D. Denis

Ocular findings in children operated on for plagiocephaly and trigonocephaly.

Clinical examination of patients affected by plagiocephaly or trigonocephaly reveals evident malformation of the orbits, and the ocular repercussions are more pronounced when children are operated on at an advanced age. Since it is generally accepted that bionocular vision is fully developed by approximately 6 months of age, a late correction of plagiocephalic or trigonocephalic skull deformities may be an obstacle to the development of normal visual function. For the present report we investigated astigmatism and strabismus in 53 children, 39 of whom were operated on for plagiocephaly and 14 for trigonocephaly. Traction on the ocular globe induced by the bony deformation caused by the craniosynostosis may explain astigmatism and strabismus.

The skull base in plagiocephaly

Between 1975 and 1992, 426 children with craniofacial malformations were treated in the Department of Pediatric Neurosurgery at the Hôpital des Enfants de la Timone in Marseille. Plagiocephaly was present in 71 (16.6%). The authors present a reproducible analysis of the skull base in plagiocephaly based on these 71 patients. A control group of Mediterranean children (n = 20) was used for comparison. Clinical anthropometric patterns were analyzed in all cases. Comparison with the control group showed a difference only in the nasion-lambda distance. Data obtained from clinical anthropometry were compared for the involved and the uninvolved sides. A threedimensional reconstruction was possible in 20 cases. The statistical correlation between the basal angles (nasion-pterional, nasion-petrosal, nasion-clino-basion, and zygomatic angles) of the involved and uninvolved sides allows a new nosographic identification of this complex malformation.

Distinctive clinical features of bilateral Duane retraction syndrome.

PURPOSE To compare the clinical characteristics of unilateral and bilateral forms of Duane retraction syndrome. METHODS A retrospective chart review of patients with Duane syndrome over a 10-year span was performed. All participants underwent a complete medical and ophthalmologic examination at the time of diagnosis. Data collected included the following: sex, age at first visit, form (unilateral/bilateral), side, type, ocular duction and version, abnormal head position, best-corrected visual acuity, refractive errors, presence of strabismus, binocular function, and associated congenital anomalies. RESULTS Of 94 patients identified, 17% were bilaterally affected. Of the bilateral patients, 12% had familial antecedents; 87.5% had the same type in both eyes with 75% bilateral type 1. Patients with bilateral Duane syndrome had significantly different visual acuity (mean, 0.1214 logMar vs 0.0035; p = 0.045), abnormal head position (56.25% vs 89%; p = 0.04), A or V patterns (69% vs 27.6%; p = 0.003), and associated congenital anomalies (50% vs 14%; p = 0.003) than patients with unilateral disease. There was also a suggestion of higher rates of ametropia, global vertical dysfunction, and abnormal stereoacuity in bilateral Duane syndrome that were not statistically significant. CONCLUSIONS Patients with bilateral Duane syndrome had lower visual acuity, a lower incidence of abnormal head posture, and a higher incidence of A or V patterns and associated congenital anomalies. Bilateral Duane retraction syndrome seems to involve multiple instances of deinnervation and fibrosis, resulting in horizontal and vertical oculomotor deficits and functional impairments.

Duane retraction syndrome: Type II with severe abducens nerve hypoplasia on magnetic resonance imaging.

Duane retraction syndrome is a congenital oculomotor disorder characterized by horizontal eye movement limitations with palpebral fissure narrowing and globe retraction on attempted adduction. Recent reports showed the absence of the subarachnoid part of the abducens nerve on magnetic resonance imaging (MRI) in types I and III. We present a case of Duane syndrome type II, in which severe hypoplasia of the abducens nerve was revealed on high-resolution MRI.

Ocular Growth in the Fetus. 1. Comparative Study of Axial Length and Biometric Parameters in the Fetus

The knowledge of ocular growth during fetal life, when compared with other fetal biometric parameters, could not only provide a better definition of malformation syndromes but could also give a better understanding of certain pathological processes in premature babies and in newborns. As the literature concerning prenatal ocular dimensions contains few data, the aim of this study was to measure the axial length of the globe (AL) in fetuses and compare this measurement with their gestational age, weight, height, head circumference (HC) and thoracic circumference (TC) in order to compile a reference table. In the present study, 76 globes from 38 fetuses (18-41 weeks gestational age) from the Department of Pathology (Timone University Hospital, Marseille) were examined. Ultrasonography A and B were used to measure the AL, and a pathological examination determined fetal weight, HC, TC and height. We were interested to find out which of the parameters studied would give the best correlation with ocular growth. Statistical analysis showed that HC remained the most discriminant factor and correlated best with ocular growth. We thus obtained an equation for ocular size according to HC that could serve as a basis for detecting pre- or postnatal ocular defects.

Ocular findings in trigonocephaly

Abstract• Background: Trigonocephaly, caused by premature closure of the metopic suture, is a rare form of craniosynostosis. The aim of this study was to assess the visual outcome in children operated on for trigonocephaly • Methods: We present eight cases of children with trigonocephaly surgically corrected by the same craniofacial technique. CT with 3D reconstruction was performed in all cases. Genitori defined three types of trigonocephaly according to the severity of the deformity of the skull base only types II and III were included in this study directed at evaluation of the ocular disorder. A complete eye examination was performed on all children by the same observer, with a follow-up of 2–6 years • Results: 3D-CT reconstruction of the skull base showed that the frontozygomatic region was affected by the deformation. Ocular examination showed considerable astigmatism in most children with late operation. A low degree of strabismus was observed in most children • Conclusion: This study demonstrated that reconstructive surgery should be performed by the age of 6 months, given the immaturity of the visual system up to that time. Close cooperation between neurosurgeons, pediatricians and ophthalmologists is of paramount importance in order to prevent this bone deformation exerting an adverse effect on visual development. The ophthalmologist must possess a basic understanding of the various craniosynostoses.

