Fig. 5.1
CT of brain. Note subdural hemorrhage extending into the interhemispheric fissure
Although not obligatory, neck injury , including injury to the soft tissues as well as the cervical chord, may also be seen [48]. Although reported by some as “rare”—as low as 4 % in one inpatient study and on autopsy seen less than half of the time-increasing attention to postmortem full neck dissection and examination of the cervical chord and nerve roots is showing that these injuries are more common than previously reported [49–51]. The head and the neck of an infant are distinctly unique in their mechanical properties, therefore the nature of the injury will be different than observed in an older population [52]. Vertebral subluxation and spinal cord injury, including axonal injury, subdural hematomas, subarachnoid hemorrhage, spinal cord nerve root avulsion and hemorrhage, and damage to the neck musculature have been reported [53, 54]. Cervical brainstem injury can lead to apnea, hypoxia, cerebral edema, increased intracranial pressure, and further respiratory depression [55–57]. Additional neck injuries including bruising and abrasions have been reported [58]. One study even suggested that infant victims may also sustain hearing loss [59]. Subdural hemorrhage is secondary to the shearing of bridging veins in the potential subdural space as a result of the repeated acceleration-deceleration forces which allows the brain to move independently within the cranial vault while the bridging veins remain fixed to the venous sinuses [31]. The relatively large head, immature brain and weak cervical neck muscles of infants renders them particularly susceptible [60]. Blunt impact to the head may cause dramatic amplification of the forces experienced by the brain [61, 62]. The blunt trauma may manifest as skull fracture (in up to 25 %) or scalp contusion [39]. Although skull fractures may be simple linear fractures, concerns about abuse are raised when the fractures are complex, depressed, stellate, or bilateral [63]. Impact directly to the vertex or occiput can cause bilateral fractures.
Although there are usually minimal if any external signs of trauma, cutaneous ecchymosis may be observed on the face, chest and/or back. Impressions are occasionally left by the perpetrators hands grasping the child during the event either on a limb or around the chest.
Up to 29 % of SBS victims can exhibit rib fractures from the perpetrator grabbing the child’s chest during shaking [64] fractures are usually posterior or posterior-lateral [39, 65, 66]. Multiple fractures may be seen, sometimes in different states of healing thus suggesting more than one abusive event. It had been suggested that rib fractures have perhaps the highest correlation with abuse [65]. In SBS, rib fractures may also occur due to the compression of the rib cage by the perpetrator’s hands [54, 67, 68]. These fractures are seen posteriorly or posterolaterally (Fig. 5.2) [69, 70]. But rib fractures are often rare, occurring 6.7 % in one study [71]. They are not required for the diagnosis of SBS [72].
Fig. 5.2
Multiple rib fractures (arrows)
Ophthalmic Manifestations
Retinal hemorrhages are the most common manifestation of shaken baby syndrome , being seen in approximately 85 % of cases [73, 74]. Though retinal hemorrhages often occur throughout all layers of the retina, the most frequent finding is flame shaped hemorrhages in the nerve fiber layer [75, 76]. There are many specific causes of white centered hemorrhages yet virtually any retinal hemorrhage in any setting can have a white center, sometimes just from the reflex of the illuminating light [77, 78]. Morad and coworkers found that almost two thirds of victims with retinal hemorrhages will show too numerous to count multilayered hemorrhages including nerve fiber layer and deeper intraretinal hemorrhages as well as pre- and subretinal hemorrhages [79]. It has also been reported to be bilateral in 62.5–100 % and unilateral in 2 %[75]. Although hemorrhages are more frequent in the posterior pole, involvement to the ora serrata has particular diagnostic significance [80]. There is also a correlation between the severity of retinal hemorrhages and the severity of brain injury [81]. Although diffuse retinal hemorrhages are usually seen in the presence of subdural hemorrhage [82], rarely, retinal hemorrhage can be seen in the absence of intracranial bleeding or brain injury [83, 84].
As the vitreous is particularly firmly attached to the macula in infants and young children [85], repetitive acceleration-deceleration forces can cause the retina to be split, with accumulation of blood within the ensuing cavity [86]. These retinoschisis cavities are seen in 8 % of victims of which up to 20 % may be unilateral [74]. The most common form of traumatic retinoschisis is sub-internal limiting membrane blood. Circumlinear perimacular folds , hypopigmentation (from disruption of the retinal pigmented epithelium) or hemorrhage may or may not be seen at the edge of a retinoschisis cavity. Perimacular folds can be seen in 2–14 % of SBS (Figs. 5.3 and 5.4) [74, 87]. When the schisis cavity settles within the surrounding folds, a “crater-like” appearance is created. Folds persist indefinitely and may be a later sign that abuse had previously occurred.
