Serial Excisions of Three Big Tumors on the Upper Right Side of

Nose, Left Nostril, and Right Side of Chin of NF 1 under Tumescent

Surgical Anesthesia in a Woman

Sri Lestari, Fesdia Sari, Nadya Hasriningrum, Elsi Kemala Putri, Yulia Eka Suryani

Dermato-Venereology Department of Dr. M Djamil Hospital/ Medical Faculty of Andalas University, Padang, Indonesia

Keywords: Neurofibromatosis type-I, excision, flaps

Abstract: Background: Neurofibromatosis type-1 (NF1) is autosomal dominant and multi-system disorders. An

incidence 1 in 3000 live births. There are three big tumors on the face that could disturb function organs

around the tumors and she wanted to remove that tumors. Case report: A case of 32-year-old woman with

chief complaint there were brownish patches and bumps that painless and not itchy on the most part of the

body since 20 years ago. At the age of 10 year-old there were appeared multiple skin colour bumps and

growing bigger all over the body. On physical examination there were multiple skin colour tumors on most

of the body, three big tumors on upper right side of nose, left nostril, and right side of chin, caféau-lait spots,

generalized distribution, disseminated. We consulted to Ophtalmologic Departement there were Lish’s

nodules on iris of her eyes, on Neurology Departement no neurologycal focal defisits were found.

Histopathological examination is neurofibroma. We excised three big tumors on the upper right side of nose,

left nostril, and right side of chin with eliptical, subcutaneous tissue pedicle island flap and long inferiorly

melolabial transposition flap designs under tumescent surgical anesthesia and there were good cosmetic

result. Discussion: NF-1 is best cared for within a multidisciplinary approach, which has access to a wide

range of subspecialists. We excised three big tumors on upper right side of nose, left nostril, and right side

of chin every two-weeks to maintanance function of organ with good cosmetic result.

1 INTRODUCTION

Neurofibromatosis (NF) is a term that has been

applied to a variety of related syndromes,

characterized by neuroectodermal tumors arising

within multiple organs and autosomal-dominant

inheritance. At least 8 different clinical phenotypes of

neurofibromatosis have been identified and are linked

to at least two genetic disorders. Neuro-fibromatosis

type I (NF-1) is the most common type of the disease

accounting 90% of the cases, and is characterized by

multiple café-au-lait spots and the occurrence of

neurofibromas along peripheral nerves (Burton et al.,

2012; Dimitrova et al., 2008).

Von Recklinghausen’s

neurofibromatosis (NF-1) is inherited in an

autosomal-dominant and has a prevalence 1 per 3000

and 1 per 5000 live births (Dimitrova et al., 2008).

The diagnosis NF-1 was made according to the

presence of four of the seven diagnostic criteria of the

National Institute of Health Consensus Development

Conference at least two of the following criteria must

be present to make the diagnosis of NF-1 (Burton et

al., 2012; Dimitrova et al., 2008).

Five or more cafe-

au- lait spots larger than 5 mm in diameter in

prepubertal patients; six or more cafe-au-lait spots

larger than 15 mm in diameter in postpubertal

patients, two or more neurofibromas of any type, or

one plexiform neurofibroma, axillary or inguinal

freckling, optic glioma, two or more Lisch’s nodules,

a distinctive osseous lesion (pseudoarthrosis of the

tibia or sphenoid wing dysplasia), a first-degree

relative diagnosed with NF-1 in accordance with the

above criteria (Dimitrova et al., 2008; Moraes et al.,

2013; Ghalayani et al., 2012; Goldberg & Alam,

2004).

There is no medical treatment for NF-1 at this

time. Neurofibroma therapy is not required and

commonly unsuccessful with high rate of recurrences.

Discrete cutaneous neurofibromas may be removed

surgically to improve cosmetic or to prevent local

irritation (e.g., from brushing for lesions in the

hairline or from rubbing against the shoe for those on

the foot). Deeper neurofibromas may require surgical

removal when they push on vital structures, such as a

Lestari, S., Sari, F., Hasriningrum, N., Putri, E. and Suryani, Y.

Serial Excisions of Three Big Tumors on the Upper Right Side of Nose, Left Nostril, and Right Side of Chin of NF 1 under Tumescent Surgical Anesthesia in a Woman.

DOI: 10.5220/0008158604110414

In Proceedings of the 23rd Regional Conference of Dermatology (RCD 2018), pages 411-414

ISBN: 978-989-758-494-7

411

dorsal root neurofibroma that infiltrates the neural

foramen and compresses the spinal cord.

