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Year : 2021  |  Volume : 8  |  Issue : 1  |  Page : 14-20

Composite axial eyelid flap in the surgical management of orbitopalpebral neurofibromatosis

Department of Cleft and Maxillofacial Surgery, BSES MG Hospital, Mumbai, Maharashtra, India

Date of Submission06-Dec-2019
Date of Acceptance13-Jul-2020
Date of Web Publication13-Jan-2021

Correspondence Address:
Dr. Megha Shah
BSES MG Hospital, Mumbai - 400 058, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jclpca.jclpca_32_19

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Background: Orbitopalpebral neurofibroma is a progressive and debilitating disease with the potential of affecting the orbital, temporal and facial region. Lack of capsule and its infiltrative nature yields to difficulty in complete removal. Aims and Objectives: In this interventional case study, nine patients between the ages of 12–30 years diagnosed with orbitopalpebral neurofibromatosis underwent palpebral remodeling with composite axial eyelid flap for esthetic purpose and alleviation of visual impediment. Material and Methods: The clinical course for every individual was scored using Ptosis, Canthal involvement, and Oculopalpebral Diastasis (PCD) score on regular follow-ups. Results: For majority of the cases, an improvement was noted on the PCD scale. Conclusion: This series demonstrates that a tailored approach to each individual helps in attaining a balance between function and esthetic outcomes.

Keywords: Axial eyelid flap, neurofibromatosis, orbitopalpebral

How to cite this article:
Ladani P, Sailer HF, Shah M, Shetye A. Composite axial eyelid flap in the surgical management of orbitopalpebral neurofibromatosis. J Cleft Lip Palate Craniofac Anomal 2021;8:14-20

How to cite this URL:
Ladani P, Sailer HF, Shah M, Shetye A. Composite axial eyelid flap in the surgical management of orbitopalpebral neurofibromatosis. J Cleft Lip Palate Craniofac Anomal [serial online] 2021 [cited 2021 Jun 18];8:14-20. Available from: https://www.jclpca.org/text.asp?2021/8/1/14/295903

  Introduction Top

Neurofibromatosis (NF) is a phakomatosis with multiple neurofibromas that can affect any organ or region.[1] NF Type I (NF1) is an inherited disorder, acquired via autosomal dominant genes, with a frequency of 1 in 3000–4000[2] live births. In the published literature, head-and-neck cases account for 1% to 22%[3],[4] of the total cases. When the head is affected, it may produce cosmetic disfigurements such as hemifacial hypertrophy, bony skeleton enlargement with erosion and defect formation, and pendulous swellings.[5]

The rapid growth of various cellular elements in peripheral nerves makes plexiform neurofibromas, the most common tumor arising in the orbit from the peripheral nerves.[6] Orbitopalpebral NF is characterized by the distorting neurofibromas affecting the orbital, temporal, and facial region which has the potential to be progressive and debilitating.[7] The palpebra may be deformed to a mild degree as a manifestation of peripheral systemic NF1, or more severely, as a component of a specific segmental orbitotemporal NF (OTV). The NF1 is a systemic presentation of NF, with signs such as multiple cafe-au-lait skin lesions, cutaneous neurofibromas, iris hamartomas, and peripheral subcutaneous plexiform neuromas.

Clinical findings of NF include ablation of the greater wing of sphenoid, hypoglobus, enophthalmos, globe malposition, increased orbital volume loss of conjugate vision, canthal malposition, and direct involvement of the globe (causing buphthalmos).[8],[9],[10],[11],[12] There may be inclusion of all layers of the palpebral complex, manifesting grossly as a mass of white, round, or elongated neurofibromata relating the so-called “bag of worms.” These are multiple neuromatous units, wherein each unit is enclosed by a distinct perineural sheath.[9],[13] The ptosis and swelling of the affected eyelid cloud the visual axis. The lacrimal gland may be replaced by disease. The levator mechanism may likewise be undermined, which affects the esthetic outcome as well[14] as functional rehabilitaion of the upper eyelid, making levator reconstruction indispensable.[8],[9],[11],[12],[15] Plexiform neurofibromas often have a lateral extent into the temporal region inclusive of the lateral face.[9]

The normal distance of the supratarsal crease from the upper lid margin is 9–10 mm in female and 7–8 mm in male in the Caucasian ethnicity. Asians have varied appearance of the fold comparatively, which is usually lower by 2–3 mm, with a large superior palpebral fold hanging over the pretarsal skin. The position where orbital septum fuses with levator aponeurosis to skin determines the level of crease formation.[9],[16]

