Funduscopic lesions associated with mortality in shaken baby syndrome.

PURPOSE: The shaken baby syndrome (SBS) has been defined as a syndrome of intraocular and intracranial hemorrhage in young children, thought to be caused by violent shaking inflicted by an adult. In many cases SBS is fatal as a result of intracranial injury. Intraocular findings include hemorrhage, which may be accompanied by characteristic retinal folds or retinoschisis lesions. This study was performed to determine whether acute ophthalmologic findings might predict a fatal outcome. METHODS: A consecutive series of 10 patients meeting a strict definition of SBS was reviewed for ophthalmic findings at presentation and outcome. RESULTS: Seven patients survived, and three died. Of the six funduscopic characteristics identified in these patients, two were significantly associated with a fatal outcome: circular perimacular retinal folds found in four patients (p = 0.048) and peripheral retinoschisis lesions seen in three patients (p = 0.012). Lack of visual response at initial examination was also significantly associated with a fatal outcome (p = 0.033). CONCLUSIONS: Ophthalmic examination of children with suspected SBS is important for prognostic as well as diagnostic purposes. Circular perimacular retinal folds, peripheral retinoschisis lesions, and lack of visual response correlated with fatal neurologic trauma and may be useful in predicting severity of central nervous system injury in shaken baby syndrome.     

Unilateral retinal hemorrhages in shaken baby syndrome.

PURPOSE: To present a detailed series of patients with unilateral retinal hemorrhages in shaken baby syndrome. METHODS: Patients with a diagnosis of Shaken Baby Syndrome evaluated by the Ophthalmology Department at Penn State University between January 1999 and January 2004 were reviewed. Patients were identified and their medical records, photographs, and computed tomographic scans were reviewed. Additional records were obtained from the Children's Hospital of Philadelphia. RESULTS: Of the 12 patients evaluated at Penn State University, the age at presentation ranged from 6 weeks to 15 months, with an average age of 4.3 months. Six (50%) had bilateral intracranial hemorrhage, while 6 (50%) had unilateral intracranial hemorrhage. The six patients with unilateral intracranial hemorrhage all had ipsilateral retinal hemorrhages. The perpetrators were male (100%) and 11 (92%) were the babies' fathers. For the five patients evaluated at Children's Hospital of Philadelphia, the age at presentation ranged from 4 weeks to 1 year, with an average of 4.35 months. Three (60%) had bilateral intracranial hemorrhage and two (40%) had unilateral intracranial hemorrhage. The perpetrators were male in four (80%) cases and three (60%) were the babies' fathers. CONCLUSIONS: These patients demonstrate the varied presentations of shaken baby syndrome. Unilateral retinal hemorrhages do not rule out the diagnosis of shaken baby syndrome.     

Manifestations of the shaken baby syndrome

Retinal hemorrhages are the most common fundus finding in the shaken baby syndrome. They vary in type and location; no particular type is pathognomonic for the condition. Retinal hemorrhages are not needed to make a diagnosis of shaken baby syndrome. However, in a child under age 3 years, the presence of extensive bilateral retinal hemorrhages raises a very strong possibility of abuse, which must be investigated. The other possible causes for hemorrhages in this age child can be investigated and eliminated. The diagnosis of abuse should be made by someone particularly trained in this area, who can put together the entire picture of inadequate or changing history, fractures of various ages, particularly rib fractures, subdural hematoma of the brain, and retinal hemorrhages. Photographs of retinal hemorrhages are very helpful to child advocacy experts who take these cases to court.     

保留晶状体的玻璃体切除术治疗婴儿摇荡综合征

目的：分析婴儿摇荡综合征（ SBS）患者黄斑前及玻璃体出血遮挡黄斑的眼部特征及视力。患者均接受保留晶状体的玻璃体切除术（ LSV）。方法：回顾性研究。选取2010年至2012年确诊为黄斑前及玻璃体出血遮挡黄斑的SBS并接受LSV的患者,对其眼部特征和人口统计学数据进行分析。均数的比较采用配对t检验,分析分类数据采用Fisher精确检验和Pearson卡方检验。 P＜0．05有统计学意义。结果：共32例患者纳入本研究,平均年龄为5．09±1．96mo。在本研究中,21例（65．6％）患者视力为无光感。患者瞳孔较大者初始视力较差（P＝0．021）,且大部分接受过神经外科手术（ P＝0．027）。行LSV的平均间隔为28．56±20．83 d。我院玻璃体切除术的并发症发生率为4．26％。术后26例（80％）患者视力为光感或更好,术后等效球镜大多为近视（ P＝0．001）。结论：SBS患者的眼科评估对于最佳视觉预后非常重要。对于未清除眼内出血致黄斑模糊的SBS患者,LSV的并发症发生率较低,不失为早期手术治疗中的好选择。     