Fig. 5.3
Right eye of infant victim of shaken baby syndrome . Note large area of retinoschisis with blood under the internal limitingmembrane. Bottom left shows a preretinal hemorrhage, at the edges of the retinoschisis there is a whiteperimacular fold. (Reprinted from Levin AV. Child abuse. In: Levin AV, Wilson T, eds: The Hospital for Sick Children’s Atlas of Pediatric Ophthalmology; 2007, p. 136 [259] with permission from Wolters Kluwer.)
Fig. 5.4
Perimacular fold (arrows) in an infant victim of shaken baby syndrome . The internal limiting membrane has flattened to form a “crater” within the folds, now filled with vitreous hemorrhage. (Reprinted from Levin AV. Child abuse. In: Levin AV, Wilson T, eds: The Hospital for Sick Children’s Atlas of Pediatric Ophthalmology; 2007, p. 137 [259] with permission from Wolters Kluwer.)
Multiple lines of research indicate that the mechanism of retinal hemorrhage formation in SBS is vitreoretinal traction during repeated acceleration and deceleration movements [88]. The vitreous attaches to the retina in very specific locations including the optic nerve, macula, peripheral retina, and superficial retinal vessels [89–91]. The predilection of hemorrhages to occur in the peripheral retina and the formation of macular retinoschisis are consistent with this anatomy. Likewise, sparing of the mid-periphery, an area with relatively less vitreous adhesion, supports this theory [88, 92]. Many researchers have developed finite element models to analyze retinal stress levels during repeated acceleration-deceleration motion [9, 14, 93–95]. A finite element model is developed using software to more accurately assess the forces obtained in an eye from shaking and impact motions [96]. Cirovic and coworkers demonstrated that the eye is most likely held in place more by the surrounding orbital fat rather than the extra ocular muscles or optic nerve [95]. Both Rangarajan and he have suggested that the repeated acceleration-deceleration causes a cumulative increase in the forces experienced at the vitreoretinal interface [9, 14, 93–95]. This accumulated force causes stress maximums at the posterior pole and the peripheral retina, where most hemorrhages are seen in SBS [9, 14, 93–96]. Porcine eyes also demonstrated this anatomic relationship [43]. When young piglets are subjected to even a single, rapid, head rotation, the location of intraocular hemorrhage was at the vitreous base [43]. These forces have been suggested to be strong enough to cause capillary disruption and peripheral ischemia, leading to later neovascularization of the peripheral retina [97–99]. Peripheral retinal nonperfusion is more common when preretinal or vitreous hemorrhage is seen [97]. The vitreous traction is strong enough to cause macular hole, retinal tears and/or perimacular folds [100–102]. Optical coherence tomography has been used to show the vitreoretinal traction causing ILM separation and folds [103].
Other theories or mechanism of injury have been suggested as causes of retinal hemorrhages . An increase in intrathoracic pressure has been hypothesized to result in restricted venous outflow from the eye in the face of unimpeded arterial inflow, thus resulting in ruptured retinal vessels. Yet multiple studies examining children who might likely have such circumstances, including cardiopulmonary resuscitation with chest compressions [104–108], seizures [106, 108–113], coughing [113, 114], and vomiting [114–116] have found little or no retinal hemorrhages. Geddes and coworkers wrote that hypoxia was the cause of retinal hemorrhages despite no experimental or clinical data, a theory she later retracted under oath in court [117]. Although increased intracranial pressure (ICP) can cause peripapillary hemorrhages in the setting of papilledema, it is not responsible for widespread multilayer hemorrhaging except in cases of hyperacute pressure elevation such as aneurysm , fatal head crush injury or fatal motor vehicle accidents, unlike the subacute pressure rises in abusive head trauma [118–121]. Papilledema is uncommon in SBS [81, 122]. The presence of any intracranial hemorrhage in association with any intraocular hemorrhage is known as Terson syndrome [123–125]. Although the mechanism is unknown, one theory invokes the passage of blood from the brain directly into the optic nerve sheath due to increased intracranial pressure. Postmortem studies of SBS victims have found optic nerve sheath hemorrhage is often discontinuous which also argues against a Terson mechanism [126–128]. Terson syndrome is rare in children [129].
