Paediatric blepharoptosis: a 10-year review.

PURPOSE: To examine the characteristics of blepharoptosis and the success of surgical intervention in a large group of children presenting to a specialist at paediatric ophthalmology center. METHODS: Ten-year retrospective case notes review of patients presenting to the Birmingham Children's Hospital for blepharoptosis surgery. Resultant database was interrogated for aetiology of ptosis; severity; surgical correction; outcome; complications and need for reoperation. RESULTS: One hundred and fifty five children (186 eyes) underwent blepharoptosis surgery. Hundred and ten patients (71%) were treated with a levator resection procedure, 28 (18%) underwent a brow suspension using Mersilene mesh, 15 (10%) with Fasanella Servat procedure and 2 (1%) with La Mange procedure. The mean post-operative follow-up was 30.82 months with 84 children completing a minimum of 12 months follow-up. Overall, 70.97% lids were successfully corrected with a single operation. In 9.14% lids, the results were fair but no further surgery was carried out. Reoperation was required in 19.89% of lids with the mean time to second surgery being 32.69 months. Amblyopia was found in 26.45% (41 children); in 3 patients, their amblyopia became manifest after the ptosis surgery. A concomitant squint was present in 14.19%, and 18.70% had a significant refractive error requiring spectacles prescription, with anisometropia present in more than 72% of these patients. CONCLUSIONS: Early referral to an ophthalmologist is necessary even though surgical correction may be delayed. Children with congenital ptosis need to be monitored for amblyopia both pre- and post-operatively, as the incidence of strabismus and refractive errors is much higher than the general population and these may develop even after ptosis surgery.

Neurodevelopmental implications of ocular motor apraxia.

Ocular motor apraxia (OMA), a disorder of saccadic initiation, may be congenital or acquired. While the acquired form is frequently associated with significant neuropathology, the congenital form is often regarded as relatively benign. Many children with congenital OMA who were observed clinically have shown neurodevelopmental disturbance over time. A retrospective review was taken of 34 consecutive patients (22 males and 12 females), seen over a 20-year period, to evaluate the frequency and type of associated neurodevelopmental problems. Age at presentation ranged from 8 weeks to 14 years, with a mean age of 10 years. Of 29 children with congenital OMA, 15 had imaging evidence of structural central nervous system abnormalities (with cerebellar hypoplasia the most frequent abnormality detected). Eleven of the 14 patients with no structural abnormality showed abnormal neurodevelopment. This study suggests that congenital OMA is not a benign diagnosis, even in the absence of overt neurological disturbance at the time of presentation.

Management of childhood epiphora.

AIMS: To examine the effectiveness of a management protocol for childhood epiphora using a joint ophthalmological and otolaryngological team approach. METHOD: A temporally defined retrospective study of 70 children (92 eyes) undergoing surgery for persistent epiphora, despite two previous technically successful probing procedures. All the operations involved a joint approach involving a paediatric ophthalmologist and a paediatric otolaryngologist. RESULTS: In children with congenital nasolacrimal obstruction this joint approach yielded a 73% (89%) success rate, while in children with acquired nasolacrimal obstruction the success rate was 57%. CONCLUSIONS: Endonasal nasolacrimal intubation and endonasal DCR are safe and effective procedures for the management of persistent epiphora in children. They avoid the need for overnight admission and carry a minimal complication rate.

Long-term follow-up of ptosis correction using Mersilene mesh.

