NEUROFIBROMA TYPE 1 WITH INTRACRANIAL INFILTRATION A CASE REPORT

Dinar Rahmania , Agus Santoso Budi 2) 1) Resident of Department o f Plastic Reconstructive and Aesthetic Surgery Airlangga University School of Medicine-Dr. Soetomo Teaching Hospital Surabaya 2) Staf of Department of Plastic Reconstructive and Aesthetic Surgery Airlangga University School of Medicine-Dr. Soetomo Teaching Hospital Surabaya Submitted : November 2016 | Accepted : December 2016 | Published : Januari 2017


INTRODUCTION
Plexiform neurofibroma occurred in the head and neck region can be disfiguring, causing cosmetic embarrassment to the affected patients.Surgical management is the mainstay of therapy.It is challenging because of the vascularity, the risk of primary hemorrhage, and infiltration of facial soft tissues as well as the need to preserve functions and maintain aesthesis Neurofibromatosis is an autosomal dominant disorder involving both the central and peripheral nervous system.Its clinical mark is the development of multiple cutaneous and subcutaneous nodular tumor (Shin, J., Persing, JA., Throne, CH 2007, ,Kapur, S., Michael, BL 2013).
The hallmark of the NF-1 disease is tumors along the nerve sheath from the dorsal root ganglion to the terminal nerve branches.Plexiform lesions occur in 16% to 40% patients with NF-1, and are found on the trunk in 43% to 44% patients, the extremities in 15% to 38% patients and in the head and neck in 18% to 42% of patients (Creange, A. et all 1999, Kapur, S., Michael, BL 2013).Plexiform lesions occur in 16% to 40% patients with NF-1, and are found on the trunk in 43% to 44% patients, the extremities in 15%

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to 38% patients and in the head and neck in 18% to 42% of patients (Creange, A. et all 1999, Kapur, S., Michael, BL 2013).Plexiform lesions occur in 16% to 40% patients with NF-1, and are found on the trunk in 43% to 44% patients, the extremities in 15%

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to 38% patients and in the head and neck in 18% to 42% of patients (Creange, A. et all 1999, Kapur, S., Michael, BL 2013).  Rahmania D, 2010)   The patient underwent surgery with coronal approach extend to facelift incision   Bottom : after left lateral cantopexy and reducing skin excess. (Rahmania D, 2010)   3 days after surgery, significant changing of of the patient appearance was gained.The function of left N.VII was decrease, but contraction response of maxilla and mandibular nerve branches was present.

RESULT AND DISCUSSION
From the descriptive study above, the management of Neurofibromas with intracranial infiltration should be performed by multidisciplinary team.Reconstruction for the destructed bone will be performed later.
Generally the cosmetic appearance of this patient is very satisfactory.Bottom : after left lateral cantopexy and reducing skin excess. (Rahmania D, 2010)   3 days after surgery, significant changing of of the patient appearance was gained.The function of left N.VII was decrease, but contraction response of maxilla and mandibular nerve branches was present.

RESULT AND DISCUSSION
From the descriptive study above, the management of Neurofibromas with intracranial infiltration should be performed by multidisciplinary team.Reconstruction for the destructed bone will be performed later.
Generally the cosmetic appearance of this patient is very satisfactory.
NF-1 due to its composition of nerve sheath cells that proliferate along the length of nerve.Plexiform neurofibromas are frequently associated with hypertrophy of the soft tissue and hyperpigmentation or hypertrichosis of the overlying skin.Their growth possibly causing destruction or compression of local tissue and furthermore causing significant morbidity.
Figure.1.ed left temporal bone, lateral wall of the left orbit, maxillary and mandible bones, also left temporal lobe herniation to left orbital cavity(Rahmania D, 2010) Figure.1.ed left temporal bone, lateral wall of the left orbit, maxillary and mandible bones, also left temporal lobe herniation to left orbital cavity(Rahmania D, 2010) Figure.1.ed left temporal bone, lateral wall of the left orbit, maxillary and mandible bones, also left temporal lobe herniation to left orbital cavity(Rahmania D, 2010)

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Figure 2).During surgery, a mass on subgaleal layer infiltrated to left temporal fascia is found.The mass moreover destruct the left temporal, left fronto zygoma and left zygoma arch bone.The left temporal lobe herniation to left orbital cavity led to a suppresion onto left oculi (Figure 3).A neurofibroma reduction and left lateral cantopexy has been performed to maintain simetrical position of the left orbit.The mass was successfully removed then leaving a defect on the left temporal side with duramater and herniation temporal lobe in left orbital cavity as its base.In this condition, the exploration of left orbital cavity and positioning the temporal lobe to the normal position can't be performed since the increase of intra cranial pressure might occur.The surgical site closed after drain was inserted (Figure 4).

(
Figure 2).During surgery, a mass on subgaleal layer infiltrated to left temporal fascia is found.The mass moreover destruct the left temporal, left fronto zygoma and left zygoma arch bone.The left temporal lobe herniation to left orbital cavity led to a suppresion onto left oculi (Figure 3).A neurofibroma reduction and left lateral cantopexy has been performed to maintain simetrical position of the left orbit.The mass was successfully removed then leaving a defect on the left temporal side with duramater and herniation temporal lobe in left orbital cavity as its base.In this condition, the exploration of left orbital cavity and positioning the temporal lobe to the normal position can't be performed since the increase of intra cranial pressure might occur.The surgical site closed after drain was inserted (Figure 4).

Figure 4 .
Figure 4. Post Operative : Top : after reductive excision of neurofibroma, skin excess was left.Bottom : after left lateral cantopexy and reducing skin excess.(RahmaniaD, 2010)   3 days after surgery, significant changing of of the patient appearance was gained.The function of left N.VII was decrease, but contraction response of maxilla and mandibular nerve branches was present.