Index of Suspicion

A 4-month-old girl is brought to the ED because of fever and loss of appetite. Yesterday she was less playful than usual and whined instead of crying. She took only 3 oz of formula, after which she vomited twice. She refused other feedings and urinated only once during the day. Four days ago, she had rhinorrhea, but had no cough, diarrhea, or sick contacts. She was lethargic this morning and had a temperature of 99.5°F (37.5°C), prompting the visit.On physical examination, the baby appears lethargic, but is afebrile and has stable vital signs. Her fontanelle is full, and she has bruises on the helix of her left ear and a left hemotympanum. In the ED, she has a tonic seizure with leftward gaze and absence of right-sided movement. Lorazepam is administered, she is intubated, and she receives a loading dose of fosphenytoin.Laboratory findings include: WBC count of 26.08×103/mcL (26.08×109/L) with 80% neutrophils, 5% monocytes, and 14% lymphocytes; hemoglobin of 9.5 g/dL (95 g/L); hematocrit of 29.7% (0.297); and platelet count of 387×103/mcL (387×109/L). Prothrombin time is 15.3 seconds, and partial thromboplastin time is 26.1 seconds. Basic metabolic panel results are normal, as are findings on urinalysis. Rapid tests for respiratory syncytial virus and influenza as well as blood and urine cultures are negative. An additional procedure reveals the reason for her condition.A 14-year-old girl is seen for a 5-day history of vertigo, nausea, vomiting, headache, and ataxia. The onset of her illness was sudden and dramatic, with her symptoms awakening her from sleep. Paramedics felt that she had a flulike syndrome and did not take her to the hospital. Later that day, she experienced clumsiness in walking and right facial weakness. On day 2 of the illness, she had worsening vertigo and was unable to stand; on day 3, an ED evaluation yielded a diagnosis of urinary tract infection, and she was treated with intravenous fluids and oral antibiotics. She denies any recent fever, illness, or trauma and uses no medications or illicit drugs.On physical examination, the girl is afebrile and has stable vital signs. She is alert and oriented, with intact cognition and language, but has mild dysarthria. She has severe right-sided facial weakness, including the forehead (lower motor neuron pattern), and a mild decrease in sensation over the right cheek. She demonstrates severe incoordination of the right arm and leg, with difficulty walking, but has full motor power. Her deep tendon reflexes are symmetric, with downgoing plantar responses.One diagnostic procedure reveals the source of her bothersome symptoms and signs.A 4-year-old boy from Honduras, who is severely developmentally delayed, is admitted for an elective Blalock-Taussig shunt for his tetralogy of Fallot. He has had a recent dental procedure. On admission, he has mild rhinorrhea and is febrile, but has no other symptoms.On physical examination, the child is irritable, but consolable. He appears cachectic and has macrocephaly, with a large, open, flat anterior fontanelle. He has a fever, tachycardia, and hypoxia. He exhibits cyanosis of his lips and nail beds and has marked clubbing of all nails. He has pectus carinatum and a grade II/VI systolic ejection murmur heard best at the left upper sternal border that radiates. His abdomen is protuberant. He moves all extremities and is able to grasp objects. There is bilateral ankle clonus. He sits without support, but cannot stand independently and is unable to walk. He speaks fewer than five words, and his developmental age is estimated to be approximately 9 months.A metabolic panel and urinalysis yield normal results. His CBC shows normal leukocyte counts and polycythemia. All blood cultures are negative. He continues to have fevers despite being given broad-spectrum antibiotics. A tuberculin test is negative. Chest radiography is normal. Echocardiography shows no cardiac valvular vegetations. An imaging procedure is performed.CT of the head revealed a small left frontotemporal subdural hematoma with blood in the falx; cerebral edema on the left with midline shift; subfalcine herniation; and infarction of the left cerebrum, right frontal lobe, and right cerebellum. MRI demonstrated edema of the entire left cerebrum, inferior right frontal lobe, area posterior to the right lateral ventricle, and right cerebellar hemisphere; a small left posterior fossa subdural hematoma; a subdural hematoma along the entire left cerebrum; possible multiple areas of subarachnoid hemorrhages of the middle cranial fossa; and a midline shift with uncal herniation (Fig. 1).Magnetic resonance angiography (MRA) showed a patent circle of Willis and patent neck arteries. The initial skeletal survey and another repeated 2 weeks later were negative. Dilated funduscopic examination revealed diffuse multilayer bilateral retinal hemorrhages involving the macula and mid-periphery. Additional