An absence of capsule around the tumor coupled with its infiltrative nature owing to the intertwining bundles, surgical removal in toto is difficult. Surgical guidelines have been laid down to manage orbital dysplasia; however, the optimism for eyelid remodeling is lacking in general. Further affecting its complete removal is the voluminous hemorrhage and destruction of the surrounding tissue and high recurrence rate. The lack of encouraging cosmetic results further does not aid it. Various methods have been proposed to manage this disease with variable outcomes.[17],[18],[19]

The principle we have utilized is removing full thickness, “en bloc,” the excess of the diseased upper eyelid, with immediate levator palpebrae aponeurosis re-insertion, and reconstruction of the lateral canthus. In a series representing nine patients with orbitopalpebral NF, we performed full-thickness transverse or transverse-oblique reduction of upper eyelid neurofibroma, followed by levator reconstruction utilising a composite axial-pattern flap from the length of preserved upper eyelid margin, vascularized by the medial origin of the marginal palpebral artery. Canthopexies were performed as required, to achieve an esthetically acceptable, functional upper eyelid.

  Materials and Methods Top

In this interventional case series, nine patients with histologically confirmed diagnosis of NF [Figure 1], who underwent palpebral remodeling surgery at BSES MG Hospital, Department of Swiss Cleft and Craniofacial Surgery, in a 5-year period (2007–2012) were studied. The patients' details and history were recorded. Clinical findings are reported in [Table 1]. The severity of eyelid and orbital involvement were grouped according to the Jackson classification[8] and also graded according to the Ptosis, Canthal involvement, and Oculopalpebral Diastasis (PCD) scale both pre- and postoperatively on clinical photographs.[9] PCD scale is a grading system to help determine the severity of PCD [Table 2].[9] This system makes it is easier to distinguish between the pre- and postoperative status of the eyelid.[2]
Figure 1: Preoperative photo

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Table 1: Demographics of the study patients

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For remodeling, the contralateral normal eyelid serves as a template. The width of the normal upper eyelid/palpebral aperture is noted from medial to lateral canthus which is then transferred on the upper and lower eyelids on the diseased eyelid. If a difference is noted, the lateral canthus position is marked to reflect the position of the remodeled lateral canthus from the medial canthus.[1] The normal recordings of the position of the supratarsal crease on the normal eyelid along with its distance from normal eyelid margin and normal eyebrow are noted at the medial, mid, and lateral points. This serves as a blueprint which is transferred on the diseased side, which usually has an excess tissue both in terms of vertical height of lid and horizontal extent of palpebral aperture. The amount of excess tissue in vertical dimension is determined first by marking the position of reconstructed supratarsal crease measuring the distance from the eyelid margin in a curvilinear line, mirroring the normal eyelid according to the measurements. This line also marks the lower excision margin of the planned full-thickness excision. A second curvilinear line is marked below the eyebrow matching the measurements of normal side between the supratarsal crease and eyebrow, which also marks the upper excision margin. A full-thickness transverse wedge incision is made between these two curvilinear lines with preservation of the levator [Figure 2]. In most cases, temporal excision of diseased tissue is noted along the involved distended eyelid, which sags laterally to cause lateral canthal displacement, a wide palpebral aperture, and lid/globe malposition. When disease extends laterally, transverse oblique modification is performed to dissect and remove in full thickness the excess lateral tissue. This wedge is in continuation with the full-thickness upper eyelid wedge. This aids simultaneous reconstruction of the normal palpebral aperture [Figure 3]. The eyelid margin of the affected eyelid has been vertically positioned in a way to correspond to the width of the contralateral normal palpebral aperture. At the position of the projected lateral canthus, full-thickness excision of the diseased upper eyelid margin is performed. Thus, a full-thickness excision of the excess lateral eyelid is continued through the tarsus in a craniomedial direction, merging into the inferior part of the transverse wedge excision of the upper eyelid. The vascularity is maintained by the medial origin of the marginal palpebral artery, forming a composite axial-pattern flap retaining the eyelid margin, tarsus, tarsal insertion of levator palpebrae superioris, and the lower preseptal fibers of the orbicularis oculi muscle [Figure 4]. The excised tissue contains full-thickness upper eyelid, orientated transversely in its medial part and widened laterally to include the distended eyelid margin. More laterally, the excised tissue is mostly skin and diseased subcutaneous tissue, extending onto the temple as necessary.[1] To retain the option of secondary frontalis suspension in cases of recurrently ptotic lid, the preservation of the frontal branch of the facial nerve during primary surgery is important.
Figure 2: Markings done comparing the diseased eyelid with the normal side

