Remodelling the upper eyelid in the management of orbitopalpebral neurofibromatosis  Daniel Marchac, Jonathan A. Britto  British Journal of Plastic Surgery  Volume 58, Issue 7, Pages 944-956 (October 2005) DOI: 10.1016/j.bjps.2005.04.019 Copyright © 2005 The British Association of Plastic Surgeons Terms and Conditions

Figure 1 Operative markings for the right upper eyelid reduction using the transverse oblique approach. The transverse extent is marked between the position of the supra-tarsal fold above the eyelid margin and below the eyebrow compared to the normal side (white arrows, between a and b). The lateral extent is made oblique and continuous with a full thickness wedge of the excess upper lid margin (yellow lines, ‘x’ plus lateral extent). British Journal of Plastic Surgery 2005 58, 944-956DOI: (10.1016/j.bjps.2005.04.019) Copyright © 2005 The British Association of Plastic Surgeons Terms and Conditions

Figure 2 Case 1 at presentation with bilateral upper eyelid neurofibroma (A). The P3C1D1 score is improved to P1C0D0 at 3 months and 1 year (B), (C). The operative markings and series are shown in Fig. 3(A)–(C). British Journal of Plastic Surgery 2005 58, 944-956DOI: (10.1016/j.bjps.2005.04.019) Copyright © 2005 The British Association of Plastic Surgeons Terms and Conditions

Figure 3 The transverse oblique excision is marked (Fig. 1 and text), with a temporal extension to debulk the lateral disease (A). The neurofibroma tissue is identified with the characteristic ‘bag of worms’ appearance (B). The closure is achieved with a lateral canthopexy and levator reconstruction (C). British Journal of Plastic Surgery 2005 58, 944-956DOI: (10.1016/j.bjps.2005.04.019) Copyright © 2005 The British Association of Plastic Surgeons Terms and Conditions

Figure 4 This 2-year-old girl (Case 2) presents with left-sided mild upper eyelid neurofibromatosis (A), which deteriorates at 2 years later (B), (C). The operative series is shown with markings (D), dissection as transverse oblique wedge (E), and layered closure with canthopexy (F). The 4-month result is shown (G). British Journal of Plastic Surgery 2005 58, 944-956DOI: (10.1016/j.bjps.2005.04.019) Copyright © 2005 The British Association of Plastic Surgeons Terms and Conditions

Figure 5 This 4-year-old Algerian boy (Case 3) presented with right sided (A) orbital expansion and exorbitism (P5 C2 D1). A combined transcranial remodelling and palpebral remodelling was undertaken with immediate levator reconstruction. At 2 years follow-up the child maintained a good visual axis, with weak levator function and good orbicularis oculi sphincter function (B), (C). British Journal of Plastic Surgery 2005 58, 944-956DOI: (10.1016/j.bjps.2005.04.019) Copyright © 2005 The British Association of Plastic Surgeons Terms and Conditions

Figure 6 This 13-year-old girl (Case 4) presented with mild palpebral but aggressive orbital neurofibromatosis with severe canthal and orbital malposition (P1 C2 D0). She underwent transcranial orbital surgery, with canthopexies and upper eyelid remodelling as described above. She maintained a good result (P1 C0 D0) at 1 year follow-up (A), (B). British Journal of Plastic Surgery 2005 58, 944-956DOI: (10.1016/j.bjps.2005.04.019) Copyright © 2005 The British Association of Plastic Surgeons Terms and Conditions

Figure 7 This 6-year-old girl (Case 5) presented initially with severe right upper eyelid neurofibromatosis with canthal descent and mild orbital dysplasia (P5 C2 D1) (A), (B). Axial CT scan indicates globe involvement with buphthalmos, proptosis, and orbital dysplasia (C). British Journal of Plastic Surgery 2005 58, 944-956DOI: (10.1016/j.bjps.2005.04.019) Copyright © 2005 The British Association of Plastic Surgeons Terms and Conditions

Figure 8 Operative series for Case 5. The tansverse ellipse is marked out with a temporal extension, and the tumour is displayed and debulked. The levator mechanism is identified and divided, and the eyelid margin retained as an axial pattern flap. Closure is in layers with levator repair and lateral canthal reconstruction. British Journal of Plastic Surgery 2005 58, 944-956DOI: (10.1016/j.bjps.2005.04.019) Copyright © 2005 The British Association of Plastic Surgeons Terms and Conditions

Figure 9 Operative results for Case 5. Upper figure (A) shows postoperative 3-month result of palpebral remodelling without orbital or medial canthal surgery. The lower figure (B) shows the result 2 years following orbital and medial canthal surgery, without further palpebral remodelling. British Journal of Plastic Surgery 2005 58, 944-956DOI: (10.1016/j.bjps.2005.04.019) Copyright © 2005 The British Association of Plastic Surgeons Terms and Conditions

Figure 10 Case 6, showing pre-operative palpebral asymmetry at the age of 10 (A) and the CT confirmation of orbital dysplasia with proptosis (B). The operative series is shown (C)–(E) and the postoperative result at 3 months (F). British Journal of Plastic Surgery 2005 58, 944-956DOI: (10.1016/j.bjps.2005.04.019) Copyright © 2005 The British Association of Plastic Surgeons Terms and Conditions

Figure 11 Case 7, at the age of 10 years with right upper lid neurofibromatosis in positions of neutral gaze and upgaze (A), (B). At 1 year post surgery, he has a restoration of the visual axis and recovery of residual motor function is acceptable in the remodelled eyelid (C), (D). After subsequent orbital remodelling, orbital floor bone graft, and medial canthal revision, he has acceptable result a 1 year post secondary surgery. British Journal of Plastic Surgery 2005 58, 944-956DOI: (10.1016/j.bjps.2005.04.019) Copyright © 2005 The British Association of Plastic Surgeons Terms and Conditions