Complications of surgery include regrowth of the

original tumor and nerve damage (Burton et al., 2012;

Dimitrova et al., 2008).

We reported three big tumors of NF1 on the upper

right side of nose, left nostril, and right side of chin of

32 year-old woman, and she wanted to remove that

tumors because of blocking of the right eye sight,

push the left of nostril and disturb the right side of

lower lip.

2 CASE

A 32 year-old woman, came to out-patient

Department of Dermato-Venereology on November

2017 with there were skin colour bumps that

painless and not itchy on the most part of the body

since twenty years ago. There were three big skin

colour bumps on the face: on the upper right side of

nose, left nostril, and right side of chin. There was

positive family history, her daughter was 6 years old

complained skin colour bumps on the back. On

physical examination we found multiple skin colour

tumors, the largest amount being on the upper right

side of nose, left nostril, and right side of chin ranging

from 1-4 cm, caféau-lait spots. On Ophtalmology

Departement there were lish nodules on her eyes and

there were no optic glioma. On Neurology

Departement there was no neurologycal focal defisits

were found. We excised three tumors on the upper

right side of nose, left nostril, and right side of chin

with eliptical, subcutaneous tissue pedicle island flap

and long inferiorly melolabial transposition flap

designs under tumescent surgical anesthesia to

maintanance the function of organ around the tumors

with good cosmetic result. Histopatological

examination is neurofibmotasosis.

2.1 Procedural Operation

First time we operated the tumor on the upper right

side of nose with eliptical design. From the area

between eyebrows, we injected local anesthesia

Pehacain® then incised with blade no.15. Using

infiltrator cannula 3 mm diameter, we delivered

surgical tumescent solution anesthesia 35 cc under the

tumor and to subcutaneous tissue around the nose and

the skin became bulging. We waited 20 minutes and

after that we injected with Pehacain® (lidocaine HCl

2% 20 mg and epinephrine 12,5ug) superficially

along the incision lines. Excised the tumor and

anastomosed the wounds in subcutaneus space with

4-0 chromic gut and epidermis with 5-0 Prolene

suture®.

Two weeks later we excised the tumor on the left

nostril with subcutaneous tissue pedicle island flap

design. From the area between eyebrows, we injected

local anesthesia Pehacain® then incised with blade

no.15. Using infiltrator cannula 3 mm diameter, we

delivered surgical tumescent solution anesthesia 40

cc under the tumor and to subcutaneous tissue around

the left nasolabial fold and left cheek and the skin

became bulging. We waited 20 minutes and we

injected local anesthesia Pehacain® superficially

along the incision lines. Excised the tumor and

anastomosed the wounds in subcutaneus space with

4-0 chromic gut and epidermis with 5-0 Prolene

suture®. Two weeks later we excised the tumor on

the right side of chin with long inferiorly melolabial

transposition flap design. From the mid right

mandibula, we injected local anesthesia Pehacain®

then incised with blade no.15. Using infiltrator

cannula 3 mm diameter, we delivered surgical

tumescent solution anesthesia 80 cc under the tumor

and to subcutaneous tissue around the chin and right

cheek, until the skin became bulging. We waited 20

minutes and we injected local anesthesia Pehacain®

superficially along the incision lines. Excised the

tumor and anastomosed the wounds in subcutaneus

space with 4-0 chromic gut and epidermis with 5-0

Prolene suture®. There were good cosmetic result

after surgery and the function of the organs became

normal.

RCD 2018 - The 23rd Regional Conference of Dermatology 2018

412

Figure 1. Design operations (A) eliptical on the upper right side of nose, subcutaneous tissue pedicle island fiap on left

nostril, and long inferiorly melolabial transposition flap on right side of chin (B) four weeks after the third operation of the

tumors.

3 DISCUSSION

The patient has done serial exicions of three big

tumors on the upper side of right nose, left nostril and

right side of chin. We excised the tumor on the upper

right side of nose with eliptical design. The elliptical

excision remains an adaptable and essential surgical

strategy. Elliptical excision are easily designed and

can be adapted to many situations. The classic ellipse

is formed by tracing 2 arcs of a circle on the skin. The

arcs, which are symmetrical with respect to the

midline axis separating them, intersect at their ends to

form a convex shape. Commonly used curvature is

variable, but typically leads to a 1:3 to 1:4 width-

length ratio between the short and long axes of the

formed ellipse. Intersection of the arcs an elliptical

angle of 30° has been traditionally assumed at the

ends (Goldberg & Alam, 2012).

