Doppler Ultrasonography of the Central Retinal Vessels in Children With Brain Death.

OBJECTIVE: The purpose of this observational study is to explore if bedside Doppler ultrasonography of the central retinal vessels has the potential to become an ancillary study to support the timely diagnosis of brain death in children. DESIGN: Seventeen-month prospective observational cohort. SETTING: Forty-four bed pediatric medical and surgical ICU in an academic teaching hospital. PATIENTS: All children 0-18 years old who were clinically evaluated for brain death at Children's National Health Systems were enrolled and followed until discharge or death. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: All patients had at least one ophthalmic ultrasound within 30 minutes of each brain death examination. The central retinal artery peak systolic blood flow velocity, resistive index, pulsatility index, and Doppler waveforms were evaluated in each patient. Thirty-five ophthalmic ultrasounds were obtained on 13 patients, 3 months to 15 years old, who each had two clinical examinations consistent with brain death. The average systolic blood pressure during the ultrasound examinations was 102 mm Hg (± 28), diastolic blood pressure 65 mm Hg (± 24), mean arterial pressure 79 mm Hg (± 23), heart rate 133 beats/min (± 27), temperature 36°C (± 0.96), arterial CO2 35 mm Hg (± 9), and end-tidal CO2 23 mm Hg (± 6). For all examinations, the average peak systolic velocity of the central retinal artery was significantly decreased at 4.66 cm/s (± 3.2). Twelve of 13 patients had both resistive indexes greater than or equal to 1, average pulsatility indexes of 3.6 (± 3.5) with transcranial Doppler waveforms consistent with brain death. Waveform analysis of the 35 ultrasound examinations revealed 11% with tall systolic peaks without diastolic flow, 17% with oscillatory flow, 29% showed short systolic spikes, and 23% had no Doppler movement detected. A rippling "tardus-parvus" waveform was present in 20% of examinations. CONCLUSION: This study supports that the combination of qualitative waveform analysis and quantitative blood flow variables of the central retinal vessels may have the potential to be developed as an ancillary study for supporting the diagnosis of brain death in children.

The Use of Ophthalmic Ultrasonography to Identify Retinal Injuries Associated With Abusive Head Trauma.

Abusive head trauma includes any nonaccidental injury inflicted to a child's head and body. It is often characterized by, but not limited to, the repetitive acceleration-deceleration forces with or without blunt head impact. It has a mortality rate of 30%, and 80% of survivors experience permanent neurologic damage. In this case series, we hypothesize that bedside ultrasonography can be useful in the identification of retinal injuries that are consistent with abusive head trauma. Ocular manifestations of abusive head trauma are identified by dilated ophthalmic examination showing retinal hemorrhages that are too numerous to count, multilayered, and extending to the periphery. Traumatic retinoschisis, splitting of the retinal layers with or without blood accumulating in the intervening space, is exclusive for abusive head trauma in infants without a history of significant cerebral crush injury. Direct visualization of intraocular structures is difficult when the eyelids are swollen shut or when dilatation must be delayed. We present a series of 11 patients with brain injuries who underwent ophthalmic point-of-care ultrasonography that revealed traumatic retinoschisis on average 60 hours earlier than direct ophthalmic visualization. Dilated ophthalmic examinations and autopsy reports confirmed retinoschisis and other forms of retinal hemorrhages that were too numerous to count, multilayered, and extending to the periphery in all 11 patients. One patient did not have a dilated ophthalmic examination; however, traumatic retinoschisis and retinal hemorrhages were confirmed on autopsy. Ocular point-of-care ultrasonography is a promising tool to investigate abusive head trauma through the identification of traumatic retinoschisis and retinal hemorrhages when pupillary dilatation and direct ophthalmic examination is delayed.

Arachnoid cyst resulting in sixth nerve palsy in a child.

A 2 year old presented with incomitant esotropia and abduction deficit consistent with sixth nerve palsy. Neuroimaging revealed an arachnoid cyst on the left. Neurosurgical shunting followed by strabismus surgery relieved the abduction deficit and esotropia. An arachnoid cyst may be a rare cause of acquired sixth nerve palsy and strabismus in children.

Solitary orbital infantile myofibroma: a case report and review of the literature.

Infantile myofibromatosis is a benign condition involving the skin, viscera, or bone that presents rarely in the orbit where rapid growth and bony destruction can mimic malignant tumors. Lesions can be solitary or scattered throughout the body. Growth in viscera such as the lungs can lead to death. Herein, we present a case of a solitary orbital myofibroma in a young child.

Considering the Ectopic Pituitary Gland in Evaluation of the Nasopharyngeal Mass.

