INTRODUCTION
Juvenile xanthogranuloma (JXG) is a benign tumor of unknown etiology formed of histiocytes
and included in the group of non-Langerhans histiocytic proliferative diseases1,2.
It is a rare condition, and although its incidence varies in the literature, it is
usually underestimated because it spontaneously regresses in some cases with many
lesions not being biopsied for diagnostic definition3,4,5.
Literature classifies lesions; 2 cm as giant JXG4; these occur more frequently in children aged 3-5 years, presenting an almost equal
distribution between the sexes. The single skin lesions are the most common, occurring
predominantly in the head and neck, followed by the trunk and limbs6,7.
JXG lesions tend to regress spontaneously within 6 months to 3 years after their first
appearance8. However, in cases of giant lesions, whose involution is less common, surgical treatment
may be indicated when the lesions cause significant functional and/or aesthetic impairment9,10.
CASE REPORT
An 8-year-old male patient was referred to the service after the excision of a 4 cm
× 6 cm occipital skin lesion in which anatomopathological examination revealed JXG.
On inspection, yellowish papular lesions were simultaneously affecting the four eyelids
in addition to bilateral lesions in the middle third of the face. According to his
father, the lesions appeared after 6 months of life and had increased progressively
since (Figure 1).
Figure 1 - A-D. Bilateral lesions affecting the upper and lower eyelids and the middle third of
the face.
Figure 1 - A-D. Bilateral lesions affecting the upper and lower eyelids and the middle third of
the face.
Due to diffuse periorbital involvement, without signs of lesion regression, surgical
treatment was chosen with the proposal of lesion excision followed by primary closure
or skin grafting, doing also local miocutnaeous flaps when the defects did not allow
primary closure. The medical team opted to perform the resections in different surgical
stages for each side.
Left upper eyelid
During dissection, this lesion, which apparently was superficial and sized 30 mm ×
7 mm, was actually extending to the frontal region, reaching deep planes and invading
the orbit, compromising the entire upper orbital cavity except for the aponeurosis
of the levator palpebrae superioris muscle.
After complete lesion resection, the frontal region was detached and advanced to cover
the upper orbital edge, and a full- thickness skin graft (retroauricular) was placed
directly on the aponeurosis of the levator palpebrae superioris muscle, with 100%
integration after 5 days (Figure 2).
Figure 2 - A-C. Resection of the left upper eyelid lesion; D. Resected lesion; E. Defect created after resection; F. Full-thickness graft.
Figure 2 - A-C. Resection of the left upper eyelid lesion; D. Resected lesion; E. Defect created after resection; F. Full-thickness graft.
Left lower eyelid/left middle third
After 3 months, the lesion in the lower eyelid allowed resection and primary closure.
On the other hand, the lesion in the left middle third of the face measured 17 mm
× 7 mm and extended to deep planes, reaching the periosteum of the maxilla and affecting
the infraorbital nerve, which required en bloc resection with the lesion. After detachment
of the malar tissue, the middle third was advanced for primary closure (Figure 3).
Figure 3 - A. Pre-operative view; B. Resection of the lesion, left middle third; C. Resected right upper eyelid lesion; D. Immediate postoperative view of a transposition flap for reconstruction of the right
lower eyelid.
Figure 3 - A. Pre-operative view; B. Resection of the lesion, left middle third; C. Resected right upper eyelid lesion; D. Immediate postoperative view of a transposition flap for reconstruction of the right
lower eyelid.
Right upper eyelid
In the same moment of the left lower eyelid resection, this lesion measured approximately
25 mm × 10 mm and extended to the orbital septum; its strip resection allowed primary
closure (Figure 3).
Right lower eyelid
In a 3 rd surgery, after also 3 months, this pretarsal lesion was close to the medial
corner, measuring 10 mm × 4 mm, with primary closure impossible after resection. A
paranasal transposition flap combined with a superomedial pedicle was used to ensure
tension-free closure (Figure 3).
Right middle third
In the same procedure, this superficial lesion, measuring 17 mm × 7 mm, was excised
and closed primarily. (Figure 3). Postoperative evolution.
Postoperative evolution
All lesions were sent for anatomopathological examination, which confirmed the diagnosis
of JXG. No visual or palpebral motor dysfunction was observed in the postoperative
period, but medium-term follow-up showed the need for volume correction of the left
upper eyelid. The patient underwent four autogenous fat- -grafting procedures in this
region (at 6-month intervals), the first using fat strips and the other three using
grafted fat, which improved the aesthetic appearance (Figure 4).
