A case report of patch aortoplasty for supravalvular aortic stenosis associated with Williams syndrome]

A 15-year-old girl underwent patch aortoplasty for supravalvular aortic stenosis in association with Williams syndrome. Pressure gradient of before and after stenosis of the aorta decreased from 104 mmHg to 16 mmHg at the postoperative catheterization. Angiography after the operation showed no stenosis. Postoperative course was excellent and discharged on 14th postoperative day. The problems of the operation of supravalvular aortic stenosis were discussed.     

Extended aortoplasty to relieve supravalvular aortic stenosis

An extended aortoplasty was used to relieve severe supravalvular aortic stenosis in 5 patients whose preoperative left ventricular-aortic gradient ranged from 85 to 140 mm Hg (median, 120 mm Hg). The stenotic ring above the commissures was divided in two places by an inverted U incision extending into the right and noncoronary sinuses. A distal vertical incision in the ascending aorta converted this into an inverted Y. The ridge above the left coronary sinus was excised. The aortic incision was repaired with an inverted Y-shaped Dacron gusset. The postoperative gradient ranged from 0 to 30 mm Hg (median, 15 mm Hg). The extended aortoplasty provides excellent relief of supravalvular aortic stenosis and, in addition, restores the aortic root geometry to a much more anatomical configuration than is achieved with the simple patch technique.     

Severe supravalvular aortic stenosis: successful surgical correction by extended aortoplasty

Surgical treatment of severe discrete supravalvular aortic stenosis by placement of a patch graft across the narrowed area of aorta at one point has been associated with a substantial incidence of residual stenosis. Successful relief of a severe stenosis was achieved by a modification of a previously reported technique in which the stenotic area is expanded at two points. Use of this technique should be considered in cases of severe discrete supravalvular aortic stenosis.     

Reoperation for diffuse supravalvular aortic stenosis with Williams syndrome —Extended patch aortoplasty and extra-anatomic bypass from the ascending aorta to the descending aorta in a median sternotomy—

A case of diffuse supravalvular aortic stenosis (SVAS) with Williams syndrome is reported. In this case of severe diffuse SVAS, we performed the diamond-patch aortoplasty in a child. However he has been suffering from residual SVAS. At 9-years old, the myocardial injury was noted by myocardial scintigraphy. Preoperative cardiac catheterization and angiography revealed the hypoplastic ascending aorta and arch with a pressure gradient of 89 mmHg at the distal site from the left subclavian artery. Through only a median stenotomy, an extended patch aortoplasty between the valsalva sinus and distal arch was performed and an extraanatomic bypass from the ascending aorta to the descending aorta was employed using a 10 mm tube graft. We realize this technique is available because this method can relieve the left ventriculus of the pressure load and operate via only median sternotomy.     

The use of Doty's extended aortoplasty in binovular twins with congenital supravalvular aortic stenosis]

Binovular twins with congenital supravalvular aortic stenosis underwent Doty's extended aortoplasty. Neither twins displayed signs of Williams syndrome. Peak systolic pressure gradients of 65 and 70 mmHg, respectively, were measured across the stenotic portion at the ascending aortae. After surgery, the pressure gradients were reduced to 15 and 20 mmHg, respectively, at the aortic valves. No new aortic regurgitation was observed postoperatively. It is important that the ascending aorta, including the stenotic part, is symmetrically incised into the noncoronary and the right coronary sinus of Valsalva. The fibrous ring should be removed as completely as possible. Doty's extended aortoplasty is a safe and effective procedure for supravalvular aortic stenosis.     

Anaesthetic management of a caesarean section in a patient with Marfan's syndrome and aortic dissection

This report describes a case of a Stanford Type B aortic dissection (originating distal to the left subclavian artery and extending to the aortic bifurcation and proximal left iliac artery) in a 31-year-old primigravid woman who was at 39 weeks gestation and had Marfan's syndrome. The dissection was managed conservatively. Caesarean section was performed under epidural anaesthesia with aggressive control of hypertension. Postoperatively, there was no extension of the dissection and no aneurysm formation. She was discharged from hospital two weeks after delivery and remained asymptomatic at six months. There are no plans for surgical intervention.     

Patch annulo-aortoplasty in an adult patient with congenital supravalvular aortic stenosis and a small aortic annulus

A 39-year-old woman with familial homozygous hypercholesterolemia had supravalvular and valvular aortic stenosis. Modified Nick’s procedure and aortic valve replacement was performed to relieve both the supravalvular and annular stenoses. At surgery, the ascending aorta was found to be narrowing at the level of the sinotubular junction, which was compatible with congenital supravalvular aortic stenosis. Histological examination of the aortic cusps showed sclerotic change due to hypercholesterolemia. These findings indicated that familial homozygous hypercholesterolemia caused valvular aortic stenosis and exacerbated congenital supravalvular aortic stenosis.     

Anaesthetic management of a patient with Freeman Sheldon syndrome

We present a case of Freeman Sheldon (Whistling Face) syndrome. The anaesthetic management of an additional cervical kyphoscoliosis is discussed.     

Anaesthetic management of a patient with myasthenia gravis and tracheal stenosis

Purpose

The combination of myasthenia gravis and tracheal obstruction presents a number of difficulties for anaesthetic management. This case illustrates the advantages of careful planning.

Clinical features

A 66-yr-old man with myasthenia gravis required resection of a stenosis at the site of an old tracheostomy. The primary goal was to accomplish safe management of the airway, a task made more difficult because the airway was shared with the surgeon. Awake fibreoptic examination of the tracheal stenosis performed in the operating room provided useful information in planning the subsequent anaesthetic. From this examination, it was found that the trachea could be intubated by a normal endotracheal tube passed through the stenosis over the fibreoptic bronchoscope. Intraoperatively, the orotracheal tube was withdrawn temporarily and replaced with an endotracheal tube placed by the surgeon into the distal trachea. Extubation was carried out judiciously and a plan for reintubation prepared in advance. The anaesthetic plan was modified because of the myasthenia gravis. Following careful investigation of the extent of the patient’s disease and its treatment, an assessment was made of the patient’s need for postoperative ventilation. The anaesthetic plan included maintenance of anticholinergic medications until the time of surgery and their early resumption postoperatively, avoidance of neuromuscular blocking agents, and careful monitoring of neuromuscular function during the anaesthetic.

Conclusion

Careful examination of the area of tracheal stenosis and a carefully considered plan for reintubation are prerequisites for this type of surgery. Clinically well controlled myasthenia gravis was managed successfully using familiar principles.     

Airway management in a patient with Williams syndrome

Williams syndrome is characterized by the triad of supravalvular aortic stenosis (SAS), mental retardation and elfin facies. Generally, difficult airway is expected in patients with Williams syndrome by characteristic face. A 26-year-old female with Williams syndrome was scheduled for abdominal myomectomy under general anesthesia. Difficult mask ventilation and tracheal intubation were anticipated because of micrognathia, mandibular retrusion, and a Mallanpati class III airway. Before induction of anesthesia the patient breathed 100% oxygen for 3 minutes. Anesthesia was induced and maintained with propofol, remifentanil and rocuronium bromide. Mask ventilation was easily performed. The direct laryngoscopic view was Cormack grade I and there was no difficulty in the tracheal intubation. After induction of anesthesia, anesthetic course was uneventful. According to the most previous clinical reports in patients with Williams syndrome in Japan, mask ventilation and tracheal intubation were performed easily contrary to preoperative airway assessment. In view of SAS, mental retardation, airway deformity and airway assessment in previous clinical reports, we should select the optimal strategy for airway management in patients with Williams syndrome.