Congenital dermoid cyst

Ease deports CONGENITAL

DERMOID

CYST*

JOHN B . ERICH, M .D . AND DAVID S . JOHNSEN, M .D. Rochester, Minnesota THOUGH congenital dermoid cysts are not rare, the one reported here is interesting because of its size, site and unusual complex nature .

1A

dermoid as "a cystic tumor consisting of at least 9o per cent dermal elements ." This interpretation is broad enough to allow for scattering of mesodermal elements such as

GROUP - REGION

CASES

I Orbital 51 49.5%

Floor of mouth III Submental 24 23.3% Submaxillary Occipital

Frontal IY Lip 15 14.6% Neck

Soft Palate FIG . I . Site of 103 dermoid cysts of the head and neck seen at the Mayo Clinic between 1910 and 1935 . (Reproduced with permission from NEw, G . B . and ERICH, J . B . Dermoid cysts of the head and neck . Surg ., Gynec . eY Obst., 65 : 48- 55 . 1937.)

Congenital dermoid cysts, also known as inclusion, sequestration and fissural dermoids, are associated with embryonal disturbances of development and result from infolding of the ectoderm along the normal embryonic lines of fusion . These extragenital dermoids occur in the face, scalp, neck, back, along the median line of the thorax and in the abdomen and sacral region . The Iiterature 1-5, $ is somewhat confusing concerning the relationship between dermoids and teratomas as to origin, definition and classification . There seems to be no sharp dividing line, and many authors consider it unnecessary to distinguish between the two ; however, for all practical purposes we prefer to define a

cartilage, bone, connective tissue and fat which occasionally are encountered in dermoids . When such elements are present, many authors refer to the tumor as a "complex dermoid" as contrasted to the "simple dermoid" which contains only skin components, that is, epidermis, derma and dermal glands . Thus the following classification may be presented for consideration : (t) Epidermoid cyst, an epidermal-lined cyst lacking definite dermal structures ; (2) dermoid cyst, a cystic tumor containing at least 9o per cent dermal elements . It may be a simple dermoid cyst containing only dermal elements, or a complex dermoid cyst containing up to to per cent of mesodermal elements in addition to dermal structures ; (3)

" From the Sections of Plastic Surgery and Laryngology, Mayo Clinic and Mayo Foundation, Rochester, Minn .

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American Journal of Surgery

Erich, Johnsen-Congenital Dermoid Cyst

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Fic . 2 . Large derinoid cyst in the substance of the right cheek in an infant six weeks of age . FIG . 3 . Appearance of child at fifteen months of age . Fic . 4 . Roentgenogram of child's head and cheek (Fig . 2) showing dental tissues and calcium deposits within the dermoid . FIG . 9 . Roentgenogram of child's head and the dermoid cyst (Fig . 3) at fifteen months of age .

teratoma, a congenital tumor usually containing elements of all three germ layers and less than 9o per cent of dermal elements . New and one of us' (J . B . E .) reviewed 10 3 cases of dermoid cysts of the head and neck . The great majority of these lesions had been noted at birth or during the first few years of life and all were related to lines of embryonic .January, 1953

fusion . While all of the cyst cavities in this series of cases were filled with sebaceous material, only 30 per cent contained tangles of hair . Microscopically, the cysts were lined with squamous epithelium resembling skin and contained hair follicles, and sweat and sebaceous glands . Organized structures such as teeth, nails or glandular tissues were not found in any

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Erich, Johnsen-Congenital Dermoid Cyst

of these tumors . None contained bone, cartilage or lymphoid tissue with the exception of one dermoid in the orbital region in which a little cartilage was discovered . This study of dermoids involving the head and neck revealed that practically all such lesions are very simple cysts of the congenital inclusion type . The anatomic distribution of the 103 dermoid cysts reviewed is shown in Figure i . Branchial cysts were not included in this review because of their microscopic appearance . The authors recognized, however, that embryologically these should be considered congenital inclusion dermoid cysts arising from branchial clefts .

