NEUROFIBROMATOSIS
IN PLASTIC
SURGERY
By JOANNES J. A. M. BLOEM* and JACQUES C. VAR DER MEuLENt * Department of Plastic and Reconstructive Surgery, Free University, Amsterdam; t Department of Plastic and Reconstructive Surgery, Erasmus University, Rotterdam, The Netherlands THE protean manifestations of neurofibromatosis are well documented. Single or multiple, the lesions may involve almost any tissue of the body. Slowly growing, unless sarcomatous change occurs, neurofibromatosis can produce internal symptoms from pressure on vital structures or stenosis of visceral tubes and ducts. Subcutaneous lesions may produce hideous cosmetic deformities. The histological appearances are also well documented but certain features are of special interest to plastic surgeons faced with manifestations of the disease:
The lesion is not circumscribed or encapsulated and at the margin may be infiltrating dermis, muscle and nerve, but never epithelium, so ulceration is rare; In the involved dermis the elastic fibres become progressively fragmented and finally disappear completely (Fig. I), thus permitting the stretching of the skin which often occurs; The lesion is very vascular and the numerous capillaries are seen on section to be widely patent as if held open by the fibrous tissue (Fig. 2); copious bleeding must therefore be expected at operation and postoperative haematomas to be likely and avoided if possible;
FIG.
I.
Fragmented
and disappearing
FIG. z.
elastic fibres in dermis involved in neurofibromatosis.
Many widely patent capillaries traverse the lesion. 50
NEUROFIBROMATOSIS
IN
PLASTIC
FIG. 3.
Case
I
FIG. 4.
Case
I postoperative.
FIG. 5.
Case
FIG. 6.
Case
2 2
preoperative.
preoperative. postoperative.
SURGERY
51
52
BRITISH JOURNAL
OF PLASTIC
SURGERY
FIG. 7.
Case 3 preoperative.
FIG. 8.
Case 3 postoperative.
The overlying skin may become pigmented pigment is melanin.
and increase the deformity;
the
Smaller lesions can often be excised completely. In larger deformities the more disfiguring part can be excised, leaving important structures intact. A local rearrangement of tumour tissue can be quite acceptable (Davis et al., 1854). Usually the scars in these areas become unobtrusive, without a tendency to hypertrophy in spite of considerable tension on the wound edges. Apparently this is due to the lack of elastic fibres and to the immature collagen. It is believed that about IO per cent of cases may show sarcomatous degeneration. So far it has never been proven (d’Agostino et al., 1963) that repeated surgical intervention will cause malignant degeneration, although this has been suggested by some authors. ILLUSTRATIVE CASES
Case I. A 26-year-old woman presented with the lesions shown in Figure 3. The overlying skin was deeply pigmented. She had never worn a dress but used trousers to hide the deformity. Excision of the excess (Fig. 4) allowed her to dress normally. Four years later the residual tumour has grown to a degree to make further excision desirable. Case 2. The disease was limited in this 3o-year-old man to the right orbital region and temporal fossa. The lateral orbital wall, the floor of the orbit and the frontal bone were
NEUROFIBROMATOSIS
IN
PLASTIC
SURGERY
53
hypoplastic (Fig. 5). At operation, the orbital floor was elevated with bone grafts and the upper eyelid was reduced in size (Fig. 6). As the patient was satisfied with the result no further treatment was und:rtaken. Case 3. This man, now 46 years old, had all the muscles of his face and the vision in his right eye destroyed by neurofibromatosis. There was gross sagging and stretching of the skin (Fig. 7). During the extensive resection of skin and tumour which was required, the cutting diathermy was used to reduce bleeding. In spite of this IO units of blood were necessary during the operation. The postoperative result still leaves much to be desired (Fig. 8) because of the mask-like immobile features. REFERENCES D’AGOSTINO,A. N., SOULE, E. H. and MILLER, R. H. (1963). Sarcomas of the peripheral nerves and somatic tissue associated with multiple neurofibromatosis. Cancer, 16, 1015. DAVIS, W. B., EDGERTON. M. T. and HOFFMEISTER, S. F. CIWL~~. Neurofibromatosis of the ~ ,_., hkad and neck. Plakic and Recomtructive Su~~rry, 14, 186.
