Abnormal Pupil Finding After an Orbital Floor Fracture in a Child.

PURPOSE: Trauma to the pupillary fibers can occur during repair of orbital floor fracture resulting in a fixed and dilated pupil. The authors report, discuss, and propose an etiology of an abnormal pupil in a child with an orbital floor fracture before surgical repair. METHODS: A case report is described. RESULTS: Although the orbital fracture was repaired, anisocoria persisted 18 months after the initial trauma. CONCLUSIONS: Pupillary function must be assessed before surgical repair of an orbital floor fracture as damage of the pupillary fibers can occur secondary to the orbital floor fracture.

Ophthalmic features of Lamb-Shaffer syndrome: a case series.

Lamb-Shaffer syndrome (LSS) is a rare neurodevelopmental disorder, genetically diagnosed in fewer than 100 individuals worldwide. We present a case series of 6 pediatric patients with LSS and describe its ophthalmic manifestations. Strabismus was present in 5 patients, with exotropia being most common. All subjects had significant refractive errors; 5 had astigmatism of at least 2 D. All patients had optic nerve abnormalities, including pallor (4), hypoplasia (2), and anomalous appearance (1), with retinal nerve fiber layer thinning demonstrated in a single subject. Other ophthalmic disorders detected were ptosis (1), nasolacrimal duct obstruction (1), and nystagmus (2).

Initial Occurrence of Bilateral Anterior Uveitis in a Patient with Juvenile Idiopathic Arthritis following Discontinuation of Topical Steroids after Bilateral Strabismus Surgery.

Post-operative inflammation is a well-known complication of intraocular surgery. This is especially true in patients with a prior history of uveitis or with a predisposition for an inflammatory response. We report the first published case of a patient who developed bilateral anterior uveitis after bilateral strabismus surgery. It was the first episode of uveitis for this patient who had a pre-existing diagnosis of juvenile idiopathic arthritis.

Refractive Amblyopia Secondary to Astigmatism in Pediatric Patients With Distichiasis.

Distichiasis is the presence of accessory eyelashes emerging from the meibomian gland orifices. It may occur as an isolated abnormality or in conjunction with other ocular and systemic defects. Lymphedema-distichiasis syndrome (LDS) is an autosomal dominant disorder characterized by distichiasis and age-dependent lower extremity swelling due to altered lymphatic flow. The authors describe four pediatric patients with distichiasis (one with genetically proven LDS) with refractive amblyopia secondary to astigmatism. [J Pediatr Ophthalmol Strabismus. 2021;58(4);e16-e18.].

Ophthalmic findings of Mycoplasma-induced rash and mucositis (MIRM) distinct from Stevens-Johnson syndrome.

PURPOSE: To identify ophthalmic manifestations of Mycoplasma-induced rash and mucositis (MIRM). METHODS: The medical records of patients with MIRM treated by the inpatient ophthalmology consult service at a single institution over a period of 4 years were reviewed retrospectively. Eye and skin findings, treatment, hospital course, and follow-up findings were documented and reviewed. RESULTS: MIRM was identified in 10 patients, 10-20 years of age (mean, 13.) All presented with oral mucosal findings and a prodrome. On initial examination, conjunctival epithelial defects were present in 2 patients; pseudomembranes, in 3. Five patients experienced progressive ophthalmic manifestations during hospitalization, including a new cornea epithelial defect in 1 patient and a new conjunctival epithelial defect in 3 patients. All were treated with topical steroids and antibiotics. Post-discharge follow-up obtained for 7 patients documented good visual outcomes and lack of significant ocular sequelae without requiring invasive intervention. CONCLUSIONS: MIRM, previously referred to as mycoplasma-associated Stevens-Johnson syndrome (SJS) among other names, is now understood to be distinct from SJS in the dermatology community. Our MIRM patients present with mild eye findings and required no surgery, similar to the mild course published in the dermatology literature. Patients with MIRM may have a more benign course than those with SJS.

Prevention of Leber congenital amaurosis through preimplantation genetic diagnosis.

Preimplantation genetic diagnosis can allow a family with a hereditary genetic mutation to conceive a disease-free child. We report the first published case of a child born without Leber congenital amaurosis through preimplantation genetic testing to a couple who had a son with a homozygous mutation in the GUCY2D gene.

Surgical treatment of capillary hemangiomas causing amblyopia.