The overall sensitivity of intraocular hemorrhage for abusive head trauma is 75 % and specificity is 94 %[74]. Starling estimated that retinal hemorrhages occur in 70 % of victims where there is impact alone, 84 % with shaking alone, and 94 % when both shaking and impact occur [8]. There is a positive correlation between retinal hemorrhages and being abused and increasing severity of retinal hemorrhages could correlate with an increase in likelihood of abuse [130]. Binenbaum and coworkers reported that the presence of retinal hemorrhage was highly associated with definite or probable abuse versus definite or probable accident (age-adjusted odds ratio 5.4 [95 % CI, 2.1–13.6]). The odds ratio in children younger than 6 months (n = 81) was 11.7 (95 % CI, 2.9–66.8) [130]. Similarly, Maguire and coworkers reported that the odds of abuse when a child has retinal hemorrhages is 14.7 with an estimated probability of 91 % [131]. Many others have also shown a statistically higher incidence of retinal hemorrhage in abusive head trauma as compared to accidents [80, 132]. The location of the hemorrhages also differs with pre-retinal, peripheral and vitreous hemorrhage all being much higher in SBS [80]. Although retinal hemorrhages can be seen in critically ill children from other causes, the bleeding is not as extensive with regard to location or layer of retinal involvement or distribution to the ora. More extensive bleeding was seen in the presence of severe coagulopathy, leukemia, traffic accidents and witnessed fatal falls down stairs, all of which are readily distinguishable via history and testing [133].
The timing for resolution of retinal hemorrhages is different depending on the type of hemorrhage seen, and not a reliable indicator of the timing of the trauma. Much is known about the time for resolution of birth hemorrhages but those time frames are not applicable for abusive head injury , where the retinal hemorrhages are caused by a different mechanism. Knowing the time of resolution of birth hemorrhages does assist the clinician in determining if the observed hemorrhages could be due to birth. Birth induced flame hemorrhages usually resolve within 72 h but may rarely persist up to 1 week [134–137]. Dot/blot hemorrhages usually resolve in 10–15 days [134, 137, 138]. Rarely, a single larger blot hemorrhage may persist as long as 4–6 weeks and intrafoveal hemorrhage may last even longer. Birth induced pre-retinal hemorrhage and vitreous hemorrhages can last weeks or even months. Retinoschisis has not been reported due to birth. Binenbaum and coworkers showed that in victims of SBS flame hemorrhages resolve prior to dot/blot hemorrhages [139, 140]. The presence of preretinal hemorrhage alone in a baby otherwise diagnosed as a victim of abuse is an indicator of an earlier onset [139, 141–143]. Numerous flame hemorrhages in victims of SBS can resolve in less than 24 h or hemorrhaging can get worse during the first days of admission. Some children can present with asymmetric retinal hemorrhages between eyes and then progress to a more symmetrical appearance with in the first 24 h [144]. This may be due to ongoing bleeding from injured vessels in the face of complex medical issues following the injury during hospitalization. Ophthalmology consultation should be obtained as soon as possible after presentation, preferably within 24 h [145].
At autopsy , significant findings may include areas of retinoschisis with vitreous gel still attached to the internal limiting membrane [76, 146]. Autopsy may also reveal small areas of sub-internal limiting blood that are difficult to detect clinically [82]. A better view of the far peripheral retina may also be obtained showing the extent of the retinal hemorrhages. Intrascleral hemorrhage at the junction of the optic nerve and globe has only been seen in postmortem specimens from children who are victims of SBS [126, 146, 147]. The presumed mechanism is shearing of posterior ciliary vessels as the eye translates in the orbit during acceleration-deceleration creating a fulcrum at the optic nerve-globe junction [148]. In a study of 18 victims of shaken baby syndrome compared to 18 victims of fatal accidental head trauma, optic nerve sheath as well as optic nerve intradural hemorrhage was seen more commonly in shaken baby syndrome (P < 0.0001) [126]. Hemorrhage in the orbital structures may involve the fat, extraocular muscles, and cranial nerves [126, 149]. Those children who die from traumatic causes other than abuse, who were found to have orbital hemorrhage, albeit in lesser amounts, either had direct orbital crush injury or mechanisms of injury that involved large or repetitive acceleration-deceleration.
Almost half of all survivors of SBS have vision issues including cortical visual impairment [28, 73, 150, 151], visual field cuts [28, 73], strabismus [28, 73, 152], ectopia lentis [150, 152], macular scarring [73, 150–153], and optic atrophy [28, 73, 108, 150, 152–158]. All survivors are at risk for amblyopia [73, 150]. Other, long term injuries , include microcephaly, macrocephaly, cranial nerve palsy, and seventh cranial nerve palsy [28, 159].
Other less commonly reported intraocular findings include retinal detachment , which is thought to be a result of the vitreoretinal traction from the acceleration-deceleration forces of shaking [102, 160, 161]. In one case this was also a proposed mechanism for a retinal pigment epithelial tear [100]. Other findings that have been reported in non-accidental trauma include corneal abnormalities, hyphema, macular hole [162], retinal avulsion at the optic nerve, subconjunctival hemorrhage, ptosis, and cataract [16, 135, 163–166].
