Journal of Plastic, Reconstructive & Aesthetic Surgery (2012) 65, 956e959

CASE REPORT

Craniofacial neurofibromatosis and tissueexpansion: Long-term results

Dhruv Singhal, Yi-Chieh Chen, Rimante Seselgyte, Philip Kuo-Ting Chen,Yu-Ray Chen*

Chang Gung Craniofacial Center, Department of Plastic and Reconstructive Surgery, Chang Gung Memorial Hospital,5, Fu-Hsin Street, Kwei-Shan, Taoyuan 333, Taiwan, ROC

Received 3 November 2011; accepted 10 November 2011

KEYWORDSCraniofacialneurofibromatosis;Tissue expansion;Radical excision

* Corresponding author. Fax: þ886 8E-mail address: uraychen@cgmh.o

1748-6815/$ - see front matterª 2011 Pdoi:10.1016/j.bjps.2011.11.034

Summary The role of tissue expansion for coverage of soft-tissue defects of the facefollowing neurofibroma resection has been briefly described previously with good results butlimited follow-up. Recent scientific evidence has arisen that neurofibromas adversely affectthe rheological properties of normal surrounding skin resulting in hyper-extensibility of dermalelements. In this context, we believe that long-term outcomes of tissue expansion for treat-ment of soft-tissue defects in patients with craniofacial neurofibromatosis would demonstratesuboptimal results because of loss of contour. Between June 1981 and June 2011, two patientsunderwent five tissue expander placements during the course of treatment for craniofacialneurofibromatosis at our institution. In both patients, tissue expansion was used to recruittissue for fascio-cutaneous flaps following radical excision of adjacent neurofibromas. Threecervical expansions were performed for cheek and lateral face recontouring. One foreheadexpansion was used for nasal reconstruction and a second forehead expansion was performedfor temporo-orbital recontouring. No perioperative complications occurred related to thetissue expander placement, expansions or radical excisions. Photographic follow-up of 15 yearsis available in each case. Cervical tissue expanded to provide cheek coverage demonstratedearly loss of contour. Loss of lateral cheek contour resulted in increased morbidity from lateraland caudal displacement of the mid-face and orbital tissues requiring multiple revisionalprocedures. Tissue expansion of the forehead for both nasal reconstruction and temporo-orbital reconstruction demonstrated excellent long-lasting results.ª 2011 Published by Elsevier Ltd on behalf of British Association of Plastic, Reconstructive andAesthetic Surgeons.

66 3 3271029.rg.tw (Y.-R. Chen).

ublished by Elsevier Ltd on behalf of British Association of Plastic, Reconstructive and Aesthetic Surgeons.

Craniofacial neurofibromatosis and tissue expansion 957

Tissue expansion is a well-described method of head andneck reconstruction following cancer extirpation, resectionof congenital lesions, burn scar and traumatic scarexcision.1e4 Only one report is available in the literaturethat specifically evaluates the role of tissue expansion inthe treatment of soft-tissue deformities following tumourexcision for craniofacial neurofibromatosis. Krastinova-Lolov et al. describe the utility of tissue expansion fortwo patients with cranio-orbital neurofibromatosis wherecheek expanded tissue was used to replace the effectedparietal region. One-year follow-up pictures of one patientdemonstrated good effect.5 However, no long-term effectsof tissue expansion in craniofacial neurofibromatosis havebeen previously reported. Skin changes in tissue expansion,such as thickening of the epidermis and thinning of thedermis, have been well-described. However, neurofibromasadversely affect the inherent rheological properties of skin.Clinical observation of neurofibroma patients revealsthickening of the dermis and subdermal tissues and frag-mentation of elastic fibres of the dermis. More recent basicscience research confirms that neurofibromas, and theirapparently normal surrounding skin, demonstrate a signifi-cant difference in irreversible hyper-extensibilitycompared to controls.6 We reviewed our experience withtissue expansion and craniofacial neurofibromatosis toclarify its role in the treatment of this condition.

