| OCR Text |
Show !ourlUll of Clinical Neuro-ophthalmology 7(2): 108-111,1987. Shaken Baby Syndrome Ocular and Computed Tomographic Findings Richard F. Spaide © 1987 Raven Press, New York Violent shaking of young infants has a variety of ocular and systemic sequelae, including retinal and subdural hemorrhages, seizures, coma, and death. The syndrome can be difficult to recognize because of the lack of external signs. The retinal examination and computed tomographic findings are absolutely essential for making the diagnosis, but very little information is available concerning this disorder in the ophthalmology literature. This paper presents the findings of a case of shaken baby syndrome and discusses the challenges in diagnosis. Key Words: Child abuse-Shaken baby syndromeRetinal hemorrhage. From the Department of Ophthalmology, Landstuhl Army Regional Medical Center. Address correspondence and reprint requests to Dr. Richard F. Spaide, Department of Ophthalmology, Landstuhl Army Regional Medical Center, APO NY 09180 U.s.A. The views expressed in this paper are those of the author, 21",<1r,,)( r,t'<:essarily those of the Department of Defense or the United Sr,':1:tt.-S /1..r~11Y. 108 Recognizing the eye findings of shaken baby syndrome is indispensable in making the diagnosis, but unfortunately, many ophthalmologists are not familiar with this disease. The purpose of this case report is to illustrate ocular and computed tomographic findings of this disorder. CASE REPORT This lO-week-old female was a twin product of a 34-week gestation delivered without complication. She had mild respiratory distress syndrome the first day of life and required oxygen therapy. Later, she was discharged and apparently did well at home for 10 weeks. On the day of admission the· parents noted that her arms and legs were shaking, Physical examination revealed a well-nourished child who was extremely lethargic; she responded only to pain and deep stimuli. She had no evidence of trauma, although she had frequent focal seizures that became more pronounced during the day. Adequate respirations threatened to become compromised, so she was intubated and received mechanical ventilation. She had two spinal taps and a computed tomograph (CT) of her head on the day of admission to the hospital. Both spinal taps produced bloody cerebral spinal fluid (CSF). The CT revealed areas of acute hemorrhage over the superior aspects of both cerebral hemispheres. On the second day of admission a neurosurgeon performed a subdural tap that also produced bloody CSF. The patient's pupils reacted poorly to light. Retinal examination showed a myriad of petechial hemorrhages over the entire extent of both retinas. Most of these hemorrhages were onefourth of a disc diameter or larger, and were so numerous they were confluent over large areas. SHAKEN BABY SYNDROME 109 After the first 4 days of hospitalization her seizure activity was controlled. She became much more responsive and she was extubated. During the time of her intubation, and subsequently, her blood gases were in normal physiologic range. A repeat computed tomography of the head (Fig. 2) on the fourth day revealed continued presence of the blood and marked edema of the brain. By the seventh hospital day the patient's pupillary function returned to normal. The retinal hemorrhages had substantially cleared leaving coalesced areas of blood (Fig. 1). Computed tomography of the head done on the tenth day (Fig. 2B) revealed beginning cerebral atrophy with relative preservation of the basal ganglia. By 2 weeks the retinal hemorrhages had almost completely regressed. No organisms grew from the spinal taps or blood cultures. All clotting studies were within physiologic limits. Other than mild anemia the child had a normal hematological profile. The mother said she had tossed the baby in the air and caught her, but she never dropped her. The father said that a few days prior to admission the infant suddenly jerked her head forward and struck her head against his chin, causing his chin to become bruised and tender. On one occasion they said they might have shaken her because she did not seem to be breathing well. The patient was discharged after 13 days. Her parents have cared for her at home while legal investigation was occurring. One month after discharge she had severe mental and motor delays. A computed tomograph of the brain done at this time revealed severe cerebral atrophy (Fig. 2C). FIG. 1. Fundus retinal photograph of a 10-week-old .baby with shaken baby syndrome, taken 1 week after admission. Many of the smaller retinal hemorrhages had resorbed while the larger ones remained. Two months after discharge the family moved and was lost to follow up. DISCUSSION Whiplash shaken infant syndrome is caused by violent shaking of a young infant (1,2). A baby's head comprises about 10% of the total volume and weight of the infant. The muscles that hold the head are extremely weak, and in addition, there is poor neural control of these muscles. During shaking, the head experiences severe accelerations and decelerations causing shear and tensile strains in the infant's soft, poorly myelinated brain. Recent investigation suggests in order to develop angular accelerations large enough to cause injury, based on models and subhuman primates, the children must suffer impacts as well as shaking (2a). These children develop small slit contusional tears on the frontal and occipital lobes (3). Small bridging veins running from the cerebral cortex to the venous sinuses break and cause subdural hemorrhages. In all probability they develop diffuse axonal injury (4), which has been produced in primate models by a similar mechanism (5). Extensive cerebral atrophy with concomitant mental retardation and developmental delays are a frequent late finding. Presenting signs include failure to thrive, hypothermia, bradycardia, lethargy, listlessness, vomiting, seizures (1,6,7), hemiplegia, coma, and bulging anterior fontanelles. Highly characteristic is the development of diffuse retinal hemorrhages. These could result from compression of the thorax such as in Purtschner's retinopathy, or from high intracranial pressure secondary to hemorrhage and edema. Additionally, blood could be accelerated backward through venous channels into the retinal vascular tree. The resultant increase in pressure could cause small vessels to rupture. Although retinal hemorrhages are common from birth trauma, after the neonatal period retinal hemorrhages in infants should be considered to be caused by child abuse until proven otherwise (8,9). Other· elements in the differential diagnosis include accidental trauma, sepsis, leukemia, and clotting disorders. Whiplash shaken infant syndrome is underdiagnosed for many reasons. The child presents with signs that do not seem to come from trauma. The caretakers rarely admit to shaking the infant (2,10,11). In our case the parents thought they might have shaken the infant once, apparently to revive her. The father claimed the infant jerked her forward with enough force to bruise his chin. ] Clin Neuro-ophthalmol, Vol. 7, No.2, 1987 no I Clin Neuro-ophthalmol, Val. 7, No.2, 1987 R. F. SPAIDE RG. 2. Computed tomographs of the head. (A) Done on the day of admission, demonstrating acute henlar" rhage (globular high density areas) consistent with subarachnoid hemorrhages. (8) Also on the day of admission, arrows point to areas in the frontal lobes demonstrating early encephalomalacia. (C) Four d,.ys later, diffuse edema of the brain. (0) Ten days after admission, diffuse encephalomacia and beginning atrophy. (E) Six weeks after admission, remarkable atrophy of the brain is evident. SHAKEN BABY SYNDROME 111 (It is hard to imagine how a 2-month-old baby can generate that much force with weak neck m:uscles.) Especially su~picious was the finding of encephalomalacia of the frontal lobes on the day of admission, suggesting that the patient suffered earlier trauma. The signs of the shaken infant syndrome can be considered by primary care physicians, but they are less adroit in observing retinal hemorrhages. Goudy media, inexperience with dilating drops, and the limited view with the direct ophthalmoscope make the infant's eye difficult to examine for the primary care physician. Ophthalmologists are skilled in examining the infant's retina with indirect ophthalmoscopy, but information in the ophthalmology literature concerning shaken infant syndrome is almost nonexistent. Finally, this syndrome is underdiagnosed because the signs are short lived. Lethargy, seizures, and retinal hemorrhages, the only visible signs in our case, did not last long. It is quite possible that less severe shaking could cause less severe and more evanescent signs. The child could be brought to the doctor for anything from lethargy to seizures, but by the time an ophthalmologic consult is considered retinal hemorrhages might have resolved. Therefore, any child who has unexplained lethargy or seizures should have an ophthalmologic examination without delay. Family therapy or even criminal prosecution is a difficult endeavor in shaken baby syndrome (10,11). As mentioned previously, the parents seldom admit to shaking the baby, and it is hard to prove exactly who did the shaking. In some cases, the external appearance of the baby can be paradoxically normal. Also, shaking seems less culpable to many people than striking a child (12). This does not paint an encouraging picture for any immediate improvement in the fate of these unfortunate infants. Hopefully, with increased awareness we may be able to more accurately characterize and quickly diagnose the disorder. As with any form of child abuse, parental therapy and education is the key to prevent the weakest from bearing the greatest strains of familial dysfunction. REFERENCES 1. Caffey J. On the theory and practice of shaking infants. Am JDis Child 1972;124:161-9. 2. Caffey J. The whiplash shaken infant syndrome: manual shaking by the extremities with whiplash-induced intracranial and intraocular bleedings, linked with residual permanent brain damage and mental retardation. Pediatrics 1974;54:396-403. 2a. Duhaime A, Gennarelli TA, Thibault LE. The shaken baby syndrome: A clinical, pathological, and biomechanical study. Presented at the 1986 Annual Meeting of the American Association of Neurological Surgeons, paper 8, April, 1986. 3. Calder 1M, Hill I, Scholtz CL. Primary brain trauma in nonaccidental injury. JClin PathoI1984;37:1095-1100. 4. Adams JH, Graham 01, Murray LS, Scott G. Diffuse axonal injury due to head injury in humans: an analysis of 45 cases. Ann NeuroI1982;12:557-63. 5. Gennarelli TA, Thibault LE, Adams JH, Graham 01, Thompson q, Marcincin RP. Diffuse axonal injury and traumatic coma in the primate. Ann NeuroI1982;12:564-74. 6. Ludwig S, Warman M. Shaken baby syndrome: A review of 20 cases. Ann Emerg Med 1984;13:104-7. 7. Carter JE, McCormick AQ. Whiplash shaking syndrome: retinal hemorrhages and computerized axial tomography of the brain. Child Abuse Neglect 1983;279-86. 8. Eisenbrey AB. Retinal hemorrhage in the battered child. Child's Brain 1979;5:40-4. 9. Kessler DB, Siegel-Stein F. Retinal hemorrhage, meningitis, and child abuse. NY State JMed 1984;59-60. 10. Benstead JG. Shaking as a culpable cause of subdural haemorrhage in infants. Med Sci Law 1983;23:242-4. 11. Eagan BA, Whelan-Williams S, Brooks WG. The abuse of infants by manual shaking: medical, social and legal issues. Florida Med Assoc 1985;72:503-7. 12. Guthkelch AN. Infantile subdural haematoma and its relationship to whiplash injuries. Br Med J1971;430-1. JClin Neuro-ophthalmol, Vol. 7, No.2, 1987 |