Diagnosis
Diagnosis of abuse in children requires a multispecialty team approach often involving child abuse pediatrics, social work, nursing, psychiatry, neurosurgery, orthopedics, radiology and others. The diagnosis should never be based solely on the ocular findings although some findings, such as multilayered too numerous to count retinal hemorrhages extending to the ora with retinoschisis in the absence of a reported obvious etiology such as fatal head crush injury, is highly specific and sensitive for a diagnosis of abuse. History (or lack of explanatory history), and systemic findings must be considered. Appropriate imaging and laboratory studies, are essential. In all cases, a thorough differential diagnosis should be considered including the elimination of concern, where appropriate, of possible coagulopathy , infection, or metabolic disorders.
Management
A trained ophthalmologist should see the child in the first 24 h, if possible, as retinal findings may not persist or can change over the course of time [144]. Photos can be obtained if able [167, 168]. Documentation of the retinal findings should include number, location, and types of hemorrhages as well as the presence of retinoschisis , asymmetry between the eyes and any particular patterns of the hemorrhage distribution. A detailed drawing of the retinal findings may also be useful to accompany the description. In writing the consultation, ophthalmologists should consider a differential diagnosis only as appropriate to the findings. If the eye findings are highly suggestive of abuse, without a recognizable alternative, then the consult may state so. If the child dies, an autopsy can be very helpful in identifying other injuries that were not previously detected on clinical examination. Gilliland and coworkers have written a protocol for preparation of ocular tissues [76, 169]. The orbit should be removed en bloc to help with the determination of abuse [170]. Consent is usually not needed for this procedure given the forensic nature of the investigation [169–171]. Autopsy evaluation of children with known trauma should include orbital contents, multiple retinal sections, gross photography , and careful documentation [169].
Battered Child Syndrome
Definition
Battered child syndrome refers to the child who sustains all other injuries of abuse usually including bruising and/or fractures. The outcome may include permanent damage or death [172].
History
Although physical child abuse has been known for centuries, the French physician, Ambroise Tardieu, is often credited as the first to describe many manifestations of child maltreatment in the medical literature [173]. His original paper described not only physical injuries but also the toll that child labor can take on the physical and mental health of victims. His work was unrecognized, criticized, and disbelieved as many assumed that children would fabricate these stories [174]. The first publicly recognized case of child abuse in the US was in New York City in 1874. A 10 year old girl, Mary Ellen McCormack, was repeatedly assaulted by her adoptive mother. The severely battered child drew the attention of neighbors who complained. Due to the lack of infrastructure and child protection laws at the time, the case was brought before the American Society for the Prevention of Cruelty to Animals (ASPCA) . As a result of this case, the first child protective agency in the world was developed, the New York Society for the Prevention of Cruelty to Children [175]. It would not be until 1962 that the American medical community first acknowledged this as the Battered Child syndrome with the landmark publication by Kempe and coworkers [172]
Epidemiology
A 10 year autopsy study suggested that approximately 55 % of battered children are less than 1 year old, and 79 % less than 2 years old, with an average age of 14.3 months [176]. Triggers for all types of physical abuse include crying in 20 % followed by disobedience (6 %), domestic disputes (5 %), toilet training (4 %), and feeding problems (3 %) [18]. It has also been reported that children who are fatally maltreated are typically from houses that are financially unstable compared to children who survive the abusive situations they suffer through [177]. Eighty percent of perpetrators are the child’s parents [2]. Child abuse cases present more commonly in winter and on weekdays [65]. There is a well-documented disproportionate rate of investigating and reporting visible minorities from lower socioeconomic strata as compared to higher socioeconomic class Caucasians [178, 179].
The cost of battered children is also very expensive. Children who have been identified as maltreated or at risk for maltreatment account for approximately 9 % of all Medicaid expenditure. Their costs are on average >$2600 higher per year compared to children who are not identified as battered [180].
Systemic Manifestations
Injuries can affect every part of the body. Child abuse should be considered in virtually any child who presents with any injury but in particular when the injuries are multiple, recurrent, inconsistent with the history provided, inconsistent with the developmental level of the child or affecting unusual body parts [181, 182]. For example, fractures in a child who is not yet ambulatory, bruising in older children that is seen on the chest or buttocks or old and new injuries presenting at the same visit should raise concern about abuse. Multiple fractures (often of different stages of healing), traumatic abdominal injuries (including rupture or contusion of viscera), and soft tissue injury are often findings in battered children [183].