hematologic testing showed no evidence of coagulopathy or thrombophilia.The patient lives with her mother, grandmother, and three older siblings ages 2, 4, and 5 years old. All children were with the father over the weekend when the patient became ill. Both parents denied prior Child Protective Service (CPS) involvement, domestic violence, and drug or alcohol use.Nevertheless, inflicted head trauma was suspected based on the medical findings and the lack of trauma history. A report was made to the CPS agency in the jurisdiction of the child's father, and the hospital's child abuse specialist was consulted. As part of the CPS and police investigation, the patient's siblings were taken to the local child advocacy center for a forensic interview, during which the oldest brother reported that his 4-year-old brother was pushing the baby vigorously in a swing, which he demonstrated. The baby flew out of the swing, landing on the side of her face, and her father picked her up. She then slept all day. Her injuries were consistent with the mechanism described by her brother. CPS has substantiated neglect on the father's part for inadequate supervision, delayed seeking of medical help, and failure to disclose the injury. CPS continues to monitor the family.The triad of unilateral ear bruising, retinal hemorrhages, and ipsilateral cerebral edema is consistent with a form of inflicted head trauma termed “tin ear syndrome.” The incidence of tin ear syndrome is unknown; it has been reported in the literature only as individual case reports. Similar to shaken baby syndrome, the mechanism of injury involves rotational acceleration of the head. In the case of tin ear syndrome, however, the head is set in motion by a direct impact to one side of a child's head.Because the infant brain is not yet myelinated, rotational acceleration results in diffuse axonal injury and tearing of bridging cortical veins, which causes subdural hematomas (SDHs). The immature neck muscles and relatively large head permit the brain to move within the skull when forces of rotational acceleration and deceleration are applied. SDH, although more common in shaken baby syndrome, also can occur in tin ear syndrome. The distribution of SDH from shaking typically is bilateral and along the falx; SDH from direct contact force to the head typically is ipsilateral in children.In this patient, although physical abuse was suspected, the subsequent disclosure from her brother provided a plausible accidental mechanism for injuries more commonly seen with abuse. The rotational acceleration of her head occurred when she was catapulted from the moving swing and landed on the left side of her head.Any child who experiences acute altered mental status along with other signs of increased intracranial pressure should undergo head CT, which can demonstrate intracranial hemorrhage, cerebral edema, and soft-tissue swelling of the scalp. Subdural collections of different densities visible on CT raise concerns about hemorrhages of different ages and the possibility of multiple episodes of trauma. MRI is likely to be helpful at this point. However, it is not possible to date intracranial hemorrhages definitively by neuroimaging; patient signs and symptoms are the best indicators for dating inflicted injuries. If the distribution of the hemorrhages raises suspicion for vascular injury or malformations, MRA can be useful in assessing the cerebrovascular anatomy.Because all retinal hemorrhages do not result from obvious inflicted head trauma, a dilated funduscopic examination should be performed on children who have acute changes in mental status as soon as possible to evaluate for and describe retinal hemorrhages. A skeletal survey should be completed for all children younger than 2 years of age for whom abuse is suspected to look for occult skeletal injuries. A “babygram,” which is a single anteroposterior view of the chest and abdomen, is insufficient because the entire skeleton is not included.Severe traumatic brain injury requires resuscitation that includes intubation and ventilation when the Glasgow Coma Scale score is less than 8 or if there is cardiopulmonary compromise. Intravenous access to maintain normal blood pressure is required. Patients may need blood products as well as dopamine or epinephrine to maintain blood pressure. Anticonvulsants should be administered for seizure activity. The greatest risk for mortality is transtentorial herniation. Thus, patients must be monitored closely for any signs of increased intracranial pressure. If signs of herniation are present, acute hyperventilation until the signs resolve, sedation, muscle relaxation, and administration of an osmotic agent are required.It is important for clinicians to remain nonjudgmental in situations that raise the suspicion of abuse, focusing on the mechanism required to cause the injury. Reasonable suspicion, not proof, of abuse and neglect is what “mandated