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CC BY 4.0 · Arch Plast Surg 2023; 50(06): 573-577
DOI: 10.1055/s-0043-1771520
Hand/Peripheral Nerve
Case Report

Surgical Management of Pachyonychia Congenita in a 3-Year-Old

Jack D. Sudduth 
1   Division of Plastic Surgery, Department of Surgery, The University of Utah Hospital, Salt Lake City, Utah
,
Christopher Clinker 
1   Division of Plastic Surgery, Department of Surgery, The University of Utah Hospital, Salt Lake City, Utah
,
Matthew Holdaway 
1   Division of Plastic Surgery, Department of Surgery, The University of Utah Hospital, Salt Lake City, Utah
,
Jessica L. Marquez 
1   Division of Plastic Surgery, Department of Surgery, The University of Utah Hospital, Salt Lake City, Utah
,
Jacob Veith 
1   Division of Plastic Surgery, Department of Surgery, The University of Utah Hospital, Salt Lake City, Utah
,
Thomas Wright 
1   Division of Plastic Surgery, Department of Surgery, The University of Utah Hospital, Salt Lake City, Utah
,
W. Bradford Rockwell 
1   Division of Plastic Surgery, Department of Surgery, The University of Utah Hospital, Salt Lake City, Utah
› Author Affiliations Funding None.
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Abstract

Pachyonychia congenita is a rare genetic disorder characterized by hypertrophic nail plates, hyperkeratotic nail beds, and thickened hyponychium of the fingers and toes, impairing manual dexterity and resulting in poor aesthetics. The current body of literature describes various treatment modalities, but no singular approach has been defined as the gold standard. In this case, the authors employed different surgical techniques for treating pachyonychia congenita to evaluate the most effective approach. A 3-year-old boy presented with hypertrophic nail growth involving all digits of both hands and feet. Three surgical procedures were performed on the patient's fingers and toes using germinal matrix excision (GME) alone, GME plus partial sterile matrix excision (pSME), or GME plus complete sterile matrix excision (cSME). The digits treated with GME + cSME exhibited no recurrence of nail growth. Those treated with GME alone exhibited recurrence of hypertrophic nail growth, although their growth slowed. Excision of GME + cSME prevented recurrence of hypertrophic nails, while GME alone or with pSME led to slower-growing hypertrophic nails. Complete excision of the germinal and sterile matrices with skin graft closure may be a definitive treatment for pachyonychia congenita, but further studies are needed to validate these findings.

Keywords

pachyonychia congenita - germinal matrix excision - nail bed excision - surgical dermatology - congenital

Authors' Contributions

All authors contributed equally to the concept, writing, and submission process of this manuscript.


Ethical Approval

This project was deemed exempt from IRB approval per local institution guidelines.


Patient Consent

Written patient consent was obtained for this study.




Publication History

Received: 03 November 2022

Accepted: 15 June 2023

Article published online:
21 December 2023

© 2023. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

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