Fractures of long bones needing orthopedic management occur in up to 28 % of cases [65, 184], but a far greater percentage have fractures that do not require treatment and may be asymptomatic, including rib and long bone epiphyseal fractures (Fig. 5.5). Battered children are at risk for obtaining multiple injuries separated over time, therefore one fracture may have time to heal before another fracture is obtained [185]. Spiral fractures may be more concerning for child abuse due to the twisting, oblique squeezing, or dragging mechanism that the fracture suggests [10]. Rib and clavicle fractures may also be due to squeezing but have rarely been reported due to birth [31, 186]. Although femur fractures were long thought to be highly indicative of abuse [187], they may also occur due to accidental injury [188]. A discriminating factor is attainment of ambulation. Femur fractures are often accidental, no matter the form of the fracture, once a child is able to ambulate [179].
Fig. 5.5
Metaphyseal lesion (arrow) due to child abuse
Abdominal injuries , including liver or bowel laceration or contusion may occur. Abdominal trauma may be suspected on the basis of elevated hepatic transaminase, elevated amylase or abdominal ecchymosis [187]. Current recommendations include obtaining an abdominal CT scan in children with suspected abuse who have greater than 80 IU/L of either aspartate aminotransferase or alanine aminotransferase [189].
Bruising patterns can also indicate abuse. Any child who is less than 6 months old that has bruising has a very high likelihood of obtaining these injuries from abuse and a very low chance of having a coagulopathy [190, 191]. Testing for bleeding disorders should be considered for a child who presents with two or more unexplained bruises. If there is a concern for a coagulopathy, at a minimum prothrombin time (PT), international normalized ratio (INR), partial thromboplastin time (PTT), should be ordered, but often these are negative and no coagulopathy is found [187]. In ambulatory children, bruising over bony prominences is more likely to be accidental [191]. Bruising in patterns that are away from bony prominences (i.e. face, back, buttocks, abdomen, ears or hands) and ecchymosis in clusters or of similar shape, are more indicative of abuse [192]. Concerning shapes of bruises can be in the form of straight edges from belts, switches, chords or bite marks. Burn patterns may also help to distinguish abuse from accidents [190, 193]. Inflicted hot water burns are often found on the lower extremities or buttocks from sitting a child in hot water or holding their limbs under the tap (Fig. 5.6) [193]. A full thickness burn can happen as quickly as 30 s in water that is 149 °F [194]. Cigarette burns can also cause full thickness injuries if they are held in place for greater than 1 s [195]. They usually have heaped edges around a central crater .
Fig. 5.6
Immersion burn. Note total involvement of lower limbs. (Courtesy of Brian Holmgren)
Ophthalmic Manifestations
Virtually any eye injury can be sustained as a result of abuse. It has been estimated that 4–6 % of abusive eye injuries first present to the ophthalmologist [4]. Eyelid or facial trauma can result in lacerations, burns, or ecchymosis anywhere from the forehead to either eye or both eyes [183]. A study on postmortem eyes from known or suspected child abuse found conjunctival hemorrhage in 8.5 % [76]. Conjunctival hemorrhages can also result from blunt trauma and increased thoracic pressure, for example during suffocation [76]. Birth related conjunctiva hemorrhages can occur and are associated with multiparity, longer labor, increased birth weight, head circumference, and gestational age [196]. They always last longer than retinal hemorrhages from birth [196]. They have been described as semilunar occurring just outside of the limbus and wedge shaped with a base towards the limbus [197]. Hyphema, leukocoria, ectopia lentis, cataracts, sixth nerve palsy, orbital hemorrhage, and globe rupture have also been reported [31, 135, 163–166, 183, 198–203].
Diagnosis
A multidisciplinary team approach, usually led by a child abuse pediatrician, is critical for the evaluation of these children. Child abuse should be suspected when the history does not match the injury, there is a history of inflicted injury, or the history is continuously variable. Although there may be child-parent interactions that seems bothersome, this in of itself does not allow for a diagnosis of abuse.
Management
The management of eye injuries due to child abuse is no different than when these injuries are sustained in non-abusive manners except for the ophthalmologist, or the team’s, duty to report to child protective services if non-accidental trauma is suspected. The ophthalmologist should carefully document all injuries. Appropriate imaging, including photographic documentation, is recommended. The ophthalmologist should coordinate with other professionals to help provide long-term treatment and follow-up for these victims [204]. Reporting a child abuse incident is not considered a breach of doctor-patient confidentiality by law and is not protected under HIPAA. Even with this protection, some health practitioners still do not obey the mandatory reporting laws [205]. After reporting an incident, the ophthalmologist is rarely asked to testify in court. Any testimony should be treated as a scholarly endeavor to educate the courtroom rather than testimony to win a case on behalf of the prosecution or defense. Ophthalmologists should be encouraged to consult with peers and the literature to ensure that they to give the most accurate scientific responses in court [206].
