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Congenital Symptomatic Scaphoid-Trapezium Coalition in a Pediatric Patient with VACTERL Association: A Case Report
Rushil Gupta, BS, Atharva M Bhagwat, BA, Rohun Gupta, MD; Brian Mailey, MD
Saint Louis University School of Medicine, Saint Louis, MO

Introduction

Carpal coalition (CC) is the union between two or more carpal bones that should otherwise have uninterrupted mobility. Of those reported among pediatric populations, the vast majority are lunotriquetral (LT, 69.2%) or capitohamate (CH, 17.3%), while scaphotrapezial (ST) is among the most rare. Here, we report a case of a pediatric patient with symptomatic congenital ST-coalition as part of VACTERL association.

Materials & Methods

A case report is presented for a 9-year-old right-hand dominant female with right wrist and snuffbox pain following a fall-on-outstretched hand, later revealed to be symptomatic congenital ST-coalition. Her medical history was significant for ventricular septal defect and tracheoesophageal fistula, suggesting possible VACTERL association.

Results

Initial radiographs revealed no acute fractures, but raised concern for a possible developmentally abnormal carpus (Figure 1). The patient returned five months later due to worsening pain in the radial aspect of her right wrist and first carpometacarpal (CMC) joint. Physical examination revealed CMC joint tenderness, severe thenar wasting, weakened thumb grip, and reduced oppositional movement. MRI revealed hypoplastic thumb and ST-coalition, with genetic testing confirming diagnosis of VACTERL association. Surgical management included a right ST- coalition resection, partial carpectomy of the proximal trapezium and distal scaphoid, and fat grafting to the ST interval (Figure 2). The procedure was successful, with no postoperative complications. The patient reported significantly decreased pain with activities that previously elicited pain such as writing and sports, with moderate pain at the surgical site.

Conclusions

Carpal coalition is a rare congenital abnormality, typically asymptomatic and found incidentally on radiographs. Most cases are often resolved conservatively, though surgery may be indicated for those with considerable pain and impairment. For pediatric patients that may have other association/syndrome indications (e.g., VACTERL, Holt-Oram) and otherwise continual wrist pain failing to respond to conservative therapy, CC should be considered within the patient's differential.

Figures

Figure 1. Baseline radiograph of right-hand wrist depicting ST-coalition prior to carpectomy.

Figure 2. Postoperative radiograph of right-hand wrist depicting ST-coalition resection.

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