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Figure 3: Markings showing the lateral extension of incision

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Figure 4: Preserved composite axial pattern flap after dissection

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In addition to the upper eyelid, lower eyelid shortening procedure was carried out in four cases to match the upper and lower eyelid length in the reconstructed palpebral aperture by performing pentagonal wedge excision and modified tarsal strip or periosteal flap canthoplasty. By approximation of the margins of the upper and lower eyelids with special care taken to approximate the gray lines and edges of the eyelid margin, the lateral canthus is reconstructed. A lateral canthoplasty is performed by attaching the lower eyelid tarsus to the inner periosteum of the superolateral orbital margin at an appropriate symmetrical position. No adjunctive canthoplasty procedures such as transosseous canthal fixation or lateral orbital periosteal strip were performed during the primary surgery. It was performed in six cases during the secondary correction. The palpebral part of the lacrimal gland was encountered in five cases and it was attached to the orbital margin. The lacrimal gland was also preserved unless it was invaded by disease, which was found in two cases. The medial canthal complex was mostly undisturbed. Hence, major orbital remodeling was not required. The surgery was individually tailored to each patient, reflecting the age of the patient and risk of recurrence, the extent of the disease, and the presence and quality of a protective Bell's reflex.[1] The upper eyelid is sutured in layers starting with the conjunctiva and the levator, usually thickened, before being re-inserted to its tarsal insertion with 6-0 Prolene. The levator closed with satisfactory tension without the need for levator shortening. Closure of the skin is achieved.

  Results Top

During the 5-year duration of the study, nine patients were included in the study who had a confirmed diagnosis of NF of the eyelids and/or orbit and underwent surgery for the same. The demographics of patients are shown in [Table 1]. Among the nine patients, four were male and five female with an age range from 12 to 30 years with an average of 20.11 years. In six patients, the right eye was involved, whereas in three patients, the left eye was involved. Three patients had a history of surgery to the same eye. Seven patients presented with no family history of NF, while two patients had at least one family member with a positive NF diagnosis. All patients displayed upper lid disease and four had a simultaneous lower lid involvement. All eyes displayed signs of conjunctival involvement, seven patients had involvement of the lacrimal gland, and six eyes had an orbital bone defect. Only four eyes had visible proptosis. Seven patients had temporal extension, one patient had extension over the forehead, while one patient had extension over the cheek. The visual acuity in the affected eye ranged from 6/12 to 6/32. The accompanying signs of NF noted in patients are café-au-lait spots in all patients, subcutaneous fibroma in four, and Lisch nodules in iris in 3 patients. No other systemic involvement was found in two patients besides the neurofibroma of the eyelids.

Patients were followed for 1–4 years, with an average of 1.98 years following surgery [Figure 5]. Three patients had only one surgery, whereas the other six had at least two operations. A total of 16 surgeries were performed. Primary recontouring surgery for the eyelid was performed as described above. Further tumor debulking was the objective for secondary eyelid procedures. We have seen recurrence in one patient. The patients were very happy to be able to retain their eye and visual acuity. No major complication observed in our series.
Figure 5: Two-year follow-up

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Some improvement was noted in majority of the patients on the PCD scale [Table 3]. An overall fall in PCD score might correspond to uncorrected canthal position, whereas an improvement in “P” grade showed a restoration of the visual axis in the patient. No complaints of corneal irritation or symptoms of exposure were noted in the immediate postoperative period. There was a resolution of epiphora in many patients. Early patient satisfaction was encouraging; however, there was some deterioration noted of esthetic and functional parameters with time.
Table 2: Ptosis, Canthal involvement, and Oculopalpebral Diastasis scale for grading the severity of palpebral neurofibroma (as suggested by Marchac and Britto, 2005)

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Table 3: Jackson Grouping and Ptosis, Canthal involvement, and Oculopalpebral Diastasis score (pre- and post-operative)

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  Discussion Top

[TAG:2]NF occurs in approximately 1 in 3000 live births. The orbital, eyelid, and temporal regions are involved to a different extent, with ptosis being the most common orbital abnormality among them all.[1],[7],[20],[21] In this article, our focus was the correction of the eyelid deformities in relatively mild-to-moderate NF cases. A minimum follow-up of 1 year was selected, as the disease is known to be progressive with time. Accordingly, in one patient, the tumor progressed during the 4th year of follow-up, and no further growth was observed in 8 patients who were followed for a period of 1–4 years.[2][/TAG:2]

