Article
A successful use of collagenase injection multiple fingers contractures in a child in the course of a generalized collagenopathy and Arnold Chiari Malformation Type I
Search Medline for
Authors
-
Kaja Gizewska-Kacprzak
- Department of Pediatric and Oncological Surgery, Urology and Hand Surgery, Pomeranian Medical University in Szczecin, Szczecin, Poland
-
Ireneusz Walaszek
- Department of Orthopaedics, Traumatology and Oncology, Pomeranian Medical University in Szczecin, Szczecin, Poland
| Published: | February 6, 2020 |
|---|
Outline
Text
Objectives/Interrogation: Dupuytren disease is extremely rare among the pediatric population. We present an extremely rare case bilateral multiple finger contractures of a yet unknown collagenopathy in a child treated with collagenase injection.
Methods: A 17-year-old girl presented in a Pediatric Hand Surgery ambulatory with advanced contractures of II-V fingers in both hands. The left hand (4th finger) had been already operated in another centre as a Dupuytren disease with postoperative complications and severe progression of the contracture. History taking and a detailed clinical examination drew hand surgeons attention to contractures of both feet, complains of recurrent headaches, nystagmus and an unusual forced position of the head - intensifying the progeria of the mandibula. A neurosurgical reevaluation of the past head scans confirmed the Arnold Chiari Malformation Type I with a severe inclination of the cerebellum tonsils into the foramen magnum and dens axis compression of the pons. The girl was qualified for an urgent neurosurgical intervention.
The girl underwent craniocervical decompression with a posterior occipital stabilisation with no complication. In an 8-month follow-up, there was a significant clinical improvement with
The patient was qualified to following hand procedures: first collagenase injection into the overgrown aponeurosis in the II-IV fingers of the right hand. Later under the general anaesthesia contractures of the right-hand were stretched reaching the full range of motion. At the same time, an open fasciectomy with a tendon reconstruction-n was performed to the III and IV fingers of the left hand. The wound healing was uncomplicated and the patient continued rehabilitation improving significantly everyday activities.
A wide genetic testing confirmed multiple mutations linked with the rare disease also including collagenopathies, but no clear diagnosis was stated.
Results and Conclusions: The collagenase injection was a successful treatment option in this rare case. What's more the unknown course of the disease favour the minimally invasive methods.
In conclusion, the treatment option for finger contractures has to be adjusted individually. A team approach in the Pediatric Hand Surgery can be an example of a holistic treatment to a young patient that can benefit from the most up-to-date methods of treatment.
