Dermoid Cysts in Children

(1)

S

Dermoid

Cysts

in Children

PEDIATRICS Vol. 57 No. 3 March 1976 379

Zane F. Pollard, M.D., Robison D. Harley, M.D., F.A.C.S., and Joe Calhoun, M.D.

From the Department of Ophthalmology, St. Christopher’s Children ‘s Hospital, Philadelphia, Pennsyleania

ABSTRACT. A retrospective study of dermoid cysts in children over the past 13 years at St. Christopher’s Chil-dren’s Hospital of Philadelphia, showed that 37% of the

dermoids in the body occurred in the orbital and pen-orbital

areas. An unusually high percentage of these occurred in the left eyebrow which appears to be the most frequent location for dermoid cysts in children. Dermoid cysts are a very common lesion encountered by ophthalmologists inasmuch as 3.3% of all ophthalmic surgical procedures in children were performed for dermoids. All 231 cases in this series were benign. The manner and time of presentation of these lesions are discussed as well as the treatment. Pediatrics,

57:379-382, 1976, DERMOID CYSTS, EYE, OPHTHALMOLOGY.

Localized lesions or lumps about the head frequently present a diagnostic problem which can be solved by means of a biopsy. In children, the procedure is more complicated than in adults because a general anesthetic is usually required. Also, lesions around the orbit are viewed with a cautious eye by ophthalmologists because of the possibility of the loss of sight when dealing with the treatment of the lesion and because of the possibility of a communication with the cranium. This paper is concerned with the dermoid cysts around the orbit which we have frequently found in the children seen by the Ophthalmology Department at St. Christopher’s Children’s Hospital during the past 13 years. We will discuss the presentation of these lesions around the eye as well as point out their location throughout the body.

METHOD

All of the surgical records at St. Christopher’s Children’s Hospital were reviewed from 1961 to

1974 to obtain the names of patients who had surgery for a dermoid tumor. Then the patholog-ical reports were studied to be sure of the diagnosis. Many pathologists classify epidermoids and dermoids together. Because of their similar source of origin from embryonic ectoderm, many feel that a differentiation between the two is only academic. In a large part of the literature the older term dermoid is used to include both types.” Some pathologists feel that the term epidermoid should be used for a lesion lined with epithelium, while the term dermoid should be used for a lesion which, in addition to having a lining derived from cutaneous epithelium, also contains skin appendages such as hair follicles, sebaceous glands, or sweat glands. For purposes of uniformity we only included those cases which, in addition to having an epithelial-lined cavity, also had at least one skin appendage.

There was a total of 231 cases with slightly

(Received April 9; revision accepted for publication July 7, 1975.)

Supported in part by a grant from the Pennsylvania Lions Eye Research and Fight for Sight, Inc.

Read before the annual meeting of the American Association of Pediatric Ophthalmology, Lake Tahoe, Nevada, February 1975.

ADDRESS FOR REPRINTS: (Z.F.P.) 575 West Peachtree Street N.E., Atlanta, Georgia 30308.

at Viet Nam:AAP Sponsored on September 8, 2020 www.aappublications.org/news

(2)

SCALP 11%

EYELID 6.9#{176}/a

NOSE 2.6%

POST ANAL <1%

EYEBROW 29.7%

EAR 9.9%

NECK I7#{176}/

GENITAL 1%

GENERALIZED

SKIN 7.3%

RESULTS

FIG. 1. Distribution of dermoids in children.

I

FIG. 2. Dermoid of left eyebrow.

380 DERMOID CYSTS

more girls than boys with a ratio of 126: 105. The orbital and pen-orbital location was the most frequent area involved in the body with a total of 87 cases or 37% (Fig. 1). A most interesting fact was the frequency of occurrence in the left eyebrow which accounted for 50 of the cases and the right eyebrow which accounted for another 20. The appearance of a typical dermoid of the left eyebrow is shown in Figure 2. This

predomi-nance of the left side was also noticed in the ear; the left ear had more than twice as many as the right ear with a ratio of 16:7. The ovary is a rather rare location for a dermoid in a child as seen by the fact that we only had two cases. We had 16 cases occur in the eyelid. We had no cases of limbal or corneal dermoids.

The lesion usually presented early in life. Table

I shows

that

55 cases

presented

before

age

1, 77 between ages 1 and 3, 42 between ages 3 and 5, 30 between ages 5 and 10, and 27 between ages 10 and 15. During this period from 1961 to 1974 there were a total of 2,615 ophthalmic proce-dures performed at St. Christopher’s Children’s Hospital; 87 or 3.3% were for dermoids around the eye. We were quite surprised to find so many dermoids out of the total number of surgical cases as we know that strabismus surgery plays such an overwhelming part among the cases which come to ophthalmic surgery in children. All of our cases were benign.

Three of our patients were of unusual interest. One was a 5-year-old girl with proptosis from a deep orbital dermoid origination from the orbital apex which had been present for four years. It was removed in toto via a lateral orbitotomy with preservation of 20/25 visual acuity. Two cases

(3)

EYELID I0/ EYEBROW 1.8%

NECK 3%

POST ANAL 45% GENITAL 42%

OTHER 7.2%

FIG. 3. Distribution of dermoids in adults.