As facial neurofibromatosis can present and evolve inmany different and unpredictable ways, a single approachto this disease process is elusive. The most common surgicalapproach is conservative management where masses thatare functionally impairing or cosmetically deforming areremoved without sacrifice of functional nerves.7 Ulti-mately, this approach leads to multiple excisions over manyyears without eradication of the disease and often marginalimprovement of both form and function.5,7 Other surgeonsargue for a more aggressive management protocol with thegoal of near-total excision.8 In such cases, the surgeon mustaddress the resulting soft-tissue defect. Skin grafting has

Figure 1 Case 1. (Left) The patient at 12 years of age with extcharacteristic hyperpigmentation. (Middle) The patient is now onereconstructed with a previously expanded cervico-facial fasciocutathe lateral cheek area with preservation of the position of criticcommissure. (Right) At 15 years follow-up, the patient demonstratcanthus. Over the past 3 years, multiple attempts to repositionsuspension of oral commissure, wedge excision of lip, and two late

been previously described, though preservation of criticalmuscles such as the masseter and temporalis is critical forappropriate bone coverage.9 The use of free flaps forreplacement of significant soft-tissue defects in bothneurofibromatosis and neurofibrosarcomas has also beenpreviously described.10,11

We review below our experience with two patients whounderwent tissue expansion during their treatment forcraniofacial neurofibromatosis.

Case 1

A 12-year-old male presented with craniofacial neurofi-bromatosis with extensive soft-tissue involvement of thecheek, orbit, hard palate, nose, temporal fossa, scalp andparapharyngeal space. In preparation for radical excision ofthe cheek, the patient underwent placement and expan-sion of a 550-cc quadrangular expander in the supra-platysmal plane of the right cervical region. Followingradical excision of the right cheek subunit, a cervicalrotational flap was mobilised for closure. Histologyconfirmed the diagnosis of plexiform neurofibromatosis. Noperioperative complications were noted. Early post-operative follow-up demonstrated improvement of cheekcontour and mid-face positioning. However, follow-up overthe next 15 years demonstrated progressive laxity of thetissue with significant displacement of the mid-face andperiorbital tissues. Multiple attempts to reposition the oralcommissure and lateral canthus (fascia lata graft suspen-sion of oral commissure to temporal fascia, wedge excisionof lip and two lateral canthopexies) over the past 3 yearshave resulted in suboptimal improvements (Figure 1).

Case 2

A 16-year-old female with craniofacial neurofibromatosisaffecting the orbit, hard palate, temporal and parietal scalp,

ensive neurofibroma involvement of the right cheek. Note themonth following radical excision of the right cheek soft-tissuesnous flap. The patient demonstrates improved appearance ofal mid-face structures including the right alar base and orales descent of the right alar base, oral commissure, and lateralthe oral commissure and lateral canthus (fascia lata graftral canthopexies) have demonstrated minimal improvement.

958 D. Singhal et al.

and right parapharyngeal space presented to our Center. Thepatient underwent tissue expansion of the forehead in thesubmuscular plane with a 500-cc crescentic expander and inthe supraplatysmal plane of the right neck with a 680-ccquadrangularexpander. Thepatient subsequentlyunderwentradical excision of the nasal and cheek subunits withexpanded tissue flap closure. Repeat expansion of the rightlateral forehead (600-cc crescentic expander) and cervicalregion (680-cc quadrangular expander) 1 year later was fol-lowed by temporo-orbital and further lateral cheek radicalexcisions with repeat rotational flap closures. The patient’shistologyconfirmedplexiformneurofibromatosis. Therewereno perioperative complications. Long-term follow-updemonstrated excellent contour following reconstruction ofthe nasal and temporo-orbital regions from the foreheadexpansions. However, lack of lateral cheek support resultedin commissure displacement with attempts at lip reductionproving unsuccessful at repositioning the commissure at 7years follow-up. More recently, the patient underwentextensive lip reduction procedures coupled with cheeksuspension to re-establish thecommissureposition (Figure2).