Retinal folds in the shaken baby syndrome

We examined two children with presumed shaken baby syndrome. Both children suffered severe, indirect closed head trauma with intracranial hemorrhage, sharply increased intracranial pressure, and extensive neurologic damage. In addition to extensive retinal and preretinal hemorrhages, bilateral symmetric white ring-shaped retinal folds were seen encircling the macula outside the vascular arcades. These retinal folds may be a hallmark of shaking injuries in child abuse victims.     

Postmortem orbital findings in shaken baby syndrome

PURPOSE: To compare postmortem orbital findings in pediatric accidental head injury to Shaken Baby Syndrome (SBS). DESIGN: Retrospective study. METHODS: Setting: Institutional. Study population: Thirty-six patients underwent postmortem modified exenteration with sectioning of the orbital contents; 18 victims of SBS and 18 cases of fatal accidental head trauma. Observation procedure: In all cases of children who died from accidental head trauma, the orbital tissues were separated to expose the optic nerve sheath. Patients with gross evidence of hemorrhage within the sheath were included. All cases of SBS were included. After accidental head injury, exenteration was performed only if optic nerve sheath hemorrhage was suspected on gross examination. All children younger than 18 years old with head injury as primary cause of death were included. SBS is defined as having at least two of the following: (1) typical abnormal findings on neuroimaging, (2) typical skeletal injury, (3) retinal hemorrhages, (4) history of abusive shaking with or without blunt head trauma, or (5) an inadequate history to explain the observed injuries. Main outcome measure: Presence or absence of orbital hemorrhage. RESULTS: Orbital tissue injury is more common in SBS than accidental head trauma without orbital fracture. In addition, optic nerve sheath and optic nerve intradural hemorrhage are also significantly more common in SBS (P < .0001). CONCLUSIONS: Our study reports new evidence of injury to orbital tissues in SBS and supports the concept that these finding are due to unique acceleration-deceleration forces of this type of abusive head injury.     

Update on shaken baby syndrome: ophthalmology

PURPOSE OF REVIEW: Shaken baby syndrome is a common problem with a high morbidity and mortality. Ophthalmologists help manage this condition and therefore must keep abreast of current advances. RECENT FINDINGS: Clinical updates include the discovery that retinal folds and traumatic retinoschisis can very rarely occur after crush head injury, but remain specific for shaken baby syndrome in other scenarios. Pathology updates include new studies on orbital histology and woodpecker anatomy that suggest the retinal and optic nerve hemorrhages in shaken baby syndrome are caused by shaking itself rather than secondary to intracranial pathology. Regarding this shaking injury, some primary prevention strategies have proven surprisingly effective. In the near future, serum biomarkers may be used as a screening tool for inflicted neurotrauma. Animal models such as the neonatal pig and computer models using finite element analysis are promising experimental techniques for studying shaken baby syndrome. Finally, child abuse recently became an accredited subspecialty of pediatrics, which will lead to further advances in patient care, education, research and prevention. SUMMARY: Ophthalmologists play a key role in the diagnosis of shaken baby syndrome. In addition, they are in a unique position to study ophthalmic aspects of the syndrome, which in turn furthers the overall understanding of this devastating condition.     

The spectrum of postmortem ocular findings in victims of shaken baby syndrome.

