Facial Aesthetic Unit Remodeling Procedure for Neurofibromatosis Type 1 Hemifacial Hypertrophy: Report on 33 Consecutive Adult Patients

Hivelin, Mikaël; Wolkenstein, Pierre; Lepage, C.; Valeyrie‐Allanore, Laurence; Meningaud, Jean Paul; Lantieri, Laurent

doi:10.1097/prs.0b013e3181d180e9

Cited by 28 publications

(17 citation statements)

References 63 publications

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“…Patients’ priorities need to be respected, as surgery in these patients is to some extent palliative. 2 …”

Section: Discussionmentioning

confidence: 99%

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Staged Ear Transfer and Reconstruction in a Severe Case of Neurofibromatosis Type 1

Abdulrauf

2020

Plastic and Reconstructive Surgery - Global Open

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Summary: Cases of neurofibromatosis type 1 may present with varying degrees of craniofacial deformities. Involvement of the ear and surrounding soft tissues may include hypertrophy, distortion, and sagging. We present the case of a 40-year-old woman with a severe plexiform neurofibroma affecting her left face and ear. Ear position was extremely altered by the chronic disease process, with sagging almost to the level of the clavicle. The patient was subjected to a 3-stage surgical intervention aimed at transfer of the external ear to its native region and debulking of the involved facial soft tissue tumors. A large Z-plasty was performed to transpose a flap containing the external ear, with its underlying extremely stretched auditory canal, to a proper location. In the second stage, further tumor debulking was performed. In the final stage, the floppy ear was set back significantly by obliteration of the auriculocephalic sulcus and was suspended by suturing to the mastoid fascia. The patient showed a remarkable improvement. A significant part of her neurofibroma was debulked, and the ear was salvaged, reduced in size, and transferred to a near-normal location. This was quite a unique and challenging case in terms of extreme external ear ptosis and malposition; however, success was achieved through adherence to basic principles and techniques, staging, collaborative support, and full commitment.

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“…Patients’ priorities need to be respected, as surgery in these patients is to some extent palliative. 2 …”

Section: Discussionmentioning

confidence: 99%

“…Elephantiasis neuromatosa is a severe form of neurofibromatosis type 1 1 , 2 in which there is diffuse proliferation of Schwann cells and axons together with a bony hypertrophy, resulting in massive disfigurement. We herein report a case of this kind associated with a severely ptotic ear.…”

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confidence: 99%

Staged Ear Transfer and Reconstruction in a Severe Case of Neurofibromatosis Type 1

Abdulrauf

2020

Plastic and Reconstructive Surgery - Global Open

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“…Hypotensive anesthesia and infiltration of tumescent fluid are effective in decreasing the intraoperative bleeding. [14,15] The multi-layered hemostatic sutures permit to remove piecewise the tumor avoiding profuse bleeding in all our procedures, and is based on vessel collapse after mechanical ligation, [4] from the superficial surface to deep layers, preserving the vital structures. The stitches placed parallel to the axial pattern of the nerve branches and controlling the depth of the stitch superficial to our plane of disection makes sure that we do not trap the facial nerve branches nevertheless, some of the network interconnection between the zygomatic and buccal branch can be trapped by the suture, without affecting the facial expression.…”

Section: Discussionmentioning

confidence: 99%

Facial Plexiform Neurofibroma excision with sequential muti-layer hemostatic sutures, the novel technique to reduce blood loss

Velazquez-Mujica

Cadavid

Francesco

et al. 2020

JST

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Plexiform neurofibromatosis is an autosomal dominant and is frequently seen at birth. Surgical excision is asociate to facial nerve damage and profussal bleeding. Sequential multi-layered hemostatic sutures is a technique frequently used in our practice for Arterio-veous malfromations (AVM). 15 patiets with facial plexiform neurofibroma were treated from 2004 to 2020 with surgical excision, in all patients the hemifacial area was affected. Although preoperative embolization is well known as a safe technique to reduce intraoperative bleeding, low rates of serious complications were reported as stroke, ischemic attack and necrosis. The multi-layered hemostatic sutures permit to remove piecewise the tumor avoiding dramatically bleeding in all our procedures, and is based on vessel collapse after mechanical ligation. The sequential multi-layer suture and the retrograde disection of the facial nerve in our practice has decresed the average of iatrogenic damage of nerve, and massive bleeding during the excision of the plexiform neurofibroma.

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“…9,14 -17 Also, it has been reported that 10% to 26% of NF-1 cases may progress to sarcomatous degeneration. 3,18,19 Only rare, well-circumscribed neurofibromas can be removed easily. However, the more common neurofi- Free-Flap Reconstruction After NF Resection broma has diffuse soft-tissue infiltration, which makes complete resection difficult.…”

Section: Discussionmentioning

confidence: 99%

Free Flap Reconstruction of Extensive Defects Following Resection of Large Neurofibromatosis

Uygur

Chang

Crosby

et al. 2011

Annals of Plastic Surgery

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Facial Aesthetic Unit Remodeling Procedure for Neurofibromatosis Type 1 Hemifacial Hypertrophy: Report on 33 Consecutive Adult Patients

Abstract: The facial aesthetic unit remodeling monobloc translesional resection technique on a preestablished pattern has been reproducible, offered increased predictability in functional and cosmetic results, and allowed us to operate on extensive hemifacial lesions with a lower transfusion risk.

Cited by 28 publications

References 63 publications

Staged Ear Transfer and Reconstruction in a Severe Case of Neurofibromatosis Type 1

Staged Ear Transfer and Reconstruction in a Severe Case of Neurofibromatosis Type 1

Facial Plexiform Neurofibroma excision with sequential muti-layer hemostatic sutures, the novel technique to reduce blood loss

Free Flap Reconstruction of Extensive Defects Following Resection of Large Neurofibromatosis

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