Medical Child Abuse
Definition
Medical Child Abuse, previously referred to as Munchausen Syndrome by proxy, caregiver-fabricated illness in a child, factitious disorder by proxy or pediatric factitious disorder, “occurs when a child receives unnecessary and harmful or potentially harmful medical care at the instigation of a caretaker” [207, 208]. Often times the caregiver creates the appearance of an illness in a child either by the falsification of history, alteration of laboratory specimens (e.g. contaminating a culture swab), or the creation of physical signs [208–210]. This type of abuse can lead to potentially harmful medical investigations and/or treatment [208].
Epidemiology
Medical child abuse is relatively uncommon with an estimated incidence of 0.5–2.0 per 100,000 children under age 16 years. Based on a 2 year prospective study conducted in the UK and Ireland, most affected children were less than 5 years of age, which is not surprising as younger children are less likely to understand or reveal what their caretaker is doing [211]. The median age was 20 months [211]. Although most victims are infants and toddlers, up to 25 % can occur in children over the age of 6 years [212–214]. As children get older they may even participate in the falsification of symptoms or history. The mother is the perpetrator in over 95 % of cases [215]. In the age of social media, mothers are now blogging about their child’s medical illness and often grossly distorting the facts that were given by the medical provider [216]. Reasons why caregivers exhibit this type of behavior is beyond the scope of this chapter, but it seems that secondary gains and perhaps narcissistic psychopathology may play a role. Males and females are victimized equally [211, 212] and sibling siblings may also be victimized, sometimes serially [211]. Abuse will often continue if a patient is hospitalized and not fully supervised, thus allowing acts to be committed undetected. Up to 75 % of the morbidity experienced by children happens while admitted [208]. Mortality rates in these children can be up to 6–9 % and another 6–9 % can suffer long term disability or permanent injury, therefore early diagnosis is often life-saving [208, 212].
Systemic Findings
There is no single presentation for this type of abuse due to the wide spectrum of manifestations which are related to the nature of the falsified symptoms as well as the potential unnecessary medical investigation or treatment that well-meaning physicians enact to address those symptoms and signs. Although apnea and failure to thrive are the most common manifestations reported, others include induced bleeding, seizures, central nervous system depression, diarrhea, vomiting, fever, traumatic retropharyngeal abscess, induced infection, chronic intestinal pseudo-obstruction, polymicrobial infections and rash [207, 208, 212, 217, 218]. Up to a quarter of these children can also be afflicted by urinary tract issues [219]. Manifestations often involve multiple organ systems and result in consultations with multiple subspecialists [208]. Perpetrators may also change physicians to avoid detection.
Ophthalmic Findings
Ophthalmic findings can occur alone or in conjunction with other organ systems. In theory, a caregiver could provide history to raise concerns for ocular disorders of any kind that would result in unnecessary and potentially harmful medical testing or care. Recurrent conjunctivitis was reported by Baskin et al. [220]. Other ophthalmic manifestations include swelling, periorbital and orbital cellulitis from repeated injections, recurrent hemorrhagic conjunctivitis, ulcerations of the eyelids, corneal epithelial defects and keratitis, anisocoria from covert instillation of mydriatics, and periorbital cellulitis [208, 220]. Figure 5.7 demonstrates a corneal injury due to medical child abuse. The patient went on to require multiple corneal transplants. Note the characteristic affectation of the inferior cornea as a result of forced instillation while the child’s eyes move upward in the Bell’s phenomena. Children may also present with nystagmus and other eye movement disorders as well as pupillary abnormalities due to the central nervous system effect of covert poisoning or suffocation induced cerebral hypoxia .
Fig. 5.7
Corneal injury from medical child abuse . Instilled agent was not clearly identified. Note that the inferior cornea is more affected and vascularized. Patient required corneal transplant
Diagnosis
The diagnosis of medical child abuse should be considered when there is any recurrent or persistent illness that cannot be explained or is not getting better with the appropriate treatment. Unfortunately, it may take upwards of 14 months to 2.7 years before the diagnosis is made [212, 214]. The diagnosis should also be considered when a patient has been subjected to multiple treatment plans or medical procedures or present with a discrepancy in history, exam, and overall health [208]. Perpetrators may seem unexpectedly without distress over the induced illness, or demonstrate remarkable concern and willingness to contribute to the child’s care, often taking over the duties of nurses, which in turns allows the perpetrator the opportunity to cause harm.
Making the diagnosis of medical child abuse first requires this diagnosis be considered. Secondly it may require careful review of medical records and previously performed evaluations including imaging or laboratory studies to identify discrepancies in provided history from observable medical findings. A multidisciplinary evaluation which involves all medical providers is necessary [207].
