Ophthalmic Manifestations of Unilateral Coronal Synostosis.

PURPOSE: To summarize the ophthalmic manifestations of unilateral coronal synostosis patients. METHODS: We performed a literature search in the electronic database of PubMed, CENTRAL, Cochrane, and Ovid Medline guided by Preferred Reporting Items for Systematic Reviews and Meta-Analysis Statement for studies evaluating ophthalmic manifestations of unilateral coronal synostosis. RESULTS: Unilateral coronal synostosis, also called unicoronal synostosis, may be mistaken for deformational plagiocephaly, an asymmetric skull flattening common in newborns. Characteristic facial features, however, distinguish the two. Ophthalmic manifestations of unilateral coronal synostosis include a "harlequin deformity", anisometropic astigmatism, strabismus, amblyopia, and significant orbital asymmetry. The astigmatism is greater on the side opposite the fused coronal suture. Optic neuropathy is uncommon unless unilateral coronal synostosis accompanies more complex multi-suture craniosynostosis. In many cases, surgical intervention is recommended; without intervention, skull asymmetry and ophthalmic disorders tend to worsen with time. Unilateral coronal synostosis can be managed by early endoscopic stripping of the fused suture and helmeting through a year of age or by fronto-orbital-advancement at approximately 1 year of age. Several studies have demonstrated that anisometropic astigmatism, amblyopia, and severity of strabismus are significantly lower after earlier intervention with endoscopic strip craniectomy and helmeting compared to treatment by fronto-orbital-advancement. It remains unknown whether the earlier timing or the nature of the procedure is responsible for the improved outcomes. As endoscopic strip craniectomy can only be performed in the first few months of life, early recognition of the facial, orbital, eyelid, and ophthalmic characteristics by consultant ophthalmologists enables expeditious referral and optimized ophthalmic outcomes. CONCLUSION: Timely identification of craniofacial and ophthalmic manifestations of infants with unilateral coronal synostosis is important. Early recognition and prompt endoscopic treatment appears to optimize ocular outcomes.

Paediatric anterior uveitis management in the USA: a single-centre, 10-year retrospective chart review exploring the efficacy and safety of systemic immunomodulatory therapy.

OBJECTIVE: To evaluate the efficacy of immunomodulatory therapy (IMT) in paediatric anterior uveitis. METHODS: Chart review of all patients ≤ 18 years treated for anterior uveitis using a stepladder approach during a 10-year period. The type and duration of IMT were noted. The data were analysed depending on chronicity, aetiology, and type of IMT using appropriate statistical tests. The outcome measures included ocular complications, the need for surgical intervention, and visual outcomes. RESULTS: One hundred and thirty-four patients (191 eyes) were analyzed. The median age at diagnosis was 7 years (interquartile range (IQR): 7.5 years). The median follow-up was 4 years (IQR: 6 years). The most common causes of anterior uveitis were Juvenile idiopathic arthritis (64 patients, 47.8%) and undifferentiated (33 patients, 24.6%). All patients were started on topical steroids and cycloplegics. 94 (70%) patients required IMT. 92 (68.6%) were started on Methotrexate as the first agent, of which 21 (22%) were switched to a different agent owing to side effects. Biologic agent was added in 55 (41%) patients. 21 (16%) required switch to a second biologic agent, 5 (3.7%) to third, and 1 (0.8%) to fourth biologic agent. At the last exam, 11 (8%) had persistent inflammation. 55 (41%) had ocular complications, and 113 (84%) had a best corrected visual acuity ≥ 20/40. CONCLUSION: Early introduction of IMT and switch to different agents may be required to control anterior uveitis and reduce the complications in children. IMT is safe and effective in treating paediatric anterior uveitis.

Francis Fontan (1929-2018): Pioneer pediatric cardiac surgeon.