Ocular Growth in the Fetus. 2. Comparative Study of the Growth of the Globe and the Orbit and the Parameters of Fetal Growth

In a previous study, we showed that the growth of the eye during fetal development compared with the other parameters of the fetus was above all connected to the head circumference, which appeared to be the most discriminant measurement. In a second study, we measured the growth of the orbit of the fetus in order to compare it with the growth of the globe and the cranial circumference. Another 60 eyeballs of 30 fetuses of 18-41 weeks gestational age were examined. The corneal diameter (CD) of the cornea was a measurement added to those used in the method in the previous study, and orbital radiography was carried out: the parameters used were the same as in the previous study measured in relation to the CD, orbital diameter (OD) and the biparietal diameter (BPD). The aim of this study was to show the links between the variations in the measurements and to estimate them in relation to fetal growth. The results confirm those of the previous study, and this study also shows that, in estimating the head circumference, the OD and CD are the most accurate indicators. This study was also concerned with the BPD: the best correlation is obtained with the OD, which can be estimated accordingly from the BPD. Although the OD is not in an area studied by ultrasonography (highly dependent on the sectional plane), this study shows that it should perhaps be reconsidered in the prenatal diagnosis of orbital malformation. It would then be necessary to take into account not only the BPD but also the relationship between the OD and the BPD.

Isolated infantile myofibromatosis of the upper eyelid: uncommon localization and long-term results after surgical management

Myofibroma, the most common juvenile fibrous disorder in infancy, usually manifests as a solitary soft tissue tumor and less commonly as simultaneous multiple tumors in both soft tissue and bones. Infantile myofibromatosis is well described in the head and neck, but cutaneous lesions rarely occur in the periorbit and orbit, where fast growth and bone damage can mimic malignant tumors. We describe a case of a solitary periorbital myofibroma in a newborn. Treatment consisted of partial excision of the tumor. Histologic and immunohistochemistry analyses provided the diagnosis of infantile myofibromatosis. The patient remains disease-free at 10 years of age, but with some visual impairment. Infantile myofibromatosis is an uncommon tumor with exceptional periorbital involvement. Differential diagnosis can be difficult when it is solely based on histologic assessment. Immunohistochemistry evaluation demonstrating cytoplasmic actin filaments in neoplastic spindle cells confirms the diagnosis. As soon as the diagnosis is made, chest and abdominal imaging must be performed to evaluate the overall prognosis and direct treatment. The treatment of choice is early conservative surgery to minimize functional and/or esthetic damage. Complete tumor excision is not always possible. Lengthy ophthalmologic monitoring is required to detect the onset of amblyopia.

Fractures de l’orbite de l’enfant

The aim of this article is to review data concerning paediatric orbital fractures. These fractures exhibit strong specificities because they occur in a growing face. Due to the craniofacial growing pattern and the peumatization of paranasal sinuses, there are differences in the anatomical location of orbital fracture with the age: before the age of seven they are mostly orbital roof and after seven they involve the orbital floor. The clinical diagnosis is confirmed with a computed tomography scan (CT scan), gold standard for the imaging in the orbital fractures. The magnetic resonance imaging (MRI) offers a better soft-tissue depiction and is useful when clinical data are not consistent with CT scan findings. The orbital fractures in children are rarely operated. In emergency the main surgical indications are the trap-door fracture involving the ocular muscles and the compressive haematomas. We hypothesize that the periosteum more likely than the bony structure is involved in the responsible trap-door fractures: the thickness and the elasticity of the periosteum leads to reposition the floor or the medial wall of the orbit to its initial position.

Mesure de l'épaisseur de la couche des fibres nerveuses rétinienne péripapillaires par tomographie à cohérence optique chez les enfants hypertones et glaucomateux

PURPOSE To compare retinal nerve fiber layer (RNFL) measurements taken with OCT in normal, ocular hypertensive, and glaucomatous eyes of children 4-18 years old. MATERIALS AND METHODS The study included 181 eyes: 106 normal, 37 with ocular hypertension, and 38 with juvenile glaucoma. Diagnostic groups were classified based on intraocular pressure, optic nerve head appearance, visual field examination, and central corneal thickness measurement. RNFL measurements were taken using the Zeiss Stratus OCT. RESULTS The RNLF average thickness was 94.2 + or -13.2 microm in glaucomatous eyes, 105.4 + or - 6.8 microm in ocular hypertensive eyes, and 104.8 + or - 10.3 microm in normal eyes. RNFL average thickness, inferior thickness, and superior thickness showed statistically significant differences between normal and glaucomatous eyes (p<0.01) and between glaucomatous [corrected] and ocular hypertensive eyes (p<0.01). There was no difference found between normal and ocular hypertensive eye. CONCLUSION OCT has already shown its diagnostic efficiency in adult glaucoma. This study shows that OCT is useful in childhood glaucoma diagnosis, helping to differentiate between ocular hypertension and beginning juvenile glaucoma.