Elhassan Bassem T, Biafora Sam, Light Terry
University of Illinois at Chicago, Chicago, IL, USA.
J Hand Surg Am. 2007 Sep;32(7):1024-30. doi: 10.1016/j.jhsa.2007.05.020.
Ulna longitudinal dysplasia is an uncommon congenital anomaly that demonstrates a wide variety of clinical manifestations. The clinical manifestations and function of patients with Bayne type IV ulna longitudinal dysplasia have not been well characterized. The purpose of this study was to report the clinical features of type IV ulna longitudinal dysplasia and the extent to which this affects a patient's ability to perform activities of daily living.
The medical records of children diagnosed with ulna longitudinal dysplasia in our institution between 1960 and 2004 were reviewed. The children found to have ulna longitudinal dysplasia with radiohumeral synostosis (Bayne type IV ulna dysplasia) were studied. The laterality of the deformity, associated musculoskeletal and nonmusculoskeletal anomalies, and treatments were recorded. Patients were interviewed regarding their ability to perform activities of daily living.
One hundred twenty-five patients with 146 affected limbs were identified with ulna dysplasia. Seventeen limbs in 14 patients (12% of affected limbs) demonstrated radiohumeral synostosis (RHS). Three of 14 patients with RHS had bilateral involvement. The elbows were fixed in 20 degrees to 90 degrees of flexion. No elbows were positioned in full extension. Eleven of the 17 involved limbs with RHS had digital anomalies. Nine of the 17 limbs had surgical reconstruction. The majority of these procedures were performed on the hand.
The elbow, forearm, wrist, and hand clinical findings associated with type IV ulna longitudinal dysplasia are variable. Surgical treatment usually focuses on correction of hand abnormalities. Many patients function satisfactorily and are able to perform daily activities without surgical intervention.
尺骨纵向发育异常是一种罕见的先天性畸形，具有多种临床表现。Bayne Ⅳ型尺骨纵向发育异常患者的临床表现和功能尚未得到充分描述。本研究的目的是报告Ⅳ型尺骨纵向发育异常的临床特征以及其对患者日常生活活动能力的影响程度。
回顾了 1960 年至 2004 年在我们机构诊断为尺骨纵向发育异常的儿童的病历。对发现患有伴有桡肱关节融合的尺骨纵向发育异常（Bayne Ⅳ型尺骨发育异常）的儿童进行了研究。记录了畸形的侧别、相关的肌肉骨骼和非肌肉骨骼异常以及治疗情况。就患者进行日常生活活动的能力对其进行了访谈。
确定了 125 例患有尺骨发育异常且有 146 条患肢的患者。14 例患者的 17 条肢体（占患肢的 12%）表现出桡肱关节融合（RHS）。14 例患有 RHS 的患者中有 3 例为双侧受累。肘部固定在 20 度至 90 度的屈曲位。没有肘部处于完全伸展位。17 条患有 RHS 的受累肢体中有 11 条存在手指异常。17 条肢体中有 9 条进行了手术重建。这些手术大多是针对手部进行的。
与Ⅳ型尺骨纵向发育异常相关的肘部、前臂、腕部和手部的临床发现各不相同。手术治疗通常侧重于纠正手部异常。许多患者功能良好，无需手术干预就能进行日常活动。