An early intervention may help prevention of development of worsening of symptoms and maintaining the anatomical structures in an anatomic positions.[8]

The surgical approach depends on the stage of disease progression and age of the patient, coupled with patient expectations.[7],[9],[22],[23],[24],[25],[26]

In Jackson Grade I group, with a soft-tissue involvement and Seeing Eye, Krohel et al. recommended in toto excision via an anterolateral orbitotomy. Anterior, lateral, or anterolateral orbitomy has been advocated by various authors. In view of the diffuse extension of NF, complete excision would be difficult and and surgical debulking without any blueprint or format may lead to unfavorable esthetic outcome through any approach utilised.[7],[8],[24],[26] We noted predictable esthetic outcome with an outline of surgery based on the composite axial eyelid flap.

In Jackson's second group with additional significant bony involvement, an intracranial approach was advocated for additional bony wall reconstruction. Marchac first advocated this surgery with bony flap reflection coupled with repositioning of the temporal lobe and reconstruction of the walls of the orbit. However, this approach has a higher rate of complications. An intraoperative navigation approach was advocated by Friedrich et al. to increase the safety of the procedure.[27] However, we employed the composite axial flap and still had satisfactory esthetic and functional outcomes. We did not correct the bony involvement. However, with our patients being from a mostly rural background who were not willing to take the risk of the complications of bony surgery, they were satisfied with the functional and esthetic outcome of only soft-tissue correction.

In previous studies, with this technique, good visual recovery was noted in all patients and many regained levator function.[2],[5],[9],[11],[13],[17],[22] Similarly, in our study, there was good early visual recovery, with six eyes being free from ptosis at the last examination. Two had some remaining ptosis which did not hinder the visual axis and in one patient, the pupil was half covered.

The canthus showed improvement in four of nine cases. This may be attributed to the fact of a deeper spread in the temporal region, which sags and affects the lateral canthus. As this was remodeling the eye, to remove an obstruction to vision and to improve esthetics, no change was expected in the eye-globe relation, which was observed with the constancy of D parameter.

The authors suggest that this procedure has multiple advantages: in mild cases, managed by debulking, there is the preservation of the eyelid margin. In additional, the least harm was noted to the palpebral sphincter due to careful interfascicular dissection of the orbicularis oculi. Postoperative complaints related to dry eye or corneal exposure was not given by the patients. In more advanced cases with extended temporal disease and distended lateral eyelid, the lateral extent of oblique wedge helped ensure the vascular supply from medial palpebral artery is undisturbed. In the present study, we included all patients with histologically positive NF from all ages and sex who presented to our clinic. Although progression is more common in children, in older patients, the tumor also might progress with time and reoperations might be needed. Hence, the treatment should be tailored to each patient. In our series, the vision was the primary objective for which 80% of the first surgeries took place, although insignificant improvement was observed during the follow-up. After achievement of an unblocked visual axis, the target was to improve the cosmetic appearance.

Disease progression was a short coming of this method, with possible recurrence. However, progression is at a variable pace in individuals and factors determining it still need to be ascertained.[11],[17] It also helps avoid the morbidity associated with aggressive resection of the tumor. Multiple surgeries are a disadvantage of this technique; however, in the opinion of the authors, it provided the patients with a chance of prolonging and may be altogether avoiding the aggressive resection, while at the same time having improved functional and cosmetic appearance.

One may expect relapse of the improvement or recurrence; however, the relief that the patient expressed is a justifiable objective one can work toward.

In the third group with soft tissue and bony involvement coupled with a displaced eye,[7] aggressive bony reconstruction would be required if opting for the surgical reconstruction and orbital exenteration also remains as a viable option. However, in our series, we did not operate on any patient with this advanced disease as they were apprehensive of the outcome and associated complications, accompanied with skeletal surgery. Cosmetic surgery does help to reduce the distress arising from the societal stigma of looks. However, an individual customized approach would be best to judge if it would be of any benefit to these patients. If the lesion is huge, does not exhibit signs of progression, and a patient is unwilling to undergo any surgery for it, it can also be left to regular follow-up.[7],[8],[24],[25],[26]

  Conclusion Top

We believe that in patients with mild-to-moderate orbital NF (as per the Jackson classification),[8] multiple surgeries tailored to fit the requirements of each patient, an acceptable functional and esthetic result can be achieved, creating a new supratarsal crease for the patient.


We acknowledge Cleft-Children International, Zurich, for funding of this surgery.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

  [Table 1], [Table 2], [Table 3]


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