The treatment of crossbones of the hand.

Over a thirty-year period at Loma Linda University, we have seen eight patients with nine crossbones of the hand. These patients fall into the group of central deficiency with a cleft hand and central polydactyly. As will be noted in the paper, these two conditions may have a similar developmental relationship. Although the literature in the past specifies mainly one option - to remove the crossbone - we consider several different options that one should think about before removal of the crossbones.

Duplicated longitudinal bracketed epiphysis 'kissing delta phalanx' in the hand.

The duplicated longitudinal bracketed epiphysis ("kissing delta") phalanx has been described rarely. These delta bones have a bizarre appearance and are often associated with other developmental abnormalities such as central polydactyly. We have seen a total of 13 kissing delta phalanges in the hands. We discuss their natural history, look at the associated anomalies and consider the treatment options for this very rare congenital anomaly.

Synostosis of the ring-small finger metacarpal in Apert acrosyndactyly hands: incidence and treatment.

SUMMARY: This study reports on the incidence of a synostosis between the ring-small metacarpal bases in patients with Apert syndrome and describes a technique to resect the synostosis and insert silicone sheets to improve hand function. Records of 9 patients (18 hands) were evaluated. Average age at follow-up was 9.5 years. Three relationships between the ring-small metacarpals were observed. Type I hands (22%) had no abnormal interconnections; type II hands (33%) possessed a synostosis since birth; type III hands (44%) did not possess a synostosis at birth, but one later developed at average age of 79 months. Total incidence of synostosis was 77%. Five patients underwent excision of the synostosis. At an average of 16 postoperative months, 4 of these patients experienced improvement in hand prehension. In patients with Apert syndrome, resection of abnormal interconnections between the ring-small metacarpals is highly recommended to improve hand prehension based on the high incidence of a synostosis in this region.

An experience of the Snow-Littler procedure.

This paper reviews the results of the Snow-Littler procedure performed in twelve hands with classical central longitudinal deficiency and in one hand with symbrachydactyly, cleft type. There were no instances of major flap necrosis although two flaps showed tip ischaemia. The width of the first web was, in the main, satisfactory but four webspace revisions were performed. Supplementary skin grafting at the time of surgery was necessary in complete and/or complex thumb index syndactylies and in the patient with symbrachydactyly. In eight cases, a transverse metacarpal ligament was reconstructed. In the five other cases, no clinical instability or radiological divergence of the index and ring fingers occurred, in spite of no transverse metacarpal ligament reconstruction. Three de-rotational osteotomies of transposed index fingers were performed in patients who had a transverse metacarpal ligament reconstruction. These results indicated significantly improved appearance and improved function following the Snow-Littler procedure.

Duplicated longitudinal bracketed epiphysis "kissing delta phalanx" in Rubinstein-Taybi syndrome.

A duplicated longitudinal bracketed epiphysis or the "kissing delta" phalanx is not well recognized in the world literature. This represents complex double-delta bones with a longitudinal epiphyseal bracket. This complex is composed of a back-to-back fusion of adjacent delta bones enclosed in a bracket. The convex side of each delta phalanx faces the other; hence the term kissing-delta bone. These delta bones have a very bizarre appearance and are associated with the Rubinstein-Taybi syndrome. We have seen a total of 36 kissing-delta phalanges in the foot. We discuss the natural history and treatment options of this very rare congenital anomaly. This is not a surgical-outcome study, but surgical treatment is observed.

Hand involvement in 13q deletion syndrome.

Deletion in the long arm of chromosome 13 is relatively rare. Fewer than 100 cases are reported in the literature. Patients with 13q deletion have widely variable phenotypes. Hand anomalies, when present, include absent or hypoplastic thumbs, bony synostoses of the metacarpals, and brachyphalangy of the middle phalanx of the little finger. We report four cases with 13q deletion seen at our Hand Clinic.

Cleft-foot closure: a simplified technique and review of the literature.

The purpose of this article is to present a simplified technique to close the cleft in the feet of children affected with congenital clefts. This technique evolved after several frustrating attempts to close the cleft with mediocre results by using triangular flaps. This report concerns nine children with 15 feet. We describe a simplified technique for cleft closure to narrow the feet. One of the children had an associated syndactyly of the foot, and two others had syndactyly of the hands. No other associated anomalies were present in these children. We operated on seven patients with a total of 42 operations. The feet with the clefts that were repaired by rectangular flaps maintained good cosmesis and function.

The dorsal transposition flap for congenital contractures of the first web space: a 20-year experience.