BACKGROUND: Ophthalmologists and ocular pathologists are called on to help identify children who have undergone violent shaking. The objective of this study was to describe the spectrum of postmortem ocular findings in victims of shaken baby syndrome and to correlate the ocular findings with the nonocular features found at autopsy. METHODS: The ocular pathology registry at the University of Ottawa Eye Institute was reviewed to identify all victims of fatal shaken baby syndrome whose eyes had been submitted for examination between Apr. 1, 1971, and Dec. 31, 1995. Autopsy reports were accessed from the hospital charts of the identified patients. RESULTS: Six patients, aged 1 to 34 months, were identified. Intraocular findings ranged from a focal globular hemorrhage at the posterior pole to extensive intraocular hemorrhage involving the entire retina with perimacular folds. All the children had evidence of optic nerve sheath hemorrhage. Nonocular findings included intracranial hemorrhage (in all cases), skull fracture (in two), rib fractures (in three) and high spinal cord hemorrhage (in four). The extent of the intraocular hemorrhage was not consistent with the nonocular findings. INTERPRETATION: Abused children may display a range of postmortem ocular findings, with intraocular hemorrhage varying from minimal to severe. These findings may not correlate with the severity of the child's other injuries. The presence of any retinal or optic nerve sheath hemorrhage in an infant, in the absence of an appropriate explanation for these findings, should raise suspicion of child abuse.     

Epiretinal membrane formation is a late manifestation of shaken baby syndrome.

Shaken baby syndrome is a constellation of injuries resulting from the intentional shaking type movement of a child who is usually younger than 3-years-old. This rapid acceleration-deceleration movement of the head is responsible for lesions attributed to shearing forces placed on the vitreoretinal structures and meningeal vessels across the dura. The ophthalmic findings include intraocular hemorrhages, perimacular retinal folds, and peripheral retinoschisis in the presence of intracranial injuries such as subdural hematomas without obvious external signs of head trauma. We describe a case of late development of an epiretinal membrane in a child with a history of shaken baby syndrome and propose a differential diagnosis list for epiretinal membrane formation in the pediatric age group.     

Correlation between retinal abnormalities and intracranial abnormalities in the shaken baby syndrome

PURPOSE: To report correlation between retinal and intracranial abnormalities and to evaluate pathogenesis of retinal hemorrhages in the shaken baby syndrome (SBS). DESIGN: Observational case series. METHODS: Seventy-five children with apparent nonaccidental head trauma consistent with SBS had complete physical examination, complete ophthalmologic examination, neuroimaging by CT or MRI, or both, and skeletal radiographic survey. In this retrospective review, ophthalmoscopic and intracranial abnormalities were correlated. RESULTS: The age of patients ranged from 2 to 48 months (mean - SD, 10.6 +/- 10.4 months). Neuroimaging was abnormal in all 75 cases. Findings included subdural hematoma (70 children, 93%), cerebral edema (33 children, 44%), subarachnoid hemorrhage (12 children, 16%), vascular infarction (nine children, 12%), intraparenchymal blood (six children, 8%), parenchymal contusion (six children, 8%), and epidural hemorrhage (one child, 1%). Sixty-four (64/75, 85%) children had retinal abnormalities, mostly (53/64, 82%) confluent multiple hemorrhages that were subretinal, intraretinal, and preretinal in 47/64 (74%) and bilateral in 52/64 (81%). No association was found between anatomic site (left, right, or bilateral) of intracranial and retinal findings (McNemar test kappa = -0.026-0.106) or between any of the intracranial findings mentioned above and the following retinal findings: normal or abnormal retinal examination, multiple (>10) or few retinal hemorrhages (< or =10), symmetric or asymmetric retinal findings, or retinoschisis (kappa = -0.127-0.104). Signs of possible increased intracranial pressure were not correlated with any retinal abnormality (kappa = -0.03-0.073). There was no correlation between evidence of impact trauma to the head and retinal hemorrhages (kappa = 0.058). Total Cranial Trauma Score and Total Retinal Hemorrhage Score, both indicating the severity of injury, were correlated (P =.032). CONCLUSIONS: Our study supports previous observations that the severity of retinal and intracranial injury is correlated in SBS. We cannot support the suggestions that in most children with SBS retinal bleeding is caused by sustained elevated intracranial, elevated intrathoracic pressure, direct tracking of blood from the intracranial space, or direct impact trauma. The correlation in severity of both eye and head findings may suggest, however, that retinal abnormalities are the result of mechanical shaking forces.