The preferred operation for correction of severe ptosis with minimal levator function is a brow suspension procedure. Owing to the poorly developed fascia lata in young children, and concerns about the side effects of harvesting fascia, we have, for many years, used synthetic Mersilene mesh as our suspending material. We present our experience with this material over the last 10 years. We have undertaken a retrospective analysis of the case notes of all patients undergoing brow suspension surgery, using Mersilene mesh, from 1990 to 1999. Functional and cosmetic success was recorded with respect to long-term stability. Data from 41 patients (71 lids, 30 bilateral and 11 unilateral procedures) were analysed. The minimum follow-up period was 12 months with a mean of 38.4 months (SD 23.3). We found a long-term stability of ptosis correction in 67/71 (94.4%) procedures (38/41 (92.7%) of patients). Parental satisfaction was achieved in 90.1% (64/71) of procedures or in 85.4% patients (35/41). Four lids (5.4%) of three patients (7.4%) required further surgery. Mersilene mesh is a safe and convenient suspensory material for brow suspension in young children, and provides good long-term results with a low complication rate.

Ophthalmic follow-up of patients with tyrosinaemia type I on NTBC.

NTBC has revolutionized the management of tyrosinaemia type I, although animal experiments have shown that long-term administration may produce corneal opacities analogous to those in tyrosinaemia type II. We have assessed the prevalence of ocular side-effects in 11 tyrosinaemia type I patients on NTBC attending the Birmingham Children's Hospital. Despite high plasma tyrosine concentrations in some patients, they did not experience symptoms or signs of ocular toxicity.

Can convulsions alone cause retinal haemorrhages in infants?

AIM: To evaluate the likelihood that, in children under the age of 2 years, convulsions alone may cause retinal haemorrhages. METHODS: Children under the age of 2 years admitted to hospital following convulsions, were examined within 48 hours of admission. The convulsions were classified by a paediatric neurologist and detailed ocular examination, including indirect ophthalmoscopy, was performed by an ophthalmologist. Statistical analysis was undertaken using Hanley's rule of three. RESULTS: 32 consecutive children admitted with convulsions were examined; 10 of them were admitted following epileptic seizures and 22 following febrile convulsions. Two of the children with febrile convulsions were admitted in status epilepticus. None of these children had retinal haemorrhages. Therefore, using Hanley's rule of three, the upper limit of 95% confidence interval of retinal haemorrhages following convulsions in children under the age of 2 years, is less than 10/100. CONCLUSIONS: In children under the age of 2 years convulsions alone are unlikely to cause retinal haemorrhages. By combining the results of this study with those previously reported from this unit in older children, the upper limit of 95% confidence interval of retinal haemorrhages, following convulsions in children under the age of 14 years, is less than 5/100. Therefore, the finding of retinal haemorrhages in a child admitted with a history of convulsion should trigger a meticulous search for other causes of these haemorrhages, particularly non-accidental injury.

The association between the oculocardiac reflex and post-operative vomiting in children undergoing strabismus surgery.

The oculocardiac reflex (OCR) is a potentially serious complication of ophthalmic surgery which is most commonly elicited during paediatric strabismus surgery. Post-operative vomiting (POV) is also extremely common after such procedures and may result in admission following planned day-case surgery. Although many factors play a part in the occurrence of POV, stimulation of the trigemino-vagal reflex arc is thought to explain the particularly high rate of vomiting after strabismus surgery. The OCR and the vaso-vagal response share this neuronal pathway, the bradycardia of the OCR often being the only objective feature of the vaso-vagal response while the patient is anaesthetised. The aim of this study was to investigate the possible association between the occurrence of the OCR and subsequent POV in children undergoing strabismus surgery. We have studied this relationship in 79 children, aged between 1 and 13 years, undergoing strabismus surgery under standardised anaesthetic conditions. A positive OCR was regarded as a drop in heart rate of 10% or more, or the onset of a dysrhythmia. An intraoperative OCR was elicited in 51 (64.6%) of the 79 children, whilst 29 (36.7%) developed POV in the subsequent 24 h period. There was a significant association between a positive intraoperative OCR and POV (p = 0.01): children with a positive OCR were 2.6 times more likely to vomit than those without the reflex. We conclude that there is an association between the occurrence of the OCR and POV and discuss possible preventive strategies.

Retinal haemorrhages and convulsions.

