Algernon B. Reese

Treatment of Retinoblastoma by Radiation and Triethylenemelamine

We have been treating retinoblastoma by radiation according to a method described by us first in 1936. 1 The technique, which was devised by Dr. Hayes E. Martin, Memorial Center for Cancer and Allied Diseases, includes the employment of special cones at a temporal and a nasal portal in an effort to deliver an adequate dose to the posterior sector of the eye, to the exclusion of the vulnerable anterior sector. Subsequent reports on the use of this technique were made in 1942, 2 1945, 3 and 1949. 4 The treatment is employed for patients with bilateral tumors whose eye with the more advanced disease has been enucleated. The less involved eye has been treated when it was thought that useful vision might be salvaged if the tumor could be arrested. Since the institution of the treatment in 1936, 1 a total of 148 patients have been treated. Forty-three patients

Management of retinoblastoma.

Retinoblastoma belongs to the tumor family of neuroblastoma, one of the more common cancers affecting children. It occupies a unique position as the only truly malignant human tumor with definite hereditary characteristics. So dominant is this trait that sterilization, or abortion, seem justified in the case of a survivor. The medical profession is often derelict in failing to advise the parents or the patient against having children. Statistics indicate that the frequency of the tumor has shown a steady increase from 1 in 34,000 in 1931 to 1 in 14,000 births in 1961 (FranCois) ,* Factors to be reckoned in this increase are lower mortality rate, i.e., more adult survivors produce affected offspring, and improvement of investigation and diagnosis. Let us assume that 70 per cent of these children, if handled carefully, will now survive and that social and other factors will limit the production of these survivors to half the normal rate. The net reproductive expectation at birth of any person with retinoblastoma will then be 35 per cent of normal. This change in recent decades from virtually zero to 35 per cent will bring a commensurate change in the frequency of the responsible gene. If the mutation rate is 2 X then in eight generations or so, retinoblastoma will occur half again as frequently as it does today. To extend the speculation further, if we can some day save all patients with this disease and enable them to reproduce at the normal rate, retinoblastoma will become the normal characteristic as the result of constant mutation pressure, and individuals without the disease will be the exceptions. While this is highly unlikely, it does suggest that as control of neoplastic and other lethal disease is added to the conquest of infection, genetically determined disease will play an increasingly significant role in the development of mankind. The tumor first manifests itself as a whitish cat’s-eye reflex in the pupil. It has multicentric origins in one or both eyes (two-thirds are unilateral and one-third bilateral); 84 per cent are multicentric in one eye alone. When the disease involves only one eye, enucleation is almost categorically advised and, if performed before metastasis or extraocular extension have occurred, proves curative. If both eyes are involved, the eye with the more advanced tumor is removed and treatment is directed to the remaining eye. There are two general therapeutic approaches, the one to treat the entire retina (radiation and chemotherapy), the one to treat only the tumor-bearing area (locally applied radioisotope, light coagulation, and diathermy). These modalities are employed according to the disease stage and the tractability of the individual case. The criteria for suitability for treatment are shown in TABLE 1. Based on 164 cases treated by radiation and triethylenemelamine (TEM) between 1953 and 1960 for which there is adequate follow-up, the cure-rate is

Exophthalmos in Leukemia

In a case of lymphatic leukemia, the ocular manifestations first led the patient to seek medical advice. Exophthalmos is a rare symptom of myelogenous leukemia, but in the case reported it was induced by an expulsive orbital hemorrhage and was accompanied by choroidal hemorrhage with retinal detachment. A report of these two cases was presented at the meeting of the Section of Ophthalmology of the New York Academy of Medicine, December 21, 1931.

Orbital Tumors and Their Surgical Treatment*: Part I

This paper will be published in two installments in successive issues of the Journal. This installment includes a discussion of exophthalmos from tumor and tumorlike lesions primary in the orbit. The next installment will include exophthalmos from lesions adjacent to the orbit and systemic and distal lesions, together with a discussion of the indicated surgical procedures.

Massive Retinal Fibrosis in Children

The author has encountered several cases characterized by a grayish-white mass protruding from the retina in young individuals. In three instances these could be proved to be due to the organization of hemorrhage occurring at birth, or from trauma after birth. Those that occurred at the time of birth were due to the same predisposing factors which produce the analogous condition of intracranial hemorrhages in the newborn. The fundus picture resembled that of retinoblastoma from which it must be differentiated. The clinical and pathological relationship of this condition to metastatic ophthalmia, Coatss disease, retinitis circinata, and disciform macular degeneration is pointed out. From the Department of Ophthalmology, Columbia University. Cases 1 and 2 were from the service of the Eye Institute, case 3 was made available through the courtesy of Dr. D. H. Rhodes. Read before the American Academy of Ophthalmology and Otolaryngology at Cincinnati, September 17, 1935.

Management of retinoblastoma

lymph nodes involved survived this long. 9. Those patients operated upon with in one month of detection, who had a localized tumor resected by lobectomy, had a 71 per cent cure rate. Recent reports have emphasized that “¿ calcified― as well as “¿ non-calcified―lung lesions seen radiologically have a high cancer potential. The only lesions that can be considered benign by X ray appearance are those with dense or laminated calcifica tion demonstrated by laminography. All physicians should be urged to per

The Ciliary Processes

The increase in volume and the alteration in position of the ciliary processes are often factors to be reckoned with in intraocular surgery, especially in the trephine operation. This contribution presents an outline of the influences producing these factors and points out the frequency of incarceration and even of excision of the ciliary processes. The possible consequences of these complications are discussed, and finally, some measures designed to prevent their occurrence are suggested. Read before the Section on Ophthalmology at the Eighty-Fourth Annual Session of the American Medical Association, Milwaukee, June 14, 1933.

The Occurrence of Ciliary Processes on the Iris.

The author reports that it is not uncommon to find one or all of the ciliary processes on the iris instead of on the ciliary body. In such instances, the process is covered by a double layer of pigment epithelium around its base. There is usually a bridge between the iris processes and the contiguous process on the corona ciliaris. Under the bridge there may be a recess, which in the cross section has the appearance of a cyst in the iris stroma. Such processes, by interrupting the dilator-muscle layer of the iris, may give the latter a congenital irregularity; their excision may incite undue reaction, and may be an exciting cause of sympathetic inflammation. From the Department of Ophthalmology, Columbia University. Read before the American Ophthalmological Society, at Lucerne-in-Quebec, Canada, July, 1934.

Acquired Ectropium Uveae

Four types of uveal ectropion are defined, but this paper deals with the form commonly seen in pathologic eyes, especially glaucoma. It is based on a study of twenty cases and describes in detail the anatomic conditions observed. To explain the changes contracture of a membrane on the anterior surface of the iris, atrophy of the iris and proliferation of its pigment epithelium are all essential.