DISCUSSION

ARTICLES

381

TABLE I

AGE DISTRIBUTION OF CHILDREN WITH DERMOID CYSTS

Age at Surgery (yr) No. of Cases

<1 55 lto3 77 3to5 42 5tolO 30 lOtol5 27 Total 231

presented as masses in the left eyebrow, both of which extended from the subcutaneous tissue below the skin of the eyebrow to penetrate the roof of the orbit to end in a blind pouch just inside the orbit. These were both removed by carefully following the tumor through the bone which formed the roof of the orbit.

Dermoid cysts are felt to be congenital lesions which may lie dormant for various periods of time until they become manifest. The location of this lesion differs markedly in children when com-pared to adults. New and Erich’ reported their experience with 1,495 cases of dermoids at the Mayo Clinic from 1910 to 1935. This study from the Mayo Clinic was in an adult population. They found 42% in the genital area and 45% in the post-anal area, but only 7% in the head and neck (Fig. 3). In our series of children, we had 84% of the cases occur in the head and neck. In the adult series the genital organs were a common location for dermoids while in our series of children it was a rare location for this lesion. Of the adult cases, 3% were in the orbital and pen-orbital area as compared to 37% in our series of children. In the Mayo Clinic series, 1.8% occurred in the eyebrow while in our series 29.7% occurred there. The rather frequent location in the left eyebrow as well as the left ear is of special interest as congenital abducens nerve palsy,4 Duane’s syndrome5 (decreased abduction of the involved eye with closure of the eyelids and retraction of the globe on attempted adduction), and Marcus-Gunn jaw-winking ptosis (ptosis of the eyelid which disappears on opening the mouth or on extending the jaw toward the side opposite the side with the ptosis) have all been reported to occur more frequently on the left side.

The dermoid cyst, which is formed from embryonic ectoderm, may extend from the orbit to the cranium, paranasal sinus, or the temporal fossa. Likewise, a dermoid cyst of the cranium or

paranasal sinus may extend to the orbit. For this reason, it is important to know preoperatively if one is dealing with a strictly ophthalmic problem or one which also encompasses other organ sys-tems. Adequately performed X-rays usually answer this important question. Although all 231 cases in our series were benign, the possibility of a malignancy such as a metastatic neuroblastoma should be kept in mind as well as other ocular lesions which can cause proptosis such as heman-gioma of the orbit or optic nerve glioma. There have been several reports of proptosis due to dermoids79 in addition to our case, but our case was the only one of its kind originating so deep in the orbit in a child so young. There has been one reported case of a malignant degeneration in an orbital dermoid, but that was in an adult.

SUMMARY

Dermoid cysts are a frequent problem in chil-dren especially around the eye which is the most frequent location of these lesions. While other lesions must be considered in the differential diagnosis, the presentation in the eyebrow, espe-cially on the left side, in a child who is otherwise in good health should lead us to suspect a dermoid cyst. Preoperative X-rays looking for bony defects should be performed to aid the clinician in deciding whether he is dealing with a strictly

(4)

382 DERMOID CYSTS

ophthalmic problem or one which also encom-passes other organ systems. The majority of these lesions can be removed by a direct approach to the lesion through the eyebrow while a few will require extension into the orbit itself in order to remove the tumor.

REFERENCES

1. Henderson JW: Orbital Tumors. Philadelphia, WB Saunders Co, 1973, pp 86-96.

2. Reece AB: Tumors of the Eye, ed 2. New York, Harper & Row, 1963, pp 507-510.

3. New GB, Erich JB: Dermoid cysts of head and neck. Surg Gynecol Obstet 65:48, 1937.

4. Apple C: Congenital abducens paralysis. Am J Ophthalmol 22:196, 1939.

5. Duane A: Congenital deficiency of abduction, asso-ciated with impairment of adduction, retraction

movements, contraction of the palpebral fissure and

oblique movements of the eye. Arch Ophthalmol 34:133, 1905.

6. Beard C: Ptosis. St. Louis, CV Mosby Co. 1969, p 44. 7. Mehra MS. Bannerji C: Enlargement of optic foramen

due to dermoid cyst of orbit. Am J Ophthalmol 60:931, 1965.

8. Mortada A: Dermoid cyst of great wing of sphenoid bone. Br J Ophthalmol 54:131, 1970.

9. Pfeiffer R, Nicholl R: Dermoids and epidermoids of the orbit. Trans Am Ophthalmol Soc 46:218, 1948. 10. Jones A: Oil cyst of the orbit with carcinomatosis. Am J

Ophthalmol 18:532, 1935.

SCIENCE

AND

THE

CITIZEN

Dr. Halsted

R. Holman,

professor

of medicine

at Stanford

University,

told

a

Senate subcommittee that science had become too important to leave exclusively to the scientists. He said the nation needed social mechanisms of informed consent for science.

The tissue is not a struggle between science and antiscience, said Dr. Holman, but a struggle to achieve a healthy relationship between science and the citizens who support it and are affected by it.

The New York Times

Wednesday, April 23, 1975

Noted by STUDENT

(5)

1976;57;379

Pediatrics

Zane F. Pollard, Robison D. Harley and Joe Calhoun