Figure 4 - A. Incision below the eyebrow; B. Removal of the grafted skin area and the fat strip shown above; C. Grafted fat strip; D. Fat grafting in the frontal region for volume improvement.
Figure 4 - A. Incision below the eyebrow; B. Removal of the grafted skin area and the fat strip shown above; C. Grafted fat strip; D. Fat grafting in the frontal region for volume improvement.
The medical team monitored the patient for 3 years from the first procedure to the
current day (Figure 5).
Figure 5 - A and C. Preoperative view; B and D. Three years postoperative view.
Figure 5 - A and C. Preoperative view; B and D. Three years postoperative view.
DISCUSSION
JXG is a rare benign skin lesion of unknown etiology. Arises as yellow or orange papulonodular
single or multiple lesions. Giant JXG, in turn, is characterized by a lesion; 2 cm3,4,5. It usually presents isolated or, in the case of multiple lesions, in distant sites4,5,6, which has drawn attention to this unusual case of diffuse periorbital involvement.
The diagnosis was confirmed histopathologically, with histopathological examination
showing dense histiocytic dermal infiltrate and Touton pathognomonic cells (multinucleated
giant cells, homogeneous eosinophilic cytoplasm, and peripheral xanthomatization)6. In this case, all pieces were analyzed, and the diagnostic hypothesis of JXG was
confirmed.
The literature shows that, when lesions similar to the present case do not involute
in the first years of life, they tend not to regress despite the use of intralesional
steroids4,5,7,9,10.
Surgical treatment was indicated in this case due to the persistence of the lesions
and the possible future complications (infraorbital growth with compression of local
structures and aesthetic impairment).
The surgical challenge, in this case, was the simultaneous involvement of all eyelids
and the middle third of the face, which defined the resection strategy for multiple
surgical times to ensure patient safety and better reconstruction results.
Resection of the lesion in left upper eyelid led to direct exposure of the aponeurosis
of the levator palpebrae superioris muscle. Full-thickness skin grafts placed directly
on the aponeurosis of the levator palpebrae superioris proved to be a viable tactical
option in cases in which more morbid reconstructive options must be avoided. Although
no study reported this maneuver, no impairment of the upper eyelid suspension movement
was observed in the medium or long term. Autogenous fat grafts were used to improve
the fat volume, although they were small.
It is important to emphasize that the complexity involving the fat grafting in this
upper eyelid was due to the thin skin thickness and local fibrosis.
The other lesions were primarily closed or submitted to local miocutaneous flaps.
It should be emphasized that the left malar lesion was deeply involved and required
en bloc resection of the left infraorbital nerve. .
It is important to be aware that these situations can happen during intraoperative
time and surgeons must have tools to solve them, even if they are simple but not common,
like the skin grafting on the aponeurosis of the levator palpebrae superioris muscle,
as did in this case.
CONCLUSION
The reviewed literature corroborates our finding that apparently superficial lesions
may infiltrate deeply into the neighboring tissues, with great adhesion and significant
bleeding if the dissection plane is inadequate5,6,9,10.
The medical team must be prepared for situations that can arise in the intraoperative
period that require the use of unusual options such as skin grafting directly on the
aponeurosis of the levator palpebrae superioris muscle.
COLLABORATIONS
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HF
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Final manuscript approval, Project Administration, Supervision, Writing - Original
Draft Preparation, Writing - Review & Editing
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AHV
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Conceptualization, Investigation, Realization of operations and/or trials, Writing
- Review & Editing
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RCL
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Conceptualization, Data Curation, Project Administration, Realization of operations
and/or trials, Writing - Original Draft Preparation, Writing - Review & Editing
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VAM
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Conceptualization, Realization of operations and/or trials, Writing - Review & Editing
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MNSJ
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Data Curation, Methodology, Realization of operations and/or trials, Writing - Original
Draft Preparation
|
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MBS
|
Conceptualization, Investigation, Realization of operations and/or trials, Writing
- Review & Editing
|
|
RG
|
Supervision, Validation, Visualization, Writing - Review & Editing
|
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1. Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo,
SP, Brazil.
Corresponding author: Rodolfo Costa Lobato Rua da Consolação, 3741, 11º andar, Cerqueira César, São Paulo, SP, Brazil. Zip code:
01416-001. E-mail: rodolfolobato49@yahoo.com.br
Article received: January 11, 2019.
Article accepted: April 18, 2019.
Conflicts of interest: none.
