gms | German Medical Science

Objectives/Interrogation: Dupuytren's disease is a benign fibromatosis of the palmar and digital fascia of the hand. It is well known in adults with an overall incidence of 3-6%. To the best of our known, there are only 13 cases documented in the English bibliography in children under 13 and only seven cases histologically confirmed in children under 10 years.

Our goal is to present the case report of a 6-year-old boy with histologically confirmed Dupuytren's disease.

Methods: 6 years-old male referred to our consultation by retraction of his fourth finger in his left hand. When he was 14 months, a longitudinal congenital cutaneous scar was operated on the ulnar side of his fifth finger and, at 3 years-old, a zeta-plasty was performed also on his fifth finger.

Currently he presents a nodule and a palmar and ulnar cord in his fourth finger that conditions an irreducible flexion of DIP joint in 80º and a clinodactyly of 15º towards ulnar. His mother reports that this deformity began two years ago (when he was 4 years old) as a bulge and it has been subsequently retracting the finger.

Surgery was performed on an outpatient basis. A palmar-ulnar hemi-Bruner incision was made on the fourth finger, identifying a subcutaneous scar that did not invade the skin and that was extended from the PIP joint, displacing the digital neurovascular bundle, to the ulnar side of the DIP joint where it was attached to the ulnar collateral ligament and the joint capsule. A complete resection of the flange was achieved with a complete intraoperative range of motion and correcting clinodactyly. A proximal advance-rotation flap and a V-Y cutaneous plasty for cutaneous closure were performed. A splint was place to keep the interphalangeal joints in extension for 3 weeks.

Results and Conclusions: The wounds healed without incident. At 5 weeks, ROM was complete and the child had been returned to his daily-activities. One year after surgery there were no signs of recurrence or involvement in another location.

Dupuytren's disease in the child is exceptional. The differential diagnosis should include camptodactyly, sequelae of burn, extraperitoneal fibromatosis, epithelioid sarcoma, giant cell tumor or fibrous hamartoma among others. Therefore, the surgical treatment is not only functional but it also must confirm the diagnosis histologically. The risk of recurrence of the disease in the pediatric age and the optimal time for surgery are still uncertain due to the limited bibliography available.