Figure 2 Case 2. (Top Left) The patient at the age of 15 withsevere neurofibroma involvement of the cheek, temporal, andnasal structures. (Top Right) Over one-year, the patientunderwent sequential expansions of the forehead and neck toprovide coverage following radical excisions and recontouringof the right temporo-orbital, cheek, and nasal regions. Thepatient is shown 7 months following her second reconstruction.Note the marked improvement in nasal and temporo-orbitalcontour. (Bottom Left) 7-year follow-up. (Bottom Right) 15-year follow-up. Note the excellent maintainence of contourof the nasal and temporo-orbital regions. The patient’s rightoral commissure, on the contrary, continued to descend andultimately required multiple cheek suspensions to the orbitalrim and aggressive labial excisions to obtain the final result.

Discussion

A review of our 30-year experience with the treatment ofcraniofacial neurofibromatosis reveals a distinct change inmanagement. Progression of disease with limited or nointervention coupled with early experience with partialexcision demonstrating the need for multiple re-operationsled to more aggressive resections of facial neurofibromas.Moreover, the more radical excisions required medial inci-sions along the alar base and oral commissure to allow forrepositioning of these critical structures and subsequentlyimproved facial harmony and aesthetic result.12 However,the next challenge was coverage of these large soft-tissuedefects. In an attempt to provide coverage with ‘liketissue’ of similar colour, we initially attempted local tissueexpansion followed by radical excision and rotational flaps.

While we were able to provide adequate coverage of allthe soft-tissue defects, we have observed that the expandedtissue in craniofacial neurofibromatosis patients revealsearly loss of contour if sufficient skeletal or cartilaginoussupport is not available for the rotated tissue. Foreheadexpansions for nasal reconstruction and temporo-orbitalreconstruction demonstrated excellent results 15 yearslater. The nasal reconstruction is supported by the nasalbones and alar cartilages to preserve the appropriatecontour. Similarly, we observe that the zygoma is an impor-tant support structure for the temporo-orbital region andtherefore this area responded favourably to tissue expansionreconstruction. However, the cheek subunit lacks anystructural support to counter the effects of gravity, therebyleading to early loss of contour. In fact, both cases demon-strated early cheek tissue sagging following reconstruction.Loss of cheek soft-tissue support laterally is critical as thisleads to secondarydisplacementof themid-face, specificallythe nasal and labial subunits, and attempts to re-suspendthese structures are especially challenging due to the hyper-elasticity of the lateral tissue. The inherent hyper-extensibility of the skin at the excision margins in craniofa-cial neurofibromatosis patients underscores the importance

of ensuring that adequate support is available at the recip-ient site.

We have transitioned away from tissue expansion forcoverage of these large soft-tissue cheek defects. Over thepast decade, we have attempted free flap reconstructionsusing the anterior lateral thigh flap, which have demon-strated better long-term results. However, we believe thatthe reconstructive technique that best maintains facialharmony in regions where no structural support is availableis the use of full-thickness skin grafts.9 Unlike radicalcancer extirpations, rarely is bone exposed during theseresections allowing the use of skin grafts. Skin graftsgenerate more scar which help immobilise central

Craniofacial neurofibromatosis and tissue expansion 959

structures which are critical for the ultimate aestheticresult. Preserving contour trumps providing ‘like tissue’coverage in this disease process because of the associatedmorbidity with the latter. We now sacrifice soft-tissueasymmetry and colour to preserve the facial contourwhen reconstructing the lateral cheek defect.

Conclusion

The fourth dimension, time, is invaluable in evaluatingsurgical results especially in rare conditions such ascraniofacial neurofibromatosis. Critical evaluation of ourown results reveals that inherent hyper-extensibility of thedermis in patients with craniofacial neurofibromatosisrequires the reconstructive surgeon to ensure that excel-lent support is available for expanded tissues. Specifically,tissue expansion for upper facial structures, such as nasaland temporo-orbital reconstructions, will provide excellentlong-term results if the recipient site structural supportremains intact. Tissue expansion for coverage of the cheeksubunit, on the other hand, should be avoided because ofloss of contour with resulting mid-face deformity andincreased aesthetic morbidity.

Conflict of interest

None declared.

Funding statement

None declared.

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