Flaherty suggests asking the following three questions when evaluating patients who could potentially be victims of caregiver fabricated illness. Are the history, signs, and symptoms of disease credible? Is the child receiving unnecessary and harmful or potentially harmful medical care ? If so, who is instigating the evaluations and treatment? [208] If a child victim of medical abuse is suspected and they are able to speak, they should be interviewed separately from the caregiver [208]. It is important to remember that up to 30 % of children with fabricated illnesses do have an underlying medical illness [ 221]. Although it is relatively rare, physicians should have a low threshold for questioning if medical abuse is happening and report it immediately to the child protective services agency [222].
Because some of the abuse happens while a child is in the hospital, the use of covert video surveillance (CVS) has now been suggested to ensure safety of the child while admitted [208, 223, 224]. This may be controversial as it can be seen as an infringement on patient privacy and/or as falsely accusing caregivers of abuse. Covert video surveillance has been found to be helpful in one series up to 50 % of the time and identified real medical problems in 10 % of cases [208, 223]. Prior to implementing a CVS system, the hospital should take precautions and develop a plan for its use as well as a response plan for intervention if the child is in fact seen to be abused [208].
Management
Management will require referral to child protective services and implementation of a safety plan to prevent on-going abuse. The safety plan will likely need to engage medical professionals who have been involved in the care of the patient, and non-involved caregivers. It may be necessary to remove a child from the possibly abusive environment to document the falsification of symptoms which would resolve as a consequence of the removal or in order to prevent on-going harm.
Before reporting, it is essential to ensure that the diagnosis, or at least the suspicion of the diagnosis, is well founded. This may require a careful review of old medical records, covert video surveillance in hospital, or admission to hospital to see if the induced disease resolves when the child is out of contact with the family. Many states do not list “medical child abuse ” on their child abuse reporting forms, therefore it is important to focus on the specific ways in which the child is suffering from abuse and report all areas of abuse sustained from this fabricated illness including physical and emotional abuse [208].
Neglect and Medical Noncompliance
Definition
Neglect includes lack of care for a child, or failure to prevent an adverse event and protect from harm [18, 225, 226]. Intentional neglect is rare. Failure to provide medical care may be due to a lack of understanding or education from the parental standpoint, or an inability to comply, due to financial or other socioeconomic or family factors [70]. But when harm occurs despite appropriate attention and intervention, then abuse is reportable and should be considered.
Epidemiology
Neglect is the most common form of child maltreatment in the United States [18, 70, 227]. Seventy-eight percent of all reported child maltreatment cases in 2012 were due to neglect [1]. One study found that neglect accounted for 51 % of deaths and these children were primarily less than 5 years old [228]. Most children who suffered from fatal neglect have had no prior report with child welfare before their death. Risks for neglect include higher number of children in the home, along with previous family involvement with child welfare [228]. Some cases of neglect have been mistaken for sudden infant death syndrome (SIDS) [229]. There is evidence that neglect can have permanent effects on the process of brain development [230].
Systemic
Neglected children often have failure to thrive and severe neglect can lead to a failure to thrive amongst infants and children [231, 232]. Psychosocial dwarfism is a medical manifestation of severe neglect. Rarely, neglect can be so severe as to lead to death by starvation. Failure to thrive is usually multifactorial, stemming from inadequate nutrition as well as a disturbed social environment [233]. Neglect may also present systemically as inadequately or untreated illness or injury, or progressive injury/disease as a result of delayed care. Physical trauma accompanying failure to thrive, including burns, bruises, and fractures, may be seen. Injury can also occur from neglectful supervision or failure to prevent harm.
Ophthalmic
Neglect may also include noncompliance with recommended medical treatment, such as compliance with eye drops or patching. Failure to provide treatment may result in permanent vision loss [215]. Often these families fail to keep their scheduled appointments [234]. Ophthalmic manifestations seen in children with failure to thrive include lagophthalmos due to listlessness and unwillingness to move in a debilitated state, leading to corneal exposure and epithelial breakdown [234].
Diagnosis
Initial diagnosis should include an assessment of the child’s needs, family resources, their efforts to provide for the child, psychosocial challenges and options for ensuring optimal health for the child [235]. It is sometimes difficult to define when neglect has occurred. Certainly if there is harm, such as untreated amblyopia or uncontrolled glaucoma, which appear to be a result of neglectful caretaking, then a diagnosis of abusive neglect may be considered. A careful history can identify whether it would have been reasonable for the caretaker to appreciate a need for prompt care. For example, if a child was struck in the eye accidentally by a stick, and was left with a red, painful eye for 3 days without care, this would fall outside what a reasonable caretaker would do if they had the means (e.g. insurance coverage) to obtain such care. A parent who is not attending appointments or providing care due to alcoholism, is also an example of neglect. Failure to attend or deliver care due to religious objection may not be neglect unless the child is suffering in a fashion which can be alleviated through medical care. A parent may not make a decision that causes harm to their child, including religious or cultural objections. Ultimately, the diagnosis is based on a combination of damages to the child and reasons for failure of care delivery.