IMPORTANCE: Extracranial pituitary ectopia is an uncommon finding characterized by all or a portion of the pituitary gland situated in an aberrant location. Often these lesions come to clinical attention only once they begin to function abnormally or increase in size significantly. OBSERVATIONS: We describe an incidentally noted, asymptomatic pedunculated nasopharyngeal lesion in a 13-month-old girl that may be an extracranial pituitary gland. Consideration of this rare anomaly cautioned against performing a biopsy and may have prevented inadvertent removal of functional pituitary tissue. We describe the embryologic origins of ectopic pituitary tissue, discuss the differential diagnosis for nasopharyngeal lesions in children, and emphasize physical and radiologic findings suggestive of ectopic pituitary tissue to prevent potential inadvertent removal of this rare anomaly. CONCLUSIONS AND RELEVANCE: We report this unusual case to review embryologic origins of ectopic pituitary tissue and to alert otolaryngologists of the need to consider ectopic pituitary tissue in the diagnosis of nasopharyngeal lesions in children.

Corneoscleral transplantation in congenital corneal staphyloma and Peters' anomaly.

An infant born with bilateral corneal clouding diagnosed clinically as congenital anterior staphyloma and Peters' anomaly was confirmed histopathologically. This case report demonstrates one clinical spectrum of Peters' anomaly. The clinical course and histopathologic findings are detailed as is a unique surgical approach of corneoscleral graft used to perserve the right globe.

Risk and clinical course of retinopathy of prematurity in 78 infants of gestational age 22-25 weeks.

PURPOSE: To characterize the retinopathy of prematurity (ROP) and survival of infants born at gestational age (GA) of 22-25 weeks. METHODS: This study was a comparative case series for the total set of 78 infants ≤25 GA screened for ROP at a level IV NICU during a 21-month period. Data are presented on infants screened for ROP from 6 weeks after birth for 22 and 23 weeks' GA infants and from 5 weeks after birth for 24 and 25 weeks' GA. Accounting for the competing risk of mortality, we implemented Cox CR regression models to assess birth weight, GA, and admission diagnosis as potential risk factors for the following time to event outcomes: type 1 disease, aggressive posterior ROP (AP-ROP), plus disease, first presentation of ROP, and worst ROP observed. RESULTS: Risk of laser treatment (subhazard ratio [SHR] = 0.56, P = 0.007) and of plus disease (SHR = 0.49, P = 0.001) was increased among those born at lower GA. Twenty infants required laser for type 1 disease at median postmenstrual age (PMA) of 35.8 weeks (range, 33.0-42.7); infants with AP-ROP had laser at PMA of 34.5 weeks (range, 33.0-36.9), 2 weeks earlier than infants without AP-ROP at PMA 36.5 weeks (range, 33.9-42.7). The cumulative probability of receiving laser therapy approached 46% (22 or 23 weeks' GA), 30% (24 weeks' GA), and 18% (25 weeks' GA). CONCLUSIONS: The 2013 screening guidelines appear to be appropriate for infants of 22 and 23 weeks' GA when ROP screening begins at PMA 31 weeks.

Uveitis in Blau syndrome from a de novo mutation of the NOD2/CARD15 gene.

Blau syndrome (MIM 186580) is a rare autoinflammatory, familial granulomatous condition that occurs secondary to a single amino acid mutation of the NOD2/CARD15 gene on chromosome 16p12-q21. We report the case of a 2.5-year-old girl who presented for ophthalmic examination in the setting of rash and synovitis. Initially, small, evanescent, ovoid corneal subepithelial opacities unique to Blau syndrome were observed. She later developed a fulminant panuveitis that responded to immunomodulatory therapy. Subsequent genetic testing confirmed the diagnosis of Blau syndrome. Despite immunosuppression, at almost 7 years of age, she continues to have persistent panuveitis with vision of 20/20.

Primary follicular lymphoma of the conjunctiva in a 6-year-old child.

Primary follicular lymphomas arise in the lymph nodes or spleen or in extranodal sites. Ocular adnexal tumors are rare. We report the case of a 6-year-old boy with a 3-mm conjunctival mass. The lesion was resected with clean surgical margins. The child has been closely monitored and has remained cancer free for 3 years.

Blood flow velocities are reduced in the optic nerve of children with elevated intracranial pressure.

The authors previously used spectral Doppler imaging to determine optic nerve blood flow velocities in normal children. In the current study, we measured central retinal artery and central retinal vein blood flow velocities by spectral Doppler imaging in 38 healthy children and 18 children with elevated intracranial pressure between ages 4 and 17. We found central retinal artery systolic blood flow velocity was significantly reduced in children with elevated increased intracranial pressure; ANOVA P = .01 (normal children 8.9 cm/s [SD 1.1] versus children with elevated intracranial pressure 7.5 cm/s [SD 1.3]). Central retinal vein maximal blood flow velocity was also significantly reduced in children with elevated intracranial pressure; ANOVA P < .02 (normal children 4.2 cm/s [SD 0.9] versus children with elevated intracranial pressure 3.6 cm/s [SD 0.7]). Spectral Doppler imaging is a noninvasive test well tolerated in children that identifies blood flow velocity changes in elevated intracranial pressure.