Feeding difficulties and foregut dysmotility in Noonan's syndrome.

PURPOSE: Noonan's syndrome is a common dysmorphic syndrome in which failure to thrive and gastrointestinal symptoms are frequent but poorly understood. DESIGN: Twenty five children with Noonan's syndrome were investigated by contrast radiology, pH monitoring, surface electrogastrography (EGG), and antroduodenal manometry (ADM). RESULTS: Sixteen had poor feeding and symptoms of gastrointestinal dysfunction. All 16 required tube feeding. Seven of 25 had symptoms of foregut dysmotility and gastro-oesophageal reflux. In the most symptomatic children (four of seven) EGG showed fasting frequency gradient loss along the stomach fundus and pylorus with antral postprandial frequency loss. ADM showed shortened fasting cycle length, with abnormal phase III and shortened postprandial activity containing phasic contractions. IMPLICATIONS: Gastroduodenal motor activity was reminiscent of 32-35 week preterm patterns. The feeding difficulties appear to resolve as gut motility matures. In Noonan's syndrome, feeding problems appear to be the result of delayed gastrointestinal motor development.

The evolution of the distal radio-ulnar joint.

The pectoral girdle and the distal radio-ulnar joint have evolved over a period of 400 million years. Clinical circumstances exist that may represent atavistic development in modern man. Current understanding of this complex area does not explain all clinical situations.

A comprehensive approach to surgical management of the type IIIA hypoplastic thumb.

In order to adequately identify pathologic anatomy and effectively reconstruct 14 type IIIA hypoplastic thumbs, a comprehensive clinical evaluation and surgical approach was employed. Eleven patients had congenital differences in the forearm, while all patients had anomalies in the wrist, hand, and digits. In addition to well-described interconnections between the flexor pollicis longus and the extensor pollicis longus, and thenar muscle hypoplasia, the authors observed duplication of musculotendinous units, anomalous muscles between the thumb and index rays, and abnormal insertions or dense adhesions along tendons as proximal as the forearm level. Successful reconstruction required an extended approach from the digit to the forearm, through which division of abnormal connections, reorientation of tendons, and lysis of adhesions was performed. Opposition transfer was needed in only 8 of the patients after the other pathologies were treated. Web-space deepening and ulnar collateral ligament reconstruction was performed when indicated. Improvement in function and appearance was achieved.

Mimickers of hand infections.

A variety of local or systemic conditions may mimic an infectious process in the hand and upper extremities. Familiarity with these conditions is important so that appropriate diagnosis and management may ensue. Most of the conditions presented in this article may be successfully managed nonsurgically; however, certain conditions such as the extravasation of a chemotherapy agent or retained foreign body require surgical intervention.

Fibrous hamartoma of infancy in the hand: a case report.

Fibrous hamartoma of infancy (FHI) is a rare, benign tumor. The proximal upper extremity is most commonly involved. A previously unreported case of FHI in the hand is presented.

Intra-articular fractures of the distal end of the radius treated with an adjustable fixator system.

Twenty-five wrists with comminuted, displaced, intra-articular fractures of the distal radius were prospectively treated with an adjustable external fixator for an average of 51 days. Twenty fractures (80% [20 of 25] were available for follow-up) in 16 adults (mean age, 34 years) were treated with the Wrist Jack external fixator system (Hand Biomechanics Lab, Sacramento, CA) and evaluated at a mean follow-up period of 25 months. Ten patients (12 fractures) sustained high-energy trauma with multiple injuries, while 6 patients (8 fractures) sustained isolated distal radius fractures. Percutaneous pins supplemented the fixation in 6 fractures. All fractures were reduced to restore articular congruity to within 1 mm. At follow-up, 5% were excellent, 75% good, 20% fair, and none as poor using the demerit point system of Gartland and Werley as modified by Sarmiento. Grip strength averaged 80% of the unaffected limb. Seventeen of the 20 fractures showed some evidence of articular incongruity at follow-up evaluation. Restoration of palmar tilt, radial inclination, radial length, and range of motion were at acceptable values. Subjective analysis confirmed 85% of the patients to have only occasional pain or none at all and 15% to have some pain with weakness or limitation of motion. Two patients required additional surgery: 1 underwent a Darrach procedure and the other a tendon transfer for a rupture of the extensor pollicis longus tendon. Results suggest that an external fixator system provides an additional alternative to the surgical armamentarium for an otherwise difficult fracture fixation problem.

Major vascular malformations of the upper extremity: long-term observation.

Vascular malformations of the upper extremity continue to be a therapeutic challenge to the hand surgeon. In the past 22 years, the authors treated 17 patients with major vascular malformations of the upper extremity. The lesions were evaluated with the aid of plain radiographs, angiography, and histology. Thirteen of the patients were female and 4 were male. The average age at treatment was 18 years, ranging from birth to 64 years. The average follow-up period was 12 years, ranging from 4 months to 36 years. The main indications for surgery were pain, progressive enlargement, neurologic compromise, diminished function, or a combination of these. The progression of these lesions under observation led to surgical management in all cases. A total of 36 excisions, 3 embolizations, and 4 amputations were done. Postoperative angiograms were obtained in selected individuals. Diffuse lesions defied total surgical removal. Recurrence and persistence was evident in 12 of the 17 patients. A knowledge of the progressive nature of these lesions and the need for long-term observation are the keystones to the successful management of these challenging yet unresolved problems.

Open reduction and internal fixation of forearm fractures in children.

We retrospectively reviewed 16 children younger than 13 years with 17 fractures of the shafts of the radius or ulna or both who had undergone an open reduction-internal fixation (ORIF). ORIF was performed when a closed reduction was deemed unacceptable in 14 radius fractures and for three unstable open fractures of the radius. The average age was 9.4 +/- 2.3 years (range, 5.0-12.5). Of the 14 fractures with an unacceptable closed reduction, soft-tissue interposition was encountered in seven. Fixation was secured by plates and screws, percutaneous Steinmann pins, or intramedullary Steinmann pins. There were no delayed unions or nonunions, no infections, and no neurovascular injuries. The average follow-up was 12.3 months; all 17 fractures had excellent results (forearm rotation loss of < 10 degrees). Our study indicates that excellent results can be expected with no increased risk of complications if the treating physician elects to proceed with an ORIF in a pediatric forearm fracture with proper indications.