Up until the mid-1900s, tricuspid atresia - a birth defect of the tricuspid valve, was once categorized as a "death sentence." The challenge of achieving positive health outcomes for affected patients was compounded by a hesitancy to operate on children. The main concern was safely administering anesthesia to young patients who were going through a strenuous operation that was often poorly tolerated. Despite these assumed limitations, Francis Fontan, a pediatric cardiothoracic surgeon at the Hospital of Tondu in Bordeaux, was able to redirect blood flow from the superior and inferior vena cava to the pulmonary arteries in 1971, which elucidated the process of advancing clinical practice in medicine. With the support of mentors and a firm belief in this new technique, Fontan pioneered his eponymous procedure and ultimately paved the way for modern cardiovascular surgical techniques that helped to prolong the life of those with single functioning ventricles. The aim of this study is to examine the genesis and the evolution of the Fontan procedure to elucidate the process of advancing clinical practice in medicine by utilizing personal interviews, Fontan's works, associated primary and secondary sources in the context of 20th century cardiothoracic surgery and innovations.

Down syndrome: a review of ocular manifestations.

Down syndrome is the most common genetically mediated intellectual disability. Although many physiologic and pathologic features of Down syndrome are discussed at length in the literature, the ocular manifestations of Down syndrome have seldom been discussed in a comprehensive fashion. Given that Down syndrome has ocular manifestations from the front to the back of the eye, it is important for physicians to become familiar with these manifestations, especially given the prevalence of Down syndrome. This review aims to discuss the varied ophthalmologic manifestations of Down syndrome - including strabismus, amblyopia, nystagmus, accommodation deficits, nasolacrimal duct obstruction, keratoconus, optic nerve pathology, neoplastic disease, and retinal pathology - to facilitate better care and visual outcomes in this important patient population.

Long-term ophthalmic outcomes in 120 children with unilateral coronal synostosis: a 20-year retrospective analysis.

BACKGROUND: Prior studies comparing ophthalmic outcomes after treating unicoronal synostosis (UCS) by early endoscopic strip craniectomy (ESC) versus later fronto-orbital advancement (FOA) are modest in sample size, or lack consistent age adjustment. We report long-term, age-adjusted ophthalmic outcomes for a large cohort after nonrandomized treatment by one of these two options. METHODS: The following data was retrieved from a retrospective review of the medical records of patients with treated UCS born since 2000: cycloplegic refractions, sensorimotor examinations, and strabismus procedures before craniofacial repair and postoperatively at approximately 18 and 60 months of age. V-pattern strabismus was graded as mild (absent or + 1/-1 oblique dysfunction) versus moderate-to-severe (≥+2/-2 oblique dysfunction or left to right vertical alignment change of ≥20Δ or ocular torticollis >15°). RESULTS: A total of 120 infants were included: 60 treated by FOA and 60 by ESC. By the late examination, aniso-astigmatism was present in 72% of FOA-treated patients and 46% of ESC-treated patients (P < 0.0001). By late examination, the age-adjusted odds ratio of moderate-to-severe V-pattern strabismus after treatment by FOA versus ESC was 2.65 (95% CI, 1.37-6.28; P = 0.02); strabismus surgery was performed in 26 infants treated by FOA compared with 13 treated by ESC (OR = 2.8; P = 0.02). Amblyopia developed in 60% of FOA-treated patients compared with 35% of those treated by ESC (OR 3.0; 95% CI, 1.3-6.7; P = 0.02). CONCLUSIONS: Our age-adjusted ophthalmic results confirm better long-term outcomes after treatment of USC by endoscopic strip craniectomy. Recognition and referral of affected infants by the earliest months of life facilitates the opportunity for endoscopic repair.

Evaluation and Management of V pattern Strabismus in Craniosynostosis.

V pattern strabismus is the most common ocular motor disorder reported in patients with craniosynostosis. Strabismus management may prove challenging, and few studies provide perspective on surgical approach. The purpose of this review is to discuss evaluation and surgical options for treating V pattern strabismus in patients with craniosynostosis. We provide a step-by-step approach to facilitate surgical planning.