Management
Resolution of barriers to care such as cost of transportation or patches, through the assistance of a social worker or other support personnel, may obviate the need for child protective service intervention. Altering care regimens to better fit the parental capabilities, if it is safe to do so, may also be useful. For example if a parent is working all day, selecting a twice daily regimen may be easier than a four times daily regimen. Management of neglect should include having a low threshold for contacting the appropriate people when lack of care or neglect is suspected [236]. Reporting is warranted when there is direct physical or social evidence of neglect or when the physician is unable to eliminate the possibility that the maltreatment contributed to the child’s illness or injury [237]. It should also be reported when an evaluation of failure to thrive is suspicious for neglect as opposed to an identified organic cause [233]. Social workers or other health care ancillary staff are often helpful in coordinating care for these children as well as communicating with the proper authorities to ensure they are aware of the concern for child neglect. Detailed communication related to the physician’s concerns, resultant harm and what interventions were attempted by medical staff to alleviate the concern of neglect is important as it assists child protection authorities to understand the medical basis and provide appropriate safety planning or supports to the family.
In situations where there is a developing pattern of noncompliance and neglect, such as missed appointments or failure to obtain patches for amblyopia therapy , consideration can be given to the use of contracts. This has been suggested in the literature regarding dental caries. These documents are signed by the primary caregiver, physician, and a witness [234, 238]. After all barriers to care have been alleviated, the ophthalmologist can write a short statement in the medical record, indicating that continued failure to attend or institute care will result in a report to child protective services. This statement is signed by the physician, a witness, and the parent/guardian. If the family then continues to demonstrate neglect and noncompliance, reporting can then ensue and will likely be more effective. Given the limited and competing resources of child protective agencies, neglect may sometimes take a lower priority when the agency is also faced with simultaneous cases of abusive head trauma or other abuse that may cause imminent harm especially that which may be life threatening. Having the evidence of a signed contract such as this may help promote intervention from protective services.
Sexual Abuse
Definition
Sexual abuse may manifest as a range of behaviors including inappropriate sexual touching, exposure of a child to inappropriate for age sexual content or scenarios, or any sexual acts. Perpetrators and victims may be of any combination of genders. Children may be too young to know that a sexual act is inappropriate or they may be threatened not to reveal its occurrence. Sexual abuse is often chronic, covert, and perpetrated by someone known to the child who occupies of position of relative power and influence over the child. Violent acts, such as rape, are rare. Sexual acts with an adult may be considered as potentially abusive or coercive even if the teen pleas that it was consensual. Age of consent varies between states. Many states have statutory rape laws that define age of consent and with whom an adolescent can consent to sex .
Epidemiology
Sexual abuse has been reported in 3–38 % of all abuse cases [57, 239, 240]. Unlike other areas of abuse where the incidence between males and females is approximately equal, sexual abuse victims are more often female [239]. A study anonymously surveying mothers from North and South Carolina found that sexual abuse may have an incidence that is 15 times more common than what is actually documented in official child abuse reports [241]. It has been suggested to be particularly underreported in males [242]. It is estimated that 6–62 % of women and 2–15 % of men have experienced child sexual abuse and that one to two thirds have occurred with a family member as the perpetrator [242]. Peak ages of vulnerability are between 9 and 12 years old [242]. Even infants may be victims. Sexual abuse may also involve the use of children in pornography.
Systemic
Sexual abuse rarely results in physical injury but uncommonly one can see evidence of trauma such as palatal bruising from fellatio, vaginal injuries, or anal injuries. Sexually transmitted disease may also occur. One must distinguish between transmission of these infections through the birth canal at the time of birth. Latency periods may vary. For example, some have estimated that the latency for chlamydia after perinatal transmission may be in excess of 2 years. The presence of gonorrhea, chlamydia, or syphilis in the vagina, anus, throat or urethra always indicate sexual contact beyond these periods [243, 244]. HIV infection in the absence of another known risk factor such as blood transfusion or perinatal acquisition, also indicates sexual contact. These infections cannot be spread to these sites via fomites. Although pubic lice, herpes simplex, papilloma virus, Trichomonas vaginalis and molluscum can be spread non sexually, their presence in these sites is also concerning [245].
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