From the Mount Vernon Centre for Plastic Surgery, Northwood

Transcription

1 THE FRONTO-NASAL GLIOMA By R. L. G. DAWSON, M.B., F.R.C.S., and I. F. K. MUIR, M.B.E., M.B., F.R.C.S. From the Mount Vernon Centre for Plastic Surgery, Northwood CHILDREN quite commonly present with swellings at the root of the nose. A small number ultimately turn out to be composed of neurological tissue, and to these the name of " Fronto-nasal Glioma " has been given. The clinical features are distinct enough for at least a tentative diagnosis to be made before operation. We propose to give a brief review of the condition and to describe two patients treated at this centre. A third child with a similar lesion has also been treated but is being described elsewhere. The lesions are essentially benign and cause symptoms by local expansion only. The main mass of the lesion may lie outside the nasal skeleton, usually to one side of the midline (as in our patients), or it may be deep to the skeleton ; while in a third group there may be a dumb-bell type of configuration with intranasal and extranasal components. The clinical picture depends largely on the position of the mass. REPORTS OF CASES Case I.--M. S., aged I½ years (Figs. I to 5). This child presented with a lump over the nasal bridge present since birth. It was slowly growing, ~ in. in diameter, and FIG. I FIG. 2 Case I. Before operation. Case I. Before operation. to the left of the midline. It was smooth and round in outline, the skin was mobile, the mass was fixed deeply. No intranasal abnormality was detected. Operation.--A longitudinal incision was made down the nasal bridge line. The tumour was shelled out from its capsule, and the extension through a hole between the r36

2 THE FRONTO-NASAL GLIG~'..iA 137 FIG. 3 FIG. 4 Fig. 3.--Case I. After operation. Note residual prominence of the left fronto-nasal process and false appearance of hypertelorism. Fig. 4.--Case I. After operation. FIG. 5 Case I. Photomicrograph showing interlacing collagenous fibres, capillaries, and uninucleate and multinucleate ceils of varying size. (High power.)

3 i3 8 BRITISH JOURNAL OF PLASTIC SURGERY nasal bones was removed. This hole was in continuity with a blind pocket on the left side of the septum, between the mucoperichondrium and the cartilage in which the tumour had been lying. Closure was made with medium silk, without nasal infracture. He developed a streptococcal infection of the wound which, however, settled with the evacuation of pus, and with a course of penicillin and sulphadiazine. He was discharged eleven days after operation. Section Report.--The tumour was composed of a stroma of interlacing collagenous fibrous tissue between which were collections of cells and their fibres. The cells varied in size and some were multinucleate. Last attendance (27th November 1954) showed that the left nasal bone was displaced laterally. There was a wide nasal bridge, and a wide septum with a redundant lateral cartilage in the left nostril. Case 2.--S. H., aged 4½ years (Figs. 6 to II). A lump on the left side of the nose was noticed three weeks after birth. This gradually grew in size, till at the age of 42 it was ½ in. in diameter. The child was otherwise well and developing normally. Examination.--There was a solid tumour roughly spherical and ½ in. in diameter FIG. 6 FIG. 7 Case 2. Before operation. Case 2. Before operation. lying under the skin on the left side of the nose. The skin was mobile over the lump, which was fixed deeply to the nasal bone. Intranasally there was no abnormality. X-ray showed a smooth-edged circular hole in the left nasal bone about 1 in. in diameter. Operation.--A vertical incision was made over the turnout, and skin flaps were dissected up. The tumour was then dissected free and found to be roughly spherical and lying in a saucer-shaped depression of the nasal bone. From the deep aspect of the tumour a stalk passed through a circular smooth-edged hole in the left nasal bone. This stalk easily broke. The margins of the hole were then removed with a chisel and the stalk was found to pass in a cephalad direction between the nasal mucoperiosteum and the bone for about i in. It was followed up by removing more bone with bone nibblers. At the completion of this dissection the nasal mucosa was intact, but the piece immediately underlying the stalk was removed and kept for section. Skin dosed by interrupted sutures. Ih'scharged six days after operation. Pathological Report.--Sections showed a mass composed of glial tissue and

4 THE FRONTO-NASAL GLIOMA I39 FIG. 8 Fig. 8.--Case 2. Fig. 9.--Case 2. After operation. fronto-nasal process. After operation. FIG. 9 Note residual prominence of the left Case 2. FIG. IO X-ray to show the smooth-edged defect in the left nasal bone.

