Amin et al. Head & Neck Oncology 2010, 2:18
http://www.headandneckoncology.org/content/2/1/18
Open Access CASE REPORT
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Case report
Living related hemi-face skin transplant using
radial forearm free flap for a xeroderma
pigmentosa patient: early outcome
Ayman Amin*
1
, Mahmoud Bassiouny
1
, Kareem Sallam
1
, Galal Ghally
1
, Hanaa El-Karaksy
2
and Alaa El-Haddad
3
Abstract
Introduction: Xeroderma pigmentosa (XP) is a hereditary disease characterized by deficient repair of DNA damage
that occurred on exposure of the skin to ultraviolet irradiation. The affected children have a propensity to develop
multiple skin cancers mainly in the face and eventually die before the age of 20.
Hypothesis: Allograft replacement of facial skin by a healthy skin from normal person might decrease the incidence of
skin cancer development, the number of surgical procedures, and eventually might improve the survival of these
miserable patients.
Methods: As Cadaveric organs are unavailable in our country. After approval from the ethical committee, confirmed
agreement of the donor and the patient's guardian, a radial forearm free flap was transplanted from an ABO
compatible mother to her 5 year old daughter with XP. The mother had an older daughter died from the same disease
at the age of 14. The flap replaced skin of the hemi face that developed precancerous lesions. The girl was kept on
adjusted doses of immunosuppressive drugs.
Results: The flap survived, wounds healed uneventfully. The flap developed a reddish spot one and half month
following transplant where baseline skin biopsy was taken. In the fifth months the girl presented with bad non
salvageable rejection that ended up loosing the flap. On long term follow up, the girl started to develop skin lesion on
the virgin half of the face. Our early cosmetic result replacing half of the facial skin was very promising. In addition the
girl did not develop skin lesions in the operated site.
Conclusion: Our early cosmetic result was very promising. In addition to this, the girl did not develop skin lesions in
the operated side of the face
Introduction
Xeroderma pigmentosa (XP) is an autosomal recessive
genetic disorder that makes the DNA of the skin unable
to repair the continuous damage inflicted on it by the
Ultra Violet (UV) rays present in sun rays [1]. A dominant
form of XP was described in a Scottish girl; these patients
have a mild clinical course [2]. The disease is character-
ized by photosensitivity, pigmentary changes, premature
skin ageing, neoplasia and abnormal DNA repair. Some
patients also have neurological complications. Affected
individuals are 1000 times more prone to UV induced
skin cancer than unaffected ones and over 90% of affected
individuals will develop skin malignancy before the end
of their second decade [3]. Development of multiple
recurrent skin malignancy is the eventual outcome of this
DNA repair failure which world-wide kills two thirds of
the affected subjects before the age of 20. Death follows a
lengthy devastating illness for both the patient and his
family where malignant lesions can develop as early as the
third or fourth year [2]. During their life span they get
exposed to repeated surgical resection of newly-devel-
oped skin cancers. Surgical resection ranges from simple
excision and skin closure to heroic cranio-facial resection
and free flap reconstruction.
In a desperate attempt to prevent malignant transfor-
mation, affected individuals have to be kept away from
sun light through out there life. There is no definitive
* Correspondence: aymanamin@hotmail.com
1
Surgical oncology department, national cancer instituite, Cairo, Egypt
Full list of author information is available at the end of the article