BACKGROUND: Capillary hemangiomas of the eyelids and orbit can cause refractive and occlusive amblyopia. Although oral and intralesional steroid injections are the most common treatment modalities, sometimes they are not successful. There is a paucity of information in the literature on the success of eliminating amblyogenic factors by treating these lesions with surgical resection. METHODS: Retrospective chart review of 10 patients in two pediatric ophthalmology practices who underwent surgical excision of a capillary hemangioma that was causing amblyopia and that had failed to regress with other treatment. RESULTS: Two patients had surgery secondary to pupillary occlusion, which was successful in relieving occlusion. Eight patients had surgery secondary to significant astigmatism. The average preoperative astigmatic difference between the affected and unaffected eye in five of these patients undergoing surgery before the age of 21 months was 2.15 D. The average postoperative astigmatic difference was 0.1 D. The average preoperative astigmatic difference between the affected and unaffected eye in three patients undergoing surgery after 21 months of age was 1.6 D. Surgery completely failed to reduce the astigmatism in two of these patients. The third patient had a decrease of 0.75 D of cylinder but still had a difference of 1.75 D between the two eyes postoperatively. Postoperative complications in this study included wound infection in one patient. CONCLUSIONS: Surgical excision of capillary hemangiomas that were resistant to other modes of treatment was useful in relieving pupillary occlusion and in decreasing the amount of astigmatism if performed before the age of 21 months in our series of patients. Our cases as well as the literature suggest that surgery should be performed at 13 months or earlier to reduce the amount of astigmatism.

Subretinal hemorrhage masquerading as a hemorrhagic choroidal detachment in a case of nonaccidental trauma.

Retinal hemorrhages are the most commonly reported ocular findings in nonaccidental trauma in children. Other reported ocular findings include perimacular folds, traumatic retinoschisis, choroidal hemorrhages, and retinal detachments. We report the clinical and pathologic findings in a case of a 10-month-old boy who sustained nonaccidental trauma and whose clinical presentation was characteristic of a hemorrhagic choroidal detachment. Postmortem examination revealed a large subretinal hemorrhage, with no evidence of choroidal hemorrhage.

Treatment of capillary hemangiomas causing refractive and occlusional amblyopia.

PURPOSE: Capillary hemangiomas of the eyelid and orbit are treated when amblyopia secondary to anisometropic astigmatism or pupillary occlusion is present or when rapid growth of the hemangioma threatens to occlude the pupil. The goal of this study was to determine whether treatment of hemangiomas resolves or prevents occlusion or results in decrease in astigmatism. METHODS: The records of 54 patients who underwent treatment for reduction in the size of a capillary hemangioma causing amblyopia or threatened amblyopia in two pediatric ophthalmology practices were reviewed. RESULTS: Twenty-eight patients were treated for amblyopia due to anisometropic astigmatism. The average amount of pretreatment astigmatism was 2.71 D, while the average amount of post-treatment astigmatism was 0.46 D. Fifteen of these patients could be tested for optotype visual acuity and all had vision acuity of 20/40 or better. Only 1 of the 15 patients treated for threatened occlusion of the pupil developed occlusion. Six of these patients cooperated with optotype visual acuity and all had vision acuity of 20/30 or better. Eleven patients were treated for pupillary occlusion. Occlusion resolved in all cases. Of the five patients treated for occlusion who cooperated with optotype visual acuity, two had a vision acuity of 20/100 or worse. CONCLUSIONS: Treatment to reduce the size of capillary hemangiomas results in resolution of occlusion, reduction in astigmatism, and prevention of pupillary occlusion. Those with occlusion are at higher risk for severe residual amblyopia and require prompt and definitive treatment.

Comparison of clinical findings in pediatric patients with albinism and different amplitudes of nystagmus.

INTRODUCTION: Visual acuity in albinism can vary and is difficult to predict. We undertook this study to investigate whether patients with albinism with absent or minimal nystagmus have better visual acuity and ocular alignment than patients with albinism and obvious nystagmus. METHODS: Our retrospective chart review of 38 pediatric patients with albinism yielded 16 patients with absent or minimal nystagmus and 22 patients with obvious nystagmus. RESULTS: Patients with albinism and absent or minimal nystagmus had best-corrected visual acuities ranging from 20/20 to 20/50, with a mean visual acuity of 20/33. Those patients with albinism and obvious nystagmus had best-corrected visual acuities ranging from 20/30 to 20/200, with a mean visual acuity of 20/80. Visual acuity was significantly better in the group with absent or minimal nystagmus (P < 0.001). Of the 16 patients with absent or minimal nystagmus, 6 were orthophoric and 10 had a strabismic deviation of 12 prism diopters (PD) or less. None of these patients required strabismus surgery. Of the 22 patients with nystagmus, 16 were orthophoric, 1 had a horizontal deviation of less than 12 PD, and 5 had larger angle horizontal deviations ranging from 20 to 50 PD. CONCLUSION: Our findings suggest that pediatric albinism patients with absent or minimal nystagmus can be expected to demonstrate better visual acuity and are less likely to show a strabismic deviation of greater than 12 PD compared with those with obvious nystagmus (P = 0.0003).