reporters,” including physicians, must report to CPS agencies. As part of the police/CPS investigation, forensic interviews of possible witnesses, including other children, can be invaluable. (Allison Jackson, MD, MPH, Zarir Khademian, MD, Rachel Y. Moon, MD, Children's National Medical Center, Washington, DC)Diffusion MRI of the brain demonstrated lesions in the right cerebellum, right pons, left thalamus, and left corpus callosum (Fig. 2A, B, C), all structures supplied by the posterior (vertebrobasilar) circulation. MRA suggested absent flow in the right vertebral artery (Fig. 2D). Cerebral angiography demonstrated irregular narrowing of the right vertebral artery (Fig. 2E) and an intraluminal thrombus (Fig. 2F).The clinical and MRI findings confirmed the diagnosis of multiple acute arterial ischemic strokes (AIS) occurring at different times over the preceding week. Angiography confirmed vertebral artery dissection, with artery-to-artery emboli from the intraluminal thrombus traveling downstream to cerebellar, brainstem, and thalamic arteries.The girl was given anticoagulation treatment with unfractionated heparin as well as neuroprotective strategies that included maintenance of normal blood pressure, blood glucose concentrations, and temperature. Results of additional investigations, including echocardiography and prothrombotic testing, were negative. She showed modest clinical improvement over 2 weeks and was discharged to the rehabilitation facility on low-molecular weight heparin (LMWH) for secondary stroke prevention.Stroke has emerged as a relatively common but underrecognized cause of neurologic disability in children. An incidence of 3 to 8 per 100,000 children per year suggests that ischemic strokes are more common than pediatric brain tumors. Most ischemic strokes in children are arterial, but 20% occur as a result of cerebral sinovenous thrombosis. The focus of this discussion is on ischemic stroke and not on episodes caused by intracranial bleeding.The sudden onset of focal neurologic deficits in a child should be considered to represent a stroke until proven otherwise. Children who suffer strokes usually present with abrupt onset of hemiparesis, but other focal deficits, including hemisensory changes, visual loss or diplopia, loss or slurring of speech, and imbalance or incoordination, should raise suspicion. Confounding signs and symptoms such as headaches, seizures, and altered consciousness are common.The differential diagnosis of an acute neurologic syndrome is lengthy (TableT1). The initial vertigo and ataxia in this patient raised additional considerations of acute cerebellitis, posterior fossa tumors, and vestibular diseases.A risk factor is identified in more than 70% of children afflicted with stroke, and many harbor multiple risks. Three large categories of associations are cardiac disease, arteriopathies, and prothrombotic disorders. Complex congenital heart lesions are associated commonly, with interventional or surgical procedures also increasing the risk. Arteriopathies include arterial dissection, Moyamoya disease, and sickle cell disease as well as inflammatory conditions related to infection (meningitis, varicella) or vasculitic conditions. Prothrombotic disorders include the factor V Leiden mutation, elevated lipoprotein (a) concentrations, and deficiencies of protein C or S. A wide variety of acute and chronic systemic illnesses may increase the risk of pediatric stroke.Stroke is diagnosed by clinical and neuroimaging findings. CT can demonstrate AIS and rule out hemorrhage, but is insensitive in the acute phase. MRI is the investigation of choice; diffusion-weighted MRI has revolutionized the early diagnosis of cerebral ischemia. Angiography can be accomplished with MR or CT techniques, although conventional angiography may be required for diagnoses such as dissection. Additional investigations such as echocardiography and prothrombotic testing are used to eliminate other risk factors.Arterial dissection accounts for 7% to 20% of pediatric AIS and may occur in the anterior (carotid) or posterior (vertebrobasilar) circulations. Dissection occurs when blood extrudes into the medial layer of the arterial wall through a tear in the endothelial surface. This abnormal surface allows pathologic thrombus formation, with subsequent localized arterial occlusion or embolization to downstream arteries. The pathophysiology of the initial vessel injury is not completely understood. Tethering of the vertebral arteries between the C1 and C2 vertebrae is likely to be relevant because neck rotation stretches the local segment of the artery. A history of recent trauma often is obtained, but the trauma usually is trivial and within the normal experiences of childhood. A history of sports injuries or chiropractic neck manipulation should be sought. Adult evidence suggests that most patients experiencing dissection harbor ultrastructural connective tissue