be growing slowly in proportion to the growth of the child . Our examination revealed a large tumor measuring 8 inches in circumference and involving the entire right side of the face in an otherwise normal six week old infant girl . (Fig . 2 .) It was a soft, multicystic tumor containing several irregular, hard, nodular masses . Roentgenograms revealed teeth and calcium within the tumor . (Fig . 4 .) Because of potential risk of operating on an infant only six weeks of age, it was decided to defer surgical removal of this benign tumor until the child was at least one year old . At the age of fifteen months the patient was brought back to the clinic by her parents . In the interim the child had been well, and growth and development had progressed normally . The tumor had increased proportionately and now measured 15 inches in circumference . (Fig . 3 .) At that time a roentgenogram revealed a soft tissue tumor of the right cheek containing several areas of bone formation . Osseous development of the skull and facial bones appeared normal . The right mandible appeared slightly short but otherwise was normally developed . (Fig . 5 .) The preoperative diagnosis was teratoma . Under intratracheal anesthesia the tumor was removed along with a small portion of buccal mucous membrane of the cheek, which was adherent . The facial nerve was closely intergrafted with firm, nodular masses within the tumor and was destroyed . The mucous membrane was closed, and a drain was inserted into the mouth to prevent saliva from collecting in the cheek. The skin was sutured in accepted fashion and a drain placed into the dependent portion of the cheek . The child received a transfusion of 200 cc . of whole blood during the operation . Recovery was rapid and uneventful . (Fig. 6.) The pathologic report was dermoid cyst of right cheek containing teeth, bone, hemorrhagic material, sebaceous material, hair and fat.

CASE REPORT

COMMENTS

An infant girl was first seen at the clinic at six weeks of age because of a tumor of the right cheek which had been present at birth . Pregnancy and delivery had been normal, and the child weighed 6 pounds, 63i ounces at birth . According to the parents the tumor seemed to

According to the definition and classification previously mentioned this tumor is a dermoid because it contains at least 9o per cent dermal elements, and it may be called a "complex dermoid" because mesodermal elements (bone and fat) were also found . This tumor was

Fic. 6. Appearance of child two weeks after surgical removal of dermoid cyst . Because the nodular cystic tumor was closely integrated with the facial nerve, it was necessary to destroy the latter . This resulted in partial paralysis of the right side of the face . The appearance of the face and the scar on the right cheek can be improved later.

American Journal of Surgery



Erich, Johnsen-Congenital Dermoid Cyst present at birth and appears to be related to faulty closure of the intermaxillary cleft thus classifying it as the congenital inclusion type of dermoid . A congenital inclusion dermoid arising from the intermaxillary cleft is extremely rare, and therefore we believe this case worthy of reporting in the literature . REFERENCES 1 . BELL, E . T . A Text-book of Pathology, 4th ed ., PP. 381-383 . Philadelphia, 1941 . Lea & Febiger . 2 . BOYD, W . Surgical Pathology, 5th ed ., pp . 155--158 . Philadelphia, 1942 . W . B . Saunders Company .

3 . EWING, J . Neoplastic Diseases : A Treatise on Tumors, chap . 49, pp . 1041-1072, Philadelphia, 1940 . W. B . Saunders Company . 4 . KARSNER, H . T. Human Pathology, 7th ed ., pp . 374- 375 . Philadelphia, 1949 . J . B . I ippincott Company . 5- MACCALLUM, W . G . A Textbook of Pathology, 7th ed ., chap. 72, pp . 1204-1220 . Philadelphia, 1840 . W . B . Saunders Company . 6 . MACDONALD, J . R . Personal communication to the authors . 7- NEW, G. B . and ERICH, J . B . Dermoid cysts of the head and neck . Surg ., Gynec . er Obst., 65 : 48 -- 55, 19378 . SMITH, L . W . and GALT, E . S . Essentials of Pathology, 2nd ed., chap . 36, PP . 378-380 . New York, 1942 . D . Appleton-Century Company, lnc .

INTRATHORACIC goiters eventually produce a variety of symptoms requiring surgery . Davidson and Turner recently reported a rare instance in which this growth eroded into the esophageal lumen . Biopsy of this tumor during esophagoscopy showed thyroid tissue without evidence of a mucosal covering and chest x-rays showed the presence of an intrathoracic goiter . This low grade, chronic erosion occurred without marked clinical symptoms ; in fact, after biopsy (which confirmed this rare diagnosis) the patient was symptom-free and therefore refused surgery. (Richard A .

Leonardo, M .D.)

January, 1 953

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