AIMS: To evaluate the incidence of retinal haemorrhages after convulsions in children. PATIENTS AND METHODS: All children who required hospital admission after an episode of convulsions were included in the study. Complete neurological and ocular examinations, including ophthalmoscopy, were undertaken within 48 hours of hospital admission. RESULTS: Thirty three children were examined according to the protocol and their seizures were classified by a paediatric neurologist. Despite the fact that some of the children also vomited or underwent cardiopulmonary resuscitation, none of the 33 children developed retinal haemorrhages. CONCLUSIONS: Convulsions rarely (if ever) give rise to retinal haemorrhages. The finding of retinal haemorrhages should stimulate a detailed assessment to exclude non-accidental injury, whatever the nature of the associated or antecedent events.

Visual failure caused by raised intracranial pressure in craniosynostosis.

Craniosynostosis, the premature fusion of one or multiple cranial sutures, can be complicated by visual failure resulting from raised intracranial pressure (ICP). Of the 290 children operated on at the Birmingham Children's Hospital between 1978 and 1995 for craniosynostosis, 9 were found to have defective visual acuity attributable to raised ICP. Mean age at presentation was 3.11 years (range: 1-6 years) and mean follow-up, 7.33 years (range: 1.5-16 years). All these patients presented significantly later than usual, and 5 of them developed recurrent craniosynostosis. At the initial examination bilateral papilloedema was seen in 4 patients, unilateral disc oedema in 1 patient, bilateral optic atrophy in 3 patients and unilateral optic atrophy in 1 patient. Following decompressive craniofacial surgery, the visual outcome was good in 4 out of 5 patients with papilloedema and poor in all patients with optic atrophy. visual failure resulting from raised ICP in craniosynostosis is a devastating complication, which appears to be associated with late presentation and recurrent craniosynostosis.

Congenital oculomotor palsy: associated neurological and ophthalmological findings.

Congenital fourth and sixth nerve palsies are rarely associated with other evidence of neurological abnormality, but there have been conflicting reports in the literature on the associations of congenital third nerve palsy. In order to clarify the situation we report a series of 14 consecutive cases presenting to a paediatric tertiary referral service over the last 12 years. In this series of children, 5 had associated neurological abnormalities, lending support to the view that congenital third nerve palsy is commonly a manifestation of widespread neurological damage. We also describe for the first time a phenomenon of digital lid elevation to allow fixation with the affected eye. Two children demonstrated this phenomenon and in each case the accompanying neurological defect was profound. The frequency and severity of associated deficits is analysed, and the mechanism of fixation with the affected eye is discussed.

Trilateral retinoblastoma. A report of five patients.

BACKGROUND: Trilateral retinoblastoma is a well recognized, although rare, syndrome. Most of the reported cases have involved a family history of retinoblastoma (RB) and the disease is almost always fatal. The authors chose to investigate the cases of trilateral retinoblastoma occurring in the West Midlands, a region of the United Kingdom with an increasing incidence of bilateral sporadic RB. METHODS: Five patients with trilateral retinoblastoma (including two were previously reported), diagnosed in 146 consecutive patients with RB in the West Midlands Health Authority Region between 1957 and 1994, are presented (an incidence of 3%). Their clinical presentation, treatment, and outcome are described. RESULTS: There were 4 patients with pineoblastoma, only one of whom had a positive family history. The mean age at diagnosis of RB and 6 months, whereas the patients with pineoblastoma were diagnosed at a mean age of 2 years 8 months. The tumors were not evident on the initial computed tomography scans. One child presented with a calcified suprasellar mass 13 months before the bilateral sporadic RB was identified. Death occurred within 1 month of diagnosis of the intracranial tumor in 3 patients who did not receive any treatment. In the other 2 patients who were treated, death occurred at 15 months and 2 years 7 months, respectively, after diagnosis of intracranial tumor. CONCLUSIONS: Early diagnosis with regular neuro-imaging and more aggressive treatment may improve prognosis in patients with RB and an intracranial tumor.