Fifty-four dorsal transposition flaps were performed in 38 patients (46 hands) for severe congenital thumb web-space contractures. At an average follow-up interval of 6 years, complications or unsatisfactory results had occurred in 100% of symbrachydactyly patients and in 27% of all other patients. Postoperative first web-space angle was augmented an average of 59 degrees with a distal flap two-point discrimination of 8 mm. Mean postoperative pinch and grip strengths were 58% and 72%, respectively, of the normal contralateral hand. Parental satisfaction with the functional results was high, and the aesthetic results were generally acceptable. With the exception of symbrachydactyly patients, the dorsal transposition flap remains an excellent reconstructive option for severe congenital contractures of the thumb web space.

Spinal accessory nerve in a trapezius-splitting surgical approach.

To elucidate the safe limits for muscle-splitting incisions of the trapezius muscle, 25 cadaver shoulders were studied. To describe the position of the nerve distances were expressed as a percentage of the distance from the tips of the vertebral spinous processes to the lateral tip of the acromion. The spinal accessory nerve was found to run a vertical course medial and parallel to the vertebral border of the scapula. Three to six nerve branches (average 3.8) also ran a vertical course lying between 33% to 50% of the distance from the tips of the vertebral spinous processes to the lateral tip of the acromion. The most lateral branch lay an average at 44% distance and never beyond 50%. Muscle-splitting incisions are relatively safe in the lateral 50% of the muscle. In the medial half they are to be avoided or pursued with great caution.

The shoulder and elbow in Apert's syndrome.

Nine children with Apert's syndrome were studied and found to have progressive bony dysplasia in the shoulder and elbow. The children showed loss of joint motion and radiographic abnormalities including subluxation and flattening of the humeral head with irregularities of the glenoid. The elbow showed flattening and subluxation of the radial head in radiographs and a positive click on physical examination. The limitation of motion and the presence of bony defects were progressive, suggesting that Apert's syndrome is a progressive generalized dysplasia.

Treatment of the upper limb in Charcot-Marie-Tooth disease.

Even though Charcot-Marie-Tooth (CMT) disease is seen frequently, relatively little has been written about the successful treatment of upper limb involvement using tendon transfers. It appears that there are several types of CMT disease and there is extreme variability in the clinical symptoms. The results of treatment were assessed by looking at four specific areas of hand function: decreased conduction velocity of the nerves, lack of opposition, weak pinch, and clawing of the fingers. A plan has been developed for tendon transfers that has worked well in clinical practice.

Intra-uterine and juxtanatal repair of syndactyly in foetal mice.

39 foetal mice with genetic syndactyly were identified in utero at 17 days of gestation, and the right hindfoot extruded through the uterus. The syndactylous digits were separated by simple incisions. In one group (n = 25) digit separation was maintained during wound healing by the interdigital application of a silver microclip. Digit separation was also assessed in a second group of newborn mice less than 24 hours old (juxtanatal population, n = 24). Two foetuses (5%) and six newborns (25%) developed digital necrosis following microclip application. In the remaining microclipped animals (23 intrauterine and 10 juxtanatal), microclip application maintained digit separation, allowing wound healing to occur with epithelialization of the separated digits. No inflammation or scar formation occurred. In the third group (n = 22) without microclip application, the digital skin reapproximated and webbing recurred during wound healing. These studies demonstrate the need to maintain digit separation during wound healing following intra-uterine or juxtanatal syndactyly repair.

Results of upper plexus thoracic outlet syndrome operation.

A review of 181 patients with thoracic outlet syndrome treated in our clinic revealed that 22 (12%) showed symptoms of compression of the distal median nerve only. Although there are several distinct anatomic abnormalities involving the upper brachial plexus, a first rib resection using the transaxillary approach was found to relieve these patients' symptoms as well as it did in those who exhibited the classic findings of ulnar nerve paresthesias. A comparison of the patients' outcomes showed no appreciable differences in the results obtained in those with distal median nerve compression symptoms only, lower plexus involvement, or combined upper and lower plexus symptoms.

Trauma-related thoracic outlet syndrome.

181 patients treated for thoracic outlet syndrome by first rib resection were reviewed. 88 (49%) indicated a single traumatic event which precipitated the thoracic outlet syndrome. 79% of patients with a history of a single traumatic episode had good to excellent results after first rib resection. A separate group of 18 patients developed work-related thoracic outlet syndrome due to repetitive activities. Ratings of good to excellent results were lower (66%) among workmen's compensation cases.

Treatment of finger dysplasia with metacarpal base defect.

The hypoplastic finger with metacarpal base defect is a rare anomaly. Metacarpal lengthening is not a reasonable treatment option because of severe hypoplasia of the metacarpal. Complete amputation with closure of the interdigital space is effective but most parents want to save any digit of their child. We prefer to release the syndactyly and save the finger, with its shortened three phalanges, during childhood. Our parents and patients have been satisfied with the short digit. At a later age the metacarpal of the hypoplastic finger can be fused to one or the other adjacent metacarpals. Alternative methods to increase function and improve appearance are presented.