5 14o BRITISH JOURNAL OF PLASTIC SURGERY collagenous tissue intermingled ; the resulting pattern was complex, but demarcation between the two tissues was precise. When last seen three months after operation it was noticed that the bridge line of the nose was straight. There was still some depression at the site of the lesion, but this FIG. I I Case 2. Masses of glial and collagenous tissue intermingled but well demarcated one from the other. The matrix surrounding the glial cells is well shown. depression seemed to be filling out. At the posterior edge of the bone defect, however, there was a rather prominent ridge. PATHOLOGY These tumours are present at birth, do not show autonomous growth, but enlarge with the normal growth of the child, and they should therefore be classified rather as examples of errors of development than as true neoplasms. Grossly, the lumps are encapsulated, and of a firm, greyish-pink flesh. Histologically the tumours are composed of a fibrous tissue capsule, enclosing masses of fibrillary astrocytes and multinucleate astrocytes (Bratton and Robinson, I946). They are therefore collections of well-differentiated, normal glial tissue. In our cases the main mass of the turnout lay between the nasal bone and the skin and was connected with the interior of the nose by a narrow stalk, passing through a hole in the nasal bones, and terminating beneath the nasal bone or alongside the septum. The nasal bone, on the side of the lesion, was deformed by the tumour. In cases reported by others, expansion was predominantly intranasal with widening of the nasal bridge. The tumours do not ulcerate or invade surrounding tissues. Rocher and Anglade (1924) reported five cases ; three of these were extranasal at the root of the nose, one was intranasal, and the other was both intranasal and extranasal. In our cases the intranasal pedicle stopped short of the cribriform

6 THE FRONTO-NASAL GLIOMA 141 plate, but it is apparent from the reports of others that a pedicle may communicate with the interior of the skull. In two of Rocher and Anglades' patients division of the pedicle was followed by a leakage of cerebrospinal fluid which necessitated ligature of the pedicle. _,~TIOLOGY Heredity does not appear to play any part, but the condition is often noticeable at the time of birth. Three theories have previously been postulated to account for the development of these malformations :-- I. The turnouts originate from an encephalocele, which has been cut off during development. Sometimes a connection with the intracranial contents may remain, as in the two patients of Rocher and Anglade. 2. The tumours are of blastomatous origin, cut off early in intrauterine life (Berblinger, quoted by Bratton and Robinson, 1946) ; other writers incline to a later extracranial separation of embryonal neuroglia. 3. Such displacement arises from marginal islands of tissue from the optic plate or neighbouring neural ectoderm (Bratton and Robinson, 1946). We would like to make the following observations :- If the first theory is correct, then the lesions should be most common in the midline, whereas, as we have indicated, they are commonly unilateral. If the third theory is correct, it seems remarkable that islands from the optic plate should migrate to the inside of the nose, yet fail to produce gliomatous lesions in the orbit where they are apparently excessively rare. Study of the development of this region suggests a further explanation more in harmony with the facts. The nose is formed by indentations of the ectoderm, below the forebrain, called olfactory pits. The ectoderm of these pits gives rise to the olfactory nerve cells which send back their processes to meet, in the olfactory ganglia, the downgrowing processes of cells from the forebrain. Furthermore, it has been shown that the cells of the olfactory epithelium are connected from the first with the brain by a bridge of protoplasm which is never severed and across which the fibres from nose and brain travel to meet each other (" Gray's Anatomy," 1938 ; Elliot-Smith, 19o8 ; Frances Ballantyne, I925). We feel that it is along this bridge of protoplasm that aberrant glial cells may migrate, passing out into the nose to form the nidus of the fronto-nasal glioma. CLINICAL FEATURES The swelling at the root of the nose may have been present since birth. Alternatively, the parents may have noticed a gradual widening of the nasal bridge. Nose-bleeding does not occur, except after interference. With larger intranasal masses nasal obstruction with consequent mouth-breathing may occur. When the lump is obvious externally it is invariably most prominent on one side. The skin over it is mobile and normal. The lump is rounded, firm, and attached to the underlying skeleton, and it may be possible to feel the gap in the bone through which the turnout has come. When all the turnout lies deep to the nasal skeleton, unilateral or bilateral widening of the nasal bridge may be observed, and intranasally a smooth swelling covered by normal nasal mucosa can be seen. True hypertelorism does not develop, and the interpupillary distance is normal. 2E

