Torticollis in children caused by congenital anomalies of the atlas

Hemi-atlas is a rare congenital anomaly in the formation of the first cervical vertebra. It may cause a rather severe and progressive torticollis. When a child is young, the neck, despite the deformity, is flexible and the torticollis can be passively corrected. However, in some patients it becomes increasingly severe and fixed. I describe the findings in seventeen patients, seven of whom were operated on between 1975 and 1983. Treatment with a brace was shown to be ineffective. In patients with severe deformities, fusion of the upper part of the cervical spine is recommended. Fusion was obtained in all seven patients in whom it was attempted, and there was good postural correction in all. Early operation is recommended if the deformity is increasing. Gradual correction in a halo cast followed by posterior fusion is recommended as the treatment of choice in patients with severe torticollis.     

Cervicobrachialgia with congenital vertebral anomalies and diastematomyelia

A case of diastematomyelia in an adult female patient is reported. The relationship of the cervicobrachialgia, which was the presenting sign, to the diastematomyelia and the congenital vertebral anomalies is discussed.     

Torticollis due to atlanto-axial rotatory fixation following general anaesthesia

Atlanto-axial rotatory fixation is uncommon, but should be suspected in any patient developing a torticollis during the recovery period of an operation performed on the head and neck under general anaesthesia. The present case report shows that the condition can occur in adults as well as children. A high degree of suspicion is required to instigate appropriate imaging. CT provides unequivocal proof and will lead to early successful treatment.     

Delayed presentation of a congenital recto-vaginal fistula associated with a recto-sigmoid tubular duplication and spinal cord and vertebral anomalies

Tubular duplication of the recto-sigmoid colon is a rare entity. Associated anomalies including fistulae to the genitourinary tract may be found. A baby girl was found to have duplication of the recto-sigmoid colon, anomalies of sacral vertebra from S1 to S5, and solitary right kidney. The septum of this duplication was divided using staplers. Because of a history of stool coming from the vagina, a meticulous examination perioperatively was performed, but no fistula could be found. Further extensive investigation failed to show any fistula. At the age of 10 she was operated on for a tethered cord. At age 14, she experienced passage of a small amount of liquid stool per vaginum. A recto-vaginal fistula was found. Via a posterior sagittal incision, the fistula was closed by a transrectal approach. She remained asymptomatic for 16 months until the fistula recurred. Using a perineal approach, a very short fistula between the vagina and the rectum was closed. The closure was reinforced by a vaginal flap. Four months later, she remains without signs of recurrence.     

Cervical vertebral erosion due to tortuous vertebral artery

A case of cervical vertebral erosion due to tortuous vertebral artery is presented. This entity is rare and only 11 cases have been reported in the literature. The present case is the first to be demonstrated by magnetic resonance imaging. The importance of considering this vascular anomaly in the differential diagnosis of cervical spinal tumors is discussed.     

指蹼瘢痕挛缩畸形的修复

目的探讨指蹊瘢痕挛缩畸形的修复方法。方法对指蹊背侧瘢痕，在指侧形成旗状瓣修复。对于掌侧瘢痕，设计矩形皮瓣转移修复。指侧不能直接拉拢缝合，而采用断层或全层皮片移植修复。结果本组68个指蹊，采用旗状瓣修复54个、矩形瓣修复14个，无皮瓣血运障碍，再造指蹊的大小及深度近似正常指蹊，效果满意。结论指蹊背侧瘢痕挛缩采用旗状瓣修复，掌侧瘢痕挛缩采用矩形瓣修复，是治疗指蹊挛缩畸形的较好方法。     

指蹼瘢痕挛缩畸形的修复

目的探讨指蹼瘢痕挛缩畸形的修复方法.方法对指蹼背侧瘢痕,在指侧形成旗状瓣修复.对于掌侧瘢痕,设计矩形皮瓣转移修复.指侧不能直接拉拢缝合,而采用断层或全层皮片移植修复.结果本组68个指蹼,采用旗状瓣修复54个、矩形瓣修复14个,无皮瓣血运障碍,再造指蹼的大小及深度近似正常指蹼,效果满意.结论指蹼背侧瘢痕挛缩采用旗状瓣修复,掌侧瘢痕挛缩采用矩形瓣修复,是治疗指蹼挛缩畸形的较好方法.     

指深屈肌腱粘连致假性缺血性肌挛缩的临床分析

目的报告临床较为少见的因指深屈肌腱粘连引起症状类似于轻度缺血性肌挛缩的诊断和治疗。方法对5例指深屈肌腱粘连致屈指畸形的患者,术中探查前臂深、浅层屈肌群未见变性、挛缩,而深层屈肌肌腱和肌腹交界处与骨及筋膜相互粘连形成纤维束带。2例行3～5指指深屈肌松解、束带切除术,2例行指深屈肌自身延长,1例行指浅屈肌移位代指深屈肌术。结果术后随访6～21个月,疗效明显,手功能评定:优(S4M5)4例,可(S2M3)1例。结论指深屈肌腱粘连引起的假性缺血性肌挛缩因与前臂缺血性肌挛缩相鉴别,早期诊断及治疗可明显改善手功能。     

Quadricepsplasty for congenital dislocation of the knee and congenital quadriceps contracture

Purpose

We encountered problems with the Curtis and Fisher technique of quadricepsplasty for congenital quadriceps contracture, including wound dehiscence, insufficient lengthening of the quadriceps and instability of the knee. We modified the operative technique to address these three problems. We undertook this study to evaluate the results of the modified technique of quadricepsplasty to determine if we succeeded in overcoming these limitations of the original technique.

Methods

Twenty children (33 knees) underwent the modified Curtis and Fisher quadricepsplasty through a lateral incision; a long tongue of the rectus femoris was raised and the vasti mobilised without dividing the lateral retinaculae till the collateral ligaments. The children were followed up for a mean period of 63 months and evaluated. The healing of the wound, active and passive range of motion (ROM) of the knee, the stability of the knee, quadriceps power and knee function were assessed.

Results

Primary wound healing occurred in 32 of 33 knees. Adequate lengthening of the quadriceps sufficient to facilitate knee flexion to 90° was possible. Considerable improvement in the ROM was noted. In non-syndromic congenital dislocation of the knee (CDK), the quadriceps power was Grade 5, but minor degrees of extensor lag was noted. In a proportion of patients, minor degrees of joint instability was present. The majority of children were community walkers. The overall results were better in non-syndromic CDK than in children with arthrogryposis, but differences of some variables were not significant.

Conclusion

The modifications to the original Curtis and Fisher technique overcame the specific problems they were expected to avoid.     

Isolated intraventricular hemorrhage due to a ruptured vertebral artery aneurysm

A rare case of ruptured aneurysm originating from the vertebral artery, and presenting an isolated clot in the fourth ventricle is reported. The patient underwent surgery via a right lateral suboccipital craniectomy on the third day. As the aneurysm was huge and partially thrombosed, trapping was performed and completed successfully. If isolated intraventricular hematoma was found without obvious parenchymal hemorrhage or subarachnoid clot, the diagnosis of vertebral artery aneurysm should be considered as a possibility in addition to posterior inferior cerebellar artery aneurysm. Complete vertebral angiography is indispensable for the recognition of this condition.     

Intrathoracic meningocele and vertebral anomalies in a case of neurofibromatosis.

A case of an intrathoracic meningocele associated with vertebral scalloping, enlarged intervertebral foramina and scoliosis in neurofibromatosis without spinal or nerve root rumors is described. A markedly attenuated dura mater anteriorly was present at autopsy and may have predisposed to meningeal herniation and scalloping.