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Page 1: Reliability of magnetic resonance imaging for diagnosis of hypopituitarism in children with optic nerve hypoplasia

Volume 14 Number 1 / February 2010 e27

and 2008 with Ophthalmology consultation. Patients with history oftrauma, external infection source, immunocompromised status, orcraniofacial surgeries were excluded. Variables analyzed includeproptosis and extraocular motility (EOM) as assessed by both PEDphysicians and consultants, and vision, presence of RAPD, dyschro-matopsia, and intraocular pressure by consultants. Primary outcomewas subperiosteal abscess or higher using Chandler's criteria of ra-diologic imaging (class $3). Agreement between consultant and PEDphysicians and odds ratio for measured variables were computed us-ing chi square.Results: 120 cases were analyzed; 58 had an abscess. Ophthalmologyidentified proptosis in 38 cases, 30 had an abscess (OR-16.9 95%CI 5.2-54.9); PED physician identified proptosis in 19 cases, 13 had an abscess(OR-2.5 95%CI 0.867- 7.164). Ophthalmology identified limited EOM in 48cases, 35 had an abscess (OR-6.4 95%CI 2.7-15.2); PED physician iden-tified abnormal EOM in 79 cases, 44 had an abscess (OR-2.4 95% CI 1.1-5.3). Agreement between Ophthalmology and PED physicians was pooron proptosis [k 5 0.27] and EOM [k\0]. IOP asymmetry of $5 mm Hgwas a predictor for abscess [OR-1.24 95% CI 1.03-1.49].Conclusions: When assessing for intra-orbital abscess, Ophthalmol-ogy consultation improved the accuracy and prediction power ofclinical variables assessed by PED physicians, and provided addi-tional important predictors not assessed by PED physicians.

103 Are retinal findings in abused children different from thosewho suffer accidental head trauma? Results of a systematicreview. Andrew D. Shaw, Patrick Watts, Sabine Maguire, SamHolden, Mala Mann, Alison KempIntroduction: The differentiation between cases of non-abusive andabusive head trauma may be complicated with increased relianceon clinical findings. We have used a systematic review to definethe distinguishing characteristics of abusive retinal hemorrhages(RH) versus non abusive RH in children with head trauma.Methods: A literature search of 12 databases, conference abstractsand references from 1950 to 2009 identified 8,422 potential studies,337 underwent two independent reviews by pediatric, ophthalmologyand pathology specialists, using standardised critical appraisal. In-clusion: primary studies of live children \11 years with RH, due toexplicitly confirmed abusive or non-abusive head trauma, with oph-thalmologist examination and no retinal comorbidity.Results: Sixty-two studies were included. Thirteen larger studieswith consecutive case ascertainment of either abusive head trauma,nonabusive head trauma, or both contained data of sufficient qualityfor further combined analysis. Within these studies, the prevalenceof retinal findings in abusive head trauma was 78% and in nonabusivehead trauma, 5.3%.Discussion: When described, RHs were more commonly bilateral,multilayered and extending to the periphery in the abused group.Schisis cavities and retinal folds were more often reported in abusivehead trauma cases. These characteristics were rarely described innon-abusive head trauma.Conclusions: Nonabusive head trauma is a rare cause of RH. Whenpresent, compared to those in abusive head trauma, are frequentlyunilateral, few and restricted to the posterior pole.

104 The incidence and clinical findings of congenitaldacryocystocele in a population-based cohort. Julia Shekunov,Gregory J. Griepentrog, Nancy N. Diehl, Brian G. MohneyIntroduction: To report the incidence, clinical findings, and outcomesin children diagnosed with congenital dacryocystocele in a well-de-fined population over a 20-year period.

Journal of AAPOS

Methods: The medical records of all patients diagnosed with con-genital dacryocystocele from January 1, 1988, to December 31,2007, were retrospectively reviewed.Results: A total of 9 children were diagnosed with dacryocystoceleduring the 20-year period, yielding an incidence of 1 in 3884 livebirths. The median age at diagnosis was 12 days (range, birth to 40days), and seven (78%) were female. Eight patients (89%) had unilat-eral disease. Clinical findings included a cystic mass in all 9, dacryo-cystitis in 3 (33%), intranasal cysts in 3 (33%), and 1 (11%) each withfacial cellulitis and dacryocystocele-induced astigmatism. Conserva-tive treatment resolved the obstruction in 3 (33%), while the remain-ing 6 (67%) each underwent one surgery. Complete resolution wasobserved in all 9 patients.Conclusions: Congenital dacryocystocele occurred in approximately1 in 3,884 live births in this population. The majority of patients werefemale with unilateral disease. Complications such as dacryocystitisor intranasal cysts occurred in 56%, while most (67%) required sur-gical intervention to resolve the obstruction.

105 Reliability of magnetic resonance imaging for diagnosis ofhypopituitarism in children with optic nerve hypoplasia. JulieShelton, Raghu H. Ramakrishnaiah, Charles M. Glasier, Paul H. PhillipsIntroduction: The purpose of our study was to determine the reliabilityof pituitary abnormalities on magnetic resonance imaging (MRI) as anindicator of hypopituitarism in children with optic nerve hypoplasia.Methods: Retrospective analysis of 41 children with optic nerve hypo-plasia evaluated at Arkansas Children's Hospital between 2002 - 2009.All patients had MRI of the brain/sella interpreted by a neuroradiologistmasked to the patient's endocrinologic status. All patients had clinicalevaluation by a pediatric endocrinologist and a standardized panel ofserologic testing that included a complete blood count, electrolytes,cortisol, ACTH, TSH, Free T4, IGF-1, IGF-BP3 levels.Results: Among the 41 children, 16 were diagnosed with hypopituita-rism. All 16 patients had pituitary abnormalities on MRI that includedabsence of the pituitary infundibulum, ectopic location or absence ofthe posterior pituitary gland, and pituitary gland hypoplasia. Amongthe 25 patients with normal endocrinologic function, only 2 had pituitaryabnormalities on MRI (thin infundibulum and absence of infundibulum).Discussion: Although we have described the association of pituitaryabnormalities on MRI and hypopituitarism in children with opticnerve hypoplasia, the diagnostic evaluation was not standardizedin our previous studies.1-2 In the current study, all of the children re-ceived an MRI of the pituitary interpreted by a masked neuroradiol-ogist and clinical evaluation by a pediatric endocrinologist withextensive serologic testing.Conclusions: Pituitary abnormalities on MRI are sensitive (100%) andspecific (92%) indicators of hypopituitarism in children with opticnerve hypoplasia.

106 Ophthalmic manifestations in mucolipidosis III. Melissa G.Shipley, Kali B. Cole, Sara CatheyIntroduction: Mucolipidosis III is a rare lysosomal storage disorderthat can manifest specific ocular findings. These include cornealclouding, hyperopic astigmatism, retinal dystrophy, and optic nerveedema. The current data represents the largest report of ophthalmicmanifestations of the disease in the literature to date.Methods: The eye examinationinations of ten patients with docu-mented mucolipidosis III based upon genetic testing and clinical find-ings were reviewed. Primary data points included: demographics,past medical history, visual acuity, cycloplegic refraction, intraocularpressure, color vision, pupillary examination, motility examination, an-terior segment examination, and dilated fundus examination.