abnormalities, although few have recognizable clinical syndromes such as Ehlers-Danlos or Marfan. Specific clinical clues to dissection include the presence of Horner syndrome (carotid dissection) or neck pain (vertebral dissection).A child suspected of having had a stroke should be seen urgently by a pediatric neurologist. Evidence-based management is lacking, but recent consensus-based publications provide useful guidelines. The immediate thrombolytic (“clot-busting”) treatments proven in adult stroke remain unproven in children, but studies are underway. Acute anticoagulation therapy with heparin or LMWH appears safe and may decrease the early progression of stroke and multiple strokes observed in this patient. Published guidelines recommend either initial aspirin (ASA) therapy or acute anticoagulation until investigations have delineated the cause for the stroke, followed by 3 to 6 months of anticoagulation for arterial dissection or a presumed cardiac cause. Early management also should provide supportive care to minimize secondary brain injury and should include maintenance of normal blood pressure, blood glucose concentration, and temperature, along with aggressive treatment of infection and immediate treatment of seizures.Patients strokes are to other or causes may be on to 5 per which also is for patients dissection or stroke, 3 to 6 months of secondary stroke is important because the risk of of in the 6 is to Early and aggressive and therapy is about of children be normal after a stroke, and the mortality is 5% to Most with deficits are most but other include and headaches, and The of illness is because the of stroke in a child the entire and a onset of a focal neurologic in a child is an and should be considered a stroke until proven otherwise. A of clinical suspicion is required to of pediatric stroke and in treatments are and the of and diagnostic cannot be MD, MD, MD, for CT performed to the developmental revealed large lesions with right uncal herniation and a mild midline shift to the subfalcine and a anterior fontanelle (Fig. differential diagnosis of brain lesions includes cerebral disease cerebral and of the patient's of there was a of suspicion for disease such as and to and is caused by the form of the The of this are by the to the form as An acute inflammatory occurs on the of the often by and The CT findings are to 2 in by a to in are multiple in the of the infection, with imaging the large lesions in this patient, which a did not any of infection by the is another to is reported in to of The infection causes cerebral and multiple often as that are the lesions in this is an important because such lesions often are multiple and are likely to occur in patients who have heart disease from in on cerebral are described as having with or without on the most commonly at the edema because of the a developmental occurs within the of the was in this patient by the of the lesions and by and disease, an infection by the also is to and typically involves the and and and the In children, however, this infection may the to of cerebral occur in childhood. On the may by and present as occur must commonly in the middle cerebral of the cerebral as in this patient. The may be single or usually fewer and than of to in imaging and edema of the brain tissue are and are should be that also is in many of the other than including of infection in and In to disease has occurred in and in has been in many of because the has been in many in also has must be of The most relevant to infection are and diagnostic in this case was the of the brain The lesions had and The was in the right and A in the left a was in the left lobe, and the was to the right lateral were large, there was a suspicion for and for testing for and infection was to the for and most likely based on findings and was disease caused by CT of the abdomen, and revealed no additional for and fever and blood cultures were negative. The fever administration of broad-spectrum and was presumed to be to of the for and were but the was for on cerebral disease was infection is diagnosed a of physical findings and imaging with by in a patient who has been in an area the is was to with of the cerebral edema, which is in was administered to the presumed infection, which is the treatment of for this The child was unable to undergo surgical and of lesions because of his cardiac and are used commonly to the of into glucose through the wall of the to cell Medical therapy such as is for patients who have or of and for patients who have medical and are for such as this patient. The of medical therapy to more with the of therapy than with the or the treatment for this disease, but it is associated with the of of and of from the which can to or of the should be considered in the differential diagnosis of increasing of and clinicians should be alert to the of in children. of Medical at

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