Optic glioma in children: surveillance, resection, or irradiation?

Eighty-seven consecutive children with newly diagnosed optic glioma were managed at University of Toronto hospitals 1958-1990. Overall the 10-year survival, relapse-free survival and freedom from second relapse rates were 84%, 68% and 85%. Twenty-seven patients relapsed or progressed, of whom 40% were free of a second relapse 10 years after the first relapse. Fourteen patients had a second relapse. Thirteen are dead. None survived 5 years after second relapse. Patients with anteriorly located tumors (N = 35), which involved the optic nerve, or chiasm and optic nerves, fared better than those with posteriorly located tumors (N = 52) with spread beyond the chiasm, 10-year survival 95% versus 76%, (p = .02), 10-year relapse-free survival 80% versus 59% (p = .02), respectively. For posterior tumors primary irradiation was more effective than primary subtotal resection for prevention of subsequent relapse, 10-year relapse-free survival 75% versus 41% (p = .02), but salvage therapy was, in part, successful and multivariate analysis of prognostic factors influencing survival for posterior tumors indicated that neither primary resection nor primary irradiation were significant factors. For first relapse, primary irradiation and the presence of neurofibromatosis were the significant favorable factors. Since 1977 and for posterior optic glioma subtotal resection or surveillance were used in 21/29 (72%) patients compared with 4/23 (17%) previously. Ten-year survival rates before and after 1977 were 78% and 67% and 10-year relapse-free survival 64% and 56%, respectively.

Primary hereditary oxalosis retinopathy.

A female infant had progressive atypical pigmentary retinopathy with type 1 hereditary oxalosis. At the age of 3 months she had a flecked retina type of retinopathy and six months later she exhibited a unique type of atypical pigmentary retinopathy. This latter abnormality was characterized by a dense parafoveal hyperpigmented ring five disc diameters in size, and composed of a confluence of small rings of hyperpigmented retinal pigment epithelium surrounding whitish highly refractile calcium oxalate crystalline deposits.

The detection and management of intracranial hypertension after initial suture release and decompression for craniofacial dysostosis syndromes.

We performed a retrospective study of 107 consecutive patients with syndromic forms of craniosynostosis (craniofacial dysostosis) seen by the craniofacial team at the Hospital for Sick Children between 1986 and 1992. There were 51 patients with Crouzon's syndrome, 33 with Apert's syndrome, 8 with Pfeiffer syndrome, 11 with Saethre-Chotzen syndrome, and 4 with kleeblättschadel anomaly. Six patients developed raised intracranial pressure (ICP) after initial suture release and decompression (Apert's syndrome, three patients; Pfeiffer syndrome, one patient; Saethre-Chotzen syndrome, two patients). Raised ICP was considered in those children who returned with a bulging fontanelle, progressive frontal bone protrusion, intermittent headaches, irritability, and vomiting. The diagnosis of raised ICP was based on papilledema (four patients), progressive macrocephaly (one patient), and ICP monitoring (one patient). No child in this group had hydrocephalus requiring cerebrospinal fluid diversion. Once raised ICP was detected in these children, a second operation was immediately performed to reduce the ICP with the intention of expanding the volume of the cranial cavity. The second procedures included: anterior cranial vault and upper orbital reshaping (four patients), posterior cranial vault reshaping (one patient), and total cranial vault reshaping (one patient). There were no perioperative complications in these patients, although one patient showed subsequent recurrence of raised ICP requiring further cranial vault re-expansion. At follow-up, ranging from 3 to 7 years, all six patients were asymptomatic without evidence of raised ICP. In our series, raised ICP occurred in 6% of the children with a craniofacial dysostosis syndrome after initial suture release and decompression.(ABSTRACT TRUNCATED AT 250 WORDS)

Polyarteritis nodosa complicated by a multiple sclerosis like syndrome.

A case is presented of a 16-year-old boy with angiographically proven polyarteritis nodosa who developed a multiple sclerosis like syndrome affecting the brain stem and cerebrum. His serum demyelinated nerve in tissue culture. The case is reviewed in detail and the mechanism of myelotoxicity is discussed.

Prophylactic lacrimal intubation for protection of the nasolacrimal apparatus during elective orbital osteotomies.