IN PLASTIC
SURGERY
By JOANNES J. A. M. BLOEM* and JACQUES C. VAR DER MEuLENt * Department of Plastic and Reconstructive Surgery, Free University, Amsterdam; t Department of Plastic and Reconstructive Surgery, Erasmus University, Rotterdam, The Netherlands THE protean manifestations of neurofibromatosis are well documented. Single or multiple, the lesions may involve almost any tissue of the body. Slowly growing, unless sarcomatous change occurs, neurofibromatosis can produce internal symptoms from pressure on vital structures or stenosis of visceral tubes and ducts. Subcutaneous lesions may produce hideous cosmetic deformities. The histological appearances are also well documented but certain features are of special interest to plastic surgeons faced with manifestations of the disease:
The lesion is not circumscribed or encapsulated and at the margin may be infiltrating dermis, muscle and nerve, but never epithelium, so ulceration is rare; In the involved dermis the elastic fibres become progressively fragmented and finally disappear completely (Fig. I), thus permitting the stretching of the skin which often occurs; The lesion is very vascular and the numerous capillaries are seen on section to be widely patent as if held open by the fibrous tissue (Fig. 2); copious bleeding must therefore be expected at operation and postoperative haematomas to be likely and avoided if possible;
FIG.
I.
Fragmented
and disappearing
FIG. z.
elastic fibres in dermis involved in neurofibromatosis.
Many widely patent capillaries traverse the lesion. 50
NEUROFIBROMATOSIS
IN
PLASTIC
FIG. 3.
Case
I
FIG. 4.
Case
I postoperative.
FIG. 5.
Case
FIG. 6.
Case
2 2
preoperative.
preoperative. postoperative.
SURGERY
51
52
BRITISH JOURNAL
OF PLASTIC
SURGERY
FIG. 7.
Case 3 preoperative.
FIG. 8.
Case 3 postoperative.
The overlying skin may become pigmented pigment is melanin.
and increase the deformity;
the
Smaller lesions can often be excised completely. In larger deformities the more disfiguring part can be excised, leaving important structures intact. A local rearrangement of tumour tissue can be quite acceptable (Davis et al., 1854). Usually the scars in these areas become unobtrusive, without a tendency to hypertrophy in spite of considerable tension on the wound edges. Apparently this is due to the lack of elastic fibres and to the immature collagen. It is believed that about IO per cent of cases may show sarcomatous degeneration. So far it has never been proven (d’Agostino et al., 1963) that repeated surgical intervention will cause malignant degeneration, although this has been suggested by some authors. ILLUSTRATIVE CASES
Case I. A 26-year-old woman presented with the lesions shown in Figure 3. The overlying skin was deeply pigmented. She had never worn a dress but used trousers to hide the deformity. Excision of the excess (Fig. 4) allowed her to dress normally. Four years later the residual tumour has grown to a degree to make further excision desirable. Case 2. The disease was limited in this 3o-year-old man to the right orbital region and temporal fossa. The lateral orbital wall, the floor of the orbit and the frontal bone were
NEUROFIBROMATOSIS
IN
PLASTIC
SURGERY
53
hypoplastic (Fig. 5). At operation, the orbital floor was elevated with bone grafts and the upper eyelid was reduced in size (Fig. 6). As the patient was satisfied with the result no further treatment was und:rtaken. Case 3. This man, now 46 years old, had all the muscles of his face and the vision in his right eye destroyed by neurofibromatosis. There was gross sagging and stretching of the skin (Fig. 7). During the extensive resection of skin and tumour which was required, the cutting diathermy was used to reduce bleeding. In spite of this IO units of blood were necessary during the operation. The postoperative result still leaves much to be desired (Fig. 8) because of the mask-like immobile features. REFERENCES D’AGOSTINO,A. N., SOULE, E. H. and MILLER, R. H. (1963). Sarcomas of the peripheral nerves and somatic tissue associated with multiple neurofibromatosis. Cancer, 16, 1015. DAVIS, W. B., EDGERTON. M. T. and HOFFMEISTER, S. F. CIWL~~. Neurofibromatosis of the ~ ,_., hkad and neck. Plakic and Recomtructive Su~~rry, 14, 186.
