The Head
Men’s heads are by no means all like to one another, nor are the sutures of the head of all men constructed in the same form.
—HIPPOCRATIC WRITINGS
POINTS TO REMEMBER:
Head trauma should never be assumed to be “minor,” especially in infants. Signs may be delayed.
Facial features may suggest the diagnosis in many congenital, endocrine, autoimmune, or other systemic diseases.
Inspection
Self-study
Before reading this chapter, inspect the first eight illustrations without reading the legends. For each, commit to writing (a) what you see and (b) a diagnosis (if you can).
Trauma
In cases of trauma or coma of unknown cause, examination of the skull is especially important. Look for bruises, swellings, and cerebrospinal fluid otorrhea or rhinorrhea (see Chapter 12).Signs of blunt head trauma in an infant may be subtle. Dermatographic imprints of the striking object may be made visible only on postmortem skin blanching (Elner et al., 1990). More than half of the scalp contusions and skull fractures found at autopsy were missed on the initial physical examination in a series of 48 cases of the so-called shaken baby syndrome (see Chapters 10 and 26). (Some prefer the term “shaking impact syndrome” [Graham, 2001] because of evidence that shaking alone cannot generate sufficient force [Duhaime et al., 1987].) It is also possible that an impact may not leave any external signs, if the fall or blow is cushioned by a load-distributing material, because the infant skull is easily deformed (Ommaya et al., 2002).
A bruise over the mastoid (Fig. 9-1A) signifies a middle fossa basal skull fracture. This is called the Battle sign, although it was first noted by Sir Prescott Hewett. The Battle sign tends to be on the same side as the fracture, although a fracture on the opposite side or bilateral fractures can cause the sign. It usually occurs within the first 48 hours after injury, although it can be delayed for 3 to 12 days. The predictive value of a positive test is essentially 100% (Alter et al., 1974). Standard computerized tomographic (CT) scans may not demonstrate the fracture line. Coronal images or “fine cut” CT scans will show the fracture at least 50% of the time (M. Schlitt, personal communication, 1998).
False negatives were noted by Battle, who stated that “it is necessary for the production of this sign that there should be a complete fracture, one penetrating both tables” (Battle, 1890).
Battle also discussed apparent false positives due to external trauma to the mastoid. These were invariably accompanied by ecchymosis over the parietal area and the external ear.
Battle’s paper was actually a compendium of signs of basal skull fracture, including anterior basal skull fracture (presenting with conjunctival and eyelid hemorrhage, epistaxis from one nostril, unilateral proptosis [Chapter 10], unilateral conjunctival chemosis [Chapter 10], or cranial nerve dysfunction) and what Battle called “optic neuritis” but what would today probably be called papilledema (see Chapter 10).
Raccoon eyes (Fig. 9-1B) could come from external trauma with no skull fracture, from a skull fracture, or from orbital bleeding. Thus, the raccoon eyes are of less diagnostic value than the Battle sign