The lacrimal excretory system may be damaged after midface trauma or tumor resection, or injured in association with elective osteotomies involving the medial and inferior orbital walls and rims. Epiphora or problems of recurrent purulent ocular discharge and dacryocystitis may arise. Using the technique described by Crawford, we recently began to intubate at the time of surgery the lacrimal systems of our patients undergoing total midface and orbital osteotomy. Examples of the outcome of this procedure are presented. There were no cases of worsened epiphora or dacryocystitis. We detailed the use of lacrimal intubation in patients who are at high risk for postoperative obstruction of the lacrimal excretory system. Although our study was small and uncontrolled, our intention is to draw attention to the potential of intubation as an adjunct to the craniofacial surgeon's usual procedures.

Acquired Brown's syndrome associated with hypogammaglobulinemia.

We describe a 10-year-old girl with hypogammaglobulinemia who presented initially with painless progressive diplopia on right upward gaze and associated tenderness in the area of the superior oblique tendon (Brown's syndrome). She was given prednisone (40 mg/day) with gradual improvement of her symptoms. Prednisone was tapered and her symptoms remained unchanged for 10 months. She then experienced rapid deterioration. Prednisone was reinstituted with similar improvement and then therapy was switched to naproxen. She remains with only slight diplopia on extreme upward gaze.

The status at two years of low-birth-weight infants born in 1974 with birth weights of less than 1,001 gm.

A two-year follow-up study of 43 infants of birth weight less than or equal to 1,000 gm born during 1974 revealed the following: average height at two years was between the tenth and twenty-fifth percentiles; average weight between the third and tenth percentiles. Fifteen (35%) developed lower respiratory tract infections during the first two years. Seven (16%) had retrolental fibroplasia. Major neurologic defects occurred in four (9%); severe developmental delay (mean developmental quotient less than 80) was found in nine others (21%). Defects of the central nervous system were closely associated with a neonatal history of intracranial hemorrhage or seizures or both.

Ophthalmic involvement as a presenting feature of nonorbital childhood parameningeal embryonal rhabdomyosarcoma.

OBJECTIVE: To describe presenting ophthalmic signs and symptoms in children with parameningeal rhabdomyosarcoma. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Twenty-three children with parameningeal rhabdomyosarcoma treated from 1978 through 1998. MAIN OUTCOME MEASURES: Each patient's presenting symptoms and history, the ophthalmic signs at presentation, the location of the tumor on computed tomographic scanning, the pathologic diagnosis after tumor biopsy, and the child's outcome after chemotherapy and radiotherapy. RESULTS: Eight of 23 patients with parameningeal rhabdomyosarcoma had ophthalmologic signs at presentation. All patients had sixth nerve palsies, and four had additional third nerve palsies. Fifth and seventh nerve involvement was seen in three children each, and two manifested additional fourth nerve palsies. In two patients, invasion of the optic canals and posterior orbit resulted in unilateral blindness in one and marked unilateral visual loss in the other. In no patient was the symptomatic history longer than 12 weeks. Seven of eight patients were treated with a combination of surgery, chemotherapy, and radiotherapy. The mean survival time for five patients who died from parameningeal rhabdomyosarcoma associated with ophthalmic signs was 27.2 months. Three patients are currently alive, with a mean survival time of 33.7 months after diagnosis. The survival time of 12 patients with parameningeal rhabdomyosarcoma and without ophthalmologic signs at presentation was better, in that nine are alive, with a mean survival time of 54 months. CONCLUSIONS: Advanced parameningeal rhabdomyosarcoma with skull base invasion may present with ophthalmic signs. These patients sought treatment late and had advanced tumors because their initial signs and symptoms were often attributed to more common ailments. Usually, the onset of the cranial nerve palsy initiated referral to the tertiary care center. The sixth nerve was the most vulnerable. Further invasion of the skull base or cavernous sinus may cause additional third, fourth, and fifth nerve palsies. The optic nerve was involved when the optic canals and orbit were encroached on by the tumor. The prognosis for survival was poor when these tumors were accompanied by ophthalmic signs.

Ocular toxicity and antenatal exposure to chloroquine or hydroxychloroquine for rheumatic diseases.

Chronic use of chloroquine and hydroxychloroquine inthe treatment of rheumatic disease carries a small risk of sight-threatening pigmentary retinopathy. To obtain safety data for its use in pregnancy, we did ophthalmic examinations in 21 children born to women who took these drugsduring pregnancy. Average daily maternal doses of the two drugs were 317 mg hydroxychloroquine and 332 mg chloroquine. The mean duration of gestational exposure was 7.2 months. No ophthalmic abnormality was detected in these children. Therapeutic use of these drugs during pregnancy may not pose a significant risk of ocular toxicity to offspring.