The tumor on the left nostril was excised with

subcutaneous tissue pedicle island flap design from

the left cheek to the left side of the nose. The two

most commonly used local flaps for repair of

cutaneous defects of the nose and in which the donor

sites of the flaps are confined to the nose are the

subcutaneous tissue pedicle island advancement flap

and the bilobe flap. The island flap is used for repair

of defects located at the anterior aspect of the alar

groove. The bilobe flap is used to repair small

cutaneous defects of the nasal tip and caudal dorsum.

It is based on subcutaneous tissue and portion of the

transverse nasalis muscle. A triangular shaped flap

with its base making up the cephalic border of the

defect is designed with the apex of the flap positioned

laterally. The posterior border of the flap rests in the

alar groove. The anterior border extends cephalically

and slighly medially from the defect and is designed

to recruit skin of the nasal side wall. The anterior

border then arcs laterally to meet the posterior border

in the alar facial sulcus. The nasal skin is undermined

widely and the proximal and distal one-third of the

flap in undermined in the subcutaneous plane. The

central one-third of the flap remains pedicled on the

subcutaneous tissue. The flap in undermined only to

the degree that there is sufficient mobility to allow the

flap to be advanced into the recipient site. The flap is

advanced and secured at the recipient site first and

Serial Excisions of Three Big Tumors on the Upper Right Side of Nose, Left Nostril, and Right Side of Chin of NF 1 under Tumescent

Surgical Anesthesia in a Woman

413

then the donor site is closed to the repair (Baker,

2007).

The tumor on the right side of chin was excised

with long inferiorly melolabial transposition flap

design. The location and size of defect prevented

repairing the wound with a single unipedicle

advancement flap because of the inelasticity of the

chin skin. A long inferiorly melolabial transposition

flap was selected. The flap was design to recruit skin

from melolabial fold. It was slightly curved in its

linear axis to parallel the melolabial crease. This

facilitated placement of the flap donor site scar

directly with in the melolabial crease. Because the

flap was long relative to the width of the base, the

standing cutaneous deformity that formed on

transposition of the flap was not excised for fear of

compromising the vascularity of the flap (Baker,

2007).

4 CONCLUSION

NF-1 demonstrates a true proliferative process of

neuroectodermal tissue and it is need

multidisciplinary approach. There were no definitive

therapy and surgical therapy only for cosmetic and

maintain the function of organ around the tumors. We

have done operated of three tumors of NF1 of 32 year-

old woman every two weeks on the upper right side

of nose, left nostril, and right side of chin with

eliptical, subcutaneous tissue pedicle island flap and

long inferiorly melolabial transposition flap designs

under tumescent surgical anesthesia to maintain the

function of organs around the tumors with good

cosmetic result.

REFERENCES

Baker, S.R., 2007. Flap classification and design. In: Baker

SR, editor. Baker local flap in facial reconstruction.

Second editon. Philadelphia; Elsevier Inc, pp. 71-107.

Baker, S.R., 2007. Transposition flaps. In: Baker SR, editor.

Baker local flap in facial reconstruction. Second editon.

Philadelphia; Elsevier Inc, pp. 133-157.

Burton, S.C., Burkhart, C.N., Goldsmith, L.A., 2012.

Neurofibromatous. In: Freedberg IM, Eisen AZ, Wolff

AK, Austen KF, Goldsmith LA, Katz SI, editors.

Dermatology in general medicine. 8

ed. New York:

Mc.Graw-Hill, pp. 1679-1686.

Dimitrova, V., Yordanova, V., Pavlova, V., Valtchev, V.,

Gospodinov, D., Parashkevova, B., & Balabanov., C.,

2008. A case of neurofibromatosis type 1. Pleven,

Bulgaria. Journal of IMAB - Annual Proceeding

(Scientific Papers), 14, pp. 1-5.

Ghalayani, P., Saberi, Z., & Sardari, F., 2012.

Neurofibromatosis type I (von Recklinghausen's

disease): A family case report and literature

review. Dental research journal, 9(4), pp. 483-488.

Goldberg, L. H., & Alam, M., 2004. Elliptical excisions:

variations and the eccentric parallelogram. Archives of

dermatology, 140(2), pp. 176-180.

Moraes, F. S., Santos, W. E. D. M., & Salomão, G. H.,

2013. Neurofibromatosis type I. Revista Brasileira de

Oftalmologia, 72(2), pp. 128-131.

RCD 2018 - The 23rd Regional Conference of Dermatology 2018

414