7 142 BRITISH JOURNAL OF PLASTIC SURGERY General underdevelopment of the nose may be present in older children, but this appearance is merely the result of interference with the growth of the nasal bones by pressure of the tumour. The rest of the facial appearance is normal. We know of no record of these tumours involving the orbit. The affected children are in other respects normal in all stages of their development. RADIOLOGICAL EXAMINATIONS A frontal metopic suture is not of diagnostic value, but a lateral view of the nose and 3o-degree and 6o-degree occipito-mental views may show a divarication of the nasal bones with a possible defect through which the tumour protrudes. The fronto-nasal processes may also be displaced. An apparent defect on the skull base, with the ethmoid air cells on each side, is more suggestive of nasopharyngeal meningocele than a fronto-nasal glioma. There is no enlargement of the lesser wing of the sphenoid, as seen in true hypertelorism. DIFFERENTIAL DIAGNOSIS The congenital presence of thcse tumours rules out many lumps that may appear in later life, such as neurofibromata, ganglioncuroma, nasal fibroma, adcnoma, carcinoma, and chondroma. Dott and Guthrie (I927) also reported an intranasal herniation of a cerebral glioma through the root of the nose. The remaining lumps appearing at a very early age are :-- I. Nasal dermoid, which starts as a central swelling in the region of the scptum and is tensely cystic, fixed deeply, producing symmetrical widening of the nasal bridgc, and often containing a sinus to the skin, through which occasionally small hairs may be seen to sprout. It commonly presents as a swelling recurring after inadequate operation. Thcre may be a palpable divarication of the septum at the nasal bridge. 2. Frontal rneningocele, which is usually higher up over the glabella, soft, compressible, and may show pulsation. It often has a good covering of skin and it may enlarge on tipping the child head downwards or when the child cries. Radiologically a gap may be found in the frontal bone, or an enlarged metopic suture. 3. Sinus Pericranff and Frontal Hcemangioma.--The appearance is of a bluish superficial mass, soft, compressible, and filling on hanging the head or crying. A communicating foramen may be seen radiologically in the region of the glabella. 4. Split Face.--This is a congenital defect sometimes associated with median cleft mandible, maxilla, lips and nose, and fused frontal lobes. The condition may, however, be manifest only in the nose, producing a notched nasal tip and a wide nasal bridge line with a double septum. Some degree of hypertelorism is always associated, and no local lump is palpable. PROGNOSIS As we have already stated, these "gliomata" are not really neoplasms at all. They grow as the child grows, but as they grow they may cause deformity of the nasal skeleton. If satisfactorily removed they will not reappear, but such removal may mean pursuing the stalk as far as the cribriform plate, and ligating it there.

8 THE FRONTO-NASAL GLIOMA 143 These stalks may leak cerebrospinal fluid, and local infection may cause meningitis. The stalks can be dissected outside the nasal mucosa however so that such a danger is not great. TREATMENT The treatment is by operation. The lump should be approached extranasally, and can be shelled out of its capsule easily once the right layer is entered. It may be necessary to remove a portion of one or other nasal bone in order to gain access to the deeper part and to the stalk. The deep extension or the stalk must be followed through the hole in the nasal bones as far as possible before ligation, and this may mean detaching the upper attachment of both nasal bones so that they can be retracted laterally. In some cases the stalk may pass beneath the nasal bone, but external to the nasal mucosa, and end gradually, or abruptly, without extending as far as the cranium. In one of our cases the stalk passed through a small hole between the nasal bones and ended in a pocket between the septal mucosa and septum. The lump having been removed, the deep layers are approximated, displaced bones replaced, and the skin is sutured. If a stalk extending to the cribriform plate has to be ligated, the administration of systemic penicillin and sulphadiazine is indicated. SUMMARY Two cases of fronto-nasal glioma are described, the literature is reviewed, and the diagnosis, pathology, and treatment are discussed. We wish to thank Mr Rainsford Mowlem for permission to treat these patients, and Miss Walker and Mr Paul Toiler for the photographs. REFERENCES BALLANTYNE, F../~[. (1925). Trans. roy. Soc. Edinb., 53, 663. BRATTON, A. B., and ROBINSON, S. H. G. (1946). J. Path. Bact., 58, 643. DOTT, N., and GUTHRIE, D. (I927). ft. Laryng., 42, 733. ELLIOT-SMITH, G. (19o8). Anat. Anz., 33, 513 " Gray's Anatomy " (1938). Seventh Edition, p London : Longmans Green. ROCHER, H. L., and ANGLADE (19Z4). Rev. Chit., Paris, 62, E #