Ophthalmologic manifestations in pediatric patients with acute disseminated encephalomyelitis.

INTRODUCTION: Acute disseminated encephalomyelitis (ADEM) is an inflammatory syndrome affecting the central nervous system that often is associated with para-infectious causes. Optic neuritis is a common feature of this demyelinating disease, and a paucity of data regarding this entity exists in the ophthalmologic literature. METHODS: We describe 10 pediatric patients with ADEM who presented at our institution between 1995 and 2003. RESULTS: Six of these patients had optic neuritis, with visual acuities in the affected eyes ranging from 20/30 to light perception. Five of those with optic neuritis had bilateral disease. All patients were treated with high-dose methylprednisolone, and 4 of these patients had recovered with a visual acuity of 20/40 or better in the affected eyes. Two patients were lost to follow-up, and final visual acuities were not available. Nine of 10 patients had an identifiable preceding viral illness or immunization. CONCLUSIONS: ADEM is a known cause of optic neuritis in children and should be considered by the ophthalmologist treating this population of patients. All patients in this series were treated with high-dose intravenous steroids followed by an oral steroid taper and/or intravenous immunoglobulin. Final visual outcomes were favorable in all patients.

Bilateral superior rectus transposition for congenital exotropia associated with anomalous medial rectus muscles.

Superior rectus transposition to the lateral rectus insertion without inferior rectus transposition has been used to correct esotropic deviations secondary to Duane syndrome and abducens nerve palsy. This is usually combined with an augmented posterior fixation suture of the superior rectus muscle to the lateral rectus muscle and ipsilateral medial rectus recession. We report a child with a large-angle congenital exotropia who was found to have anomalous medial rectus muscles bilaterally. Bilateral superior rectus transposition to the medial rectus insertion with bilateral lateral rectus recessions achieved good ocular alignment in primary position.

Esotropia in Children with Ventricular-Peritoneal Shunts.

PURPOSE: Compared with the general population, patients with hydrocephalus are more likely to have strabismus. This study was undertaken to examine characteristics and outcomes of children with esotropia and ventricular-peritoneal shunt placement due to hydrocephalus. METHODS: This is a retrospective chart review of all pediatric patients with esotropia and a history of ventricular-peritoneal shunt placement seen by our pediatric ophthalmology service between January 2000 and December 2010. RESULTS: Sixteen patients between the age of 3 months and 5.6 years met study criteria. Nine were premature and all but one of the patients had developmental delay. Although all patients had a ventricular-peritoneal shunt, the diagnosis leading to shunt placement was intraventricular hemorrhage or congenital hydrocephalus in 75% of the patients. In all but 3 patients the hydrocephalus was diagnosed before the esotropia. Ten children had congenital esotropia and 6 had acquired esotropia. Eleven of the 16 children required glasses: 5 had a myopic prescription and 6 had a hyperopic prescription. Treatment of the esotropia resulted in 9 patients (56%) with successful ocular alignment (<10 prism diopters) on their last visit: 7 underwent strabismus surgery and 2 were treated with glasses only. Of the 9 patients who had strabismus surgery, 6 had congenital esotropia and 3 had acquired esotropia. Among patients who underwent strabismus surgery, 78% had successful ocular alignment at their last visit. CONCLUSIONS: While acquired accommodative esotropia is more common in the general population, children with ventricular-peritoneal shunts may be more likely to have congenital esotropia. Although developmental delay is very frequent, successful ocular alignment may be possible in this patient population.

Recurrent Staphylococcus aureus chalazia in hyperimmunoglobulinemia E (Job's) syndrome.

PURPOSE: To report a case of recurrent Staphylococcus aureus chalazia in a patient with hyperimmunoglobulinemia E syndrome (Job's Syndrome). DESIGN: Case report. METHODS: Three separate surgical incisions and curettages of multiple, recurrent chalazia of the right upper eyelid were performed over a course of 3 months. Cultures and pathologic specimens were obtained. Postoperative treatment consisted of oral erythromycin and amoxicillin/clavulonate, topical tobramycin/dexamethasone ointment, and warm compresses. RESULTS: Pathology of the tarsus confirmed the diagnosis of multiple chalazia. Cultures of the chalazia contents were positive for Staphylococcus aureus. No further recurrence was observed following the third surgical procedure over a 3-month follow-up period. CONCLUSION: Characteristic Staphylococcus aureus skin infections in immunodeficient patients with hyperimmunoglobulinemia E syndrome can involve the eyelids and may be recurrent despite appropriate medical and surgical therapy.

Ocular manifestations of Noonan syndrome in the pediatric patient.

Noonan syndrome is a rare syndrome with both ocular findings and multiple organ involvement. We retrospectively identified all of the pediatric patients with Noonan syndrome seen at our institution and reviewed the ocular findings to determine specific ocular manifestations in pediatric Noonan syndrome patients.

Time of onset of uveitis in children with juvenile rheumatoid arthritis.

BACKGROUND: Recently, it has been advocated to decrease the frequency of eye examinations to screen for uveitis in children with juvenile rheumatoid arthritis (JRA) because of the low yield of positive findings after an initial normal eye examination. This study was undertaken to determine the time interval for the development of uveitis after the diagnosis of JRA and to further describe patients who develop uveitis related to JRA. METHODS: This was a retrospective chart review of all patients with JRA examined by either of 2 pediatric ophthalmologists from August 1984 to June 2001. All patients were also under the care of the Pediatric Rheumatology Division at Schneider Children's Hospital. Age of diagnosis of JRA, disease onset subtype of JRA, antinuclear antibody titers, age of diagnosis of uveitis, and complications from uveitis were recorded. RESULTS: One hundred fifty eight patients with JRA had eye examinations; 39 (25%) developed uveitis. Sixteen patients had uveitis on the initial eye examination, and 23 subsequently developed uveitis. When uveitis was absent at the initial eye examination, the mean time to develop it was 20 months (range, 4-81 months). CONCLUSIONS: A normal initial eye examination does not preclude the development of uveitis in patients with JRA. We recommend continuing the current standards of ophthalmologic examinations to screen for uveitis in children with JRA as prescribed by the Section on Rheumatology and Ophthalmology of the American Academy of Pediatrics.

Intracranial infection associated with preseptal and orbital cellulitis in the pediatric patient.

PURPOSE: To identify risk factors in children admitted with preseptal or orbital cellulitis with associated intracranial infection. METHODS: A retrospective chart review identified 10 patients (< or = 18 years) with a diagnosis of preseptal or orbital cellulitis and a concurrent or subsequent diagnosis of intracranial infection. RESULTS: Diagnoses confirmed by imaging included sinusitis (n = 10), preseptal cellulitis (n = 4), orbital cellulitis (n = 6), orbital subperiosteal abscess (n = 5), Pott's puffy tumor (n = 4), epidural empyema (n = 2), epidural abscess (n = 6), and brain abscess (n = 2). The timing of diagnosis of intracranial infection ranged from hospital day 1 to 21. All but 1 patient had positive microbial cultures. Seven of 10 patients had positive microbial cultures from two or more sites, 70% of which were polymicrobial; Streptococcus species and Staphylococcus species were the most commonly isolated bacterial pathogens. All patients required both medical and surgical therapy; all 10 patients underwent sinus surgery; 8 patients required neurosurgical craniotomy; and 5 patients underwent orbital surgery. There were no deaths. CONCLUSION: Intracranial involvement should be suspected in any patient age > or = 7 years with preseptal or orbital cellulitis associated with orbital subperiosteal abscess, Pott's puffy tumor, concurrent sinusitis, complaints of headache, and continuing fever despite intravenous antibiotics. Given the high incidence of polymicrobial infection found on cultures in this series, broad-spectrum antibiotics are strongly indicated. When imaging the orbits and sinuses in such patients, we recommend including the brain to rule out intracranial involvement.

Shaken baby syndrome without intracranial hemorrhage on initial computed tomography.

OBJECTIVE: We sought to describe the unique characteristics of children diagnosed with shaken baby syndrome (SBS) despite the absence of intracranial hemorrhage on cranial computerized tomography (CT) on hospital admission. METHODS: Using an international e-mail-based listserv for professionals with an interest in child abuse, we identified and reviewed the charts of children hospitalized in different medical centers who were diagnosed with SBS although CT disclosed no signs of intracranial bleeding. Children with normal imaging were not included. RESULTS: Eight cases were identified. All children had cerebral edema in CT, which was severe on 7/8 cases (88%). All of these children had extensive retinal hemorrhage. The prognosis was poor; 5/8 infants died (63% mortality), and the rest had permanent neurologic damage. CONCLUSION: The diagnosis of SBS can be established even when CT at presentation does not demonstrate intracranial hemorrhage. We hypothesize that rapidly developing cerebral edema may cause increased intracranial pressure and tamponade that prevents the accumulation of intracranial blood. The prognosis in these cases is grave.