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Multicystic encephalopathy in abusive head trauma

Bela Kubat, Rob A C Bilo, Rick R van Rijn

Clinical Neuropathology

|April 15, 2014

Insights

Determining abusive head trauma (AHT) in infants is challenging. Brain damage patterns in multicystic encephalopathy do not reliably indicate injury timing, but AHT should be considered when no medical cause is found.

Area of Science:

Pediatric neuropathology
Forensic medicine
Infant brain injury

Background:

Abusive head trauma (AHT) diagnosis in infants is difficult, particularly with delayed presentation.
Infants surviving acute AHT may develop multicystic encephalopathy, with age-dependent brain changes potentially indicating injury timing.
Understanding these patterns is crucial for identifying perpetrators and aiding legal proceedings.

Purpose of the Study:

To analyze brain damage patterns in infants with AHT-induced multicystic encephalopathy.
To determine if cystic changes correlate with age at trauma or survival period.
To investigate the etiological mechanisms and implications for diagnosing AHT.

Main Methods:

Retrospective analysis of nine archival cases of trauma-induced multicystic encephalopathy (2005-2011).
Histological evaluation of hematoxylin-eosin-stained whole-hemisphere and cerebellar slides.
Assessment of topographic distribution of macroscopic and microscopic brain changes.

Main Results:

Cerebral hemispheres were more affected than the cerebellum.
Cystic change magnitude did not correlate with age at trauma or survival period.
Asymmetrical cerebral hemisphere involvement was noted in all cases, pronounced in three; injury patterns indicated ischemia and hypoperfusion.

Conclusions:

Brain damage magnitude and distribution do not aid in estimating the time of abusive head trauma.
Ischemia and hypoperfusion are primary mechanisms of brain injury in these cases.
Multicystic encephalopathy without a clear medical explanation warrants consideration of remote abusive head trauma.

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Abstract

Objective:

The proof of abusive head trauma (AHT) in infants is difficult, especially in cases with a long posttraumatic survival period. In the acute phase, injury to the cranio-cervical junction causes disturbances in respiratory and cardiac control, leading to apnea and bradycardia. Infants who survive the acute phase may subsequently develop multicystic encephalopathy. Because some types of changes are age-dependent, examination of the patterns of brain damage in these cases could provide information about the time in which they were inflicted. In particular, this could apply to the extent of the cystic changes, namely that the severity thereof may decrease with older age upon infliction of the trauma. This could potentially date the injury and thereby help to identify the perpetrator. We present an analysis of the patterns of brain damage in cases of AHT-induced multicystic encephalopathy and comment on the possible etiology and the implications thereof.

Materials:

Nine archival cases of trauma-induced multicystic encephalopathy, originating between the years 2005 and 2011, were identified. In 8 of these cases, hematoxilin-eosin-stained whole-hemisphere histologic slides, as well as small histologic slides of cerebellar hemispheres, were available for the evaluation of the topographic distribution of the macroscopic and microscopic changes.

Results:

The cerebral hemispheres were more affected than the cerebellum. The magnitude of the cystic changes did not correlate with the age at which the trauma had occurred, nor the surviva period. All cases showed asymmetrical affection of the cerebral hemispheres, which in 3 cases was very pronounced. The analysis revealed both ischemia- and hypoperfusion-induced injury patterns.

Conclusion:

Analysis of the magnitude and the distribution of the damage do not assist in the estimation of the period at which the trauma had occurred. The evaluation showed that ischemia, and to a lesser extent, hypoperfusion, were the major mechanisms of brain injury in these cases, which does not narrow the differential diagnosis of the underlying problem. However, in cases of multicystic encephalopathy, in the absence of a plausible medical explanation for the development of this condition, a remote (abusive) head trauma should be considered.

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