intracavernous cranial nerves. The lateral transmaxillosphenoidal approach is similar to the transmaxillosphenoidal approach used for the removal of pituitary adenomas invading the medial compartment of the cavernous sinus. By opening the lateral wall of the sphenoid sinus just above and laterally to the carotid artery, tumor can be removed medial to the intracavernous cranial nerves and lateral to the ICA.     

Submandibular puncture of the facial vein: An original route for endovascular therapy of cavernous sinus dural fistulas

Introduction

Carotid cavernous sinus fistulas are a potentially severe pathology. Their basic standard treatment is an occlusion of the CCF performed by retrograde venous catheterization via the inferior petrous sinus. When the inferior petrous sinuses are occluded, other alternative venous routes are possible with various subsequent difficulties and risks. We report an original and safe method for endovascular treatment using submandibular puncture of the facial vein.

Clinical cases

We report 4 cases of patients with severe unilateral carotid cavernous sinus fistula associated with the occlusion of both inferior petrous sinuses. A submandibular surgical puncture of the ipsilateral inferior facial vein permitted the catheterization of the fistula. Complete occlusion of carotid cavernous sinus fistula was obtained by using a combination of microcoils and Onyx™.

Discussion

When inferior petrous sinuses are occluded, endovascular treatment of carotid cavernous sinus fistulas is more difficult. After reviewing the other treatment options reported in the literature and their respective advantages and adverse effects, we describe an original technique based on the surgical puncture of the ipsilateral facial vein. The occlusion of the fistula is then obtained by using a combination of microcoils and Onyx™.

Conclusion

When the inferior petrous sinuses are occluded, an endovascular treatment for a carotid cavernous sinus fistula can be performed using an original and secure method. This method relies on a simple surgical puncture of the facial vein in the submandibular region, which then permits a retrograde catheterization of the carotid cavernous sinus fistula with no significant risk.     

Rare Presentation of Foot Postaxial Polydactyly

Polydactyly is a prevalent birth anomaly observed in the foot, and a number of classification systems have been suggested for this condition. Postaxial (fifth or little toe) polydactyly is the most common type. We encountered an exceedingly rare presentation of foot postaxial polydactyly that, to our inspection, had neither been previously classified nor described in published studies. In the present report, we have described an otherwise healthy 2-year-old female who had presented to our clinic with an isolated, extra little toe on her left foot. Foot radiographs revealed the presence of all 5 metatarsals; however, the fifth metatarsal was blocked and did not give rise to the fifth toe. Instead, the fifth (medial normal) and sixth (lateral extra) toes had originated from a single, separate accessory bud from the fourth metatarsal, and the main fourth metatarsal had given rise to the normal fourth toe. The lateral sixth toe was excised, and a periosteal sleeve of the excised extra toe was used for reconstruction of the lateral collateral ligament. We propose that this heretofore unmentioned presentation of postaxial polydactyly be added to the existing systems of classification of pedal polydactyly. A review of the published data pertaining to pedal polydactyly has also been presented.     

Anestesia em cirurgia para correção de pentalogia de Cantrell: relato de caso

Pentalogy of Cantrell is a congenital anomaly associated with defects in the abdominal wall, sternum, diaphragm, and diaphragmatic pericardium formation, in addition to the development of cardiac abnormalities. It is a rare disease with an estimated incidence of one case for every 65,000 births, being more common in males (60% of cases). It has a reserved prognosis with mortality around 63%, and a maximum of 9 months survival after surgery. There are few case reports addressing the pentalogy of Cantrell, which is justified by the rarity of this pathology. In this report our objective was to describe a surgical case of a female patient and make some anesthetic considerations about this rare congenital malformation.     

Anesthetic management of parturients with Arnold Chiari malformation-I: a multicenter retrospective study

BackgroundConsensus regarding the safest mode of delivery and anesthetic management for parturients with Arnold Chiari malformation-I (ACM-I) remains controversial. This study assessed their anesthetic management and reported anesthetic complications during hospitalization for delivery.MethodsThis was a multicenter, retrospective, cohort study of patients with ACM-I undergoing vaginal or cesarean delivery. Data were obtained from the electronic databases of four United States academic institutions using International Classification of Diseases (ICD) codes from 2007–2017 at three sites and 2004–2017 at one site. The primary outcome was anesthetic complications.ResultsData were analyzed for 185 deliveries in 148 patients. Diagnosis of ACM-I was made prior to delivery in 147 (80%) cases. Pre-delivery neurosurgical consultation for management of ACM-I was performed in 53 (36%) patients. Pre-existing symptoms were recorded for 89 (48%) of the deliveries. Vaginal deliveries occurred in 80 (43%) cases, and 62 women (78%) received neuraxial labor analgesia. Cesarean delivery was performed in 105 (57%) cases, of which 70 women (67%) had neuraxial anesthesia and 34 (32%) received general anesthesia. Post-dural puncture headache was reported in three (2%) patients who had neuraxial anesthesia, and in two (12%) patients with syringomyelia. There was one (3%) reported case of aspiration pneumonia with general anesthesia.ConclusionsThe findings suggest that anesthetic complications occur infrequently in patients with ACM-I regardless of the anesthetic management. Although institutional preference in anesthetic and obstetric care appears to drive patient management, the findings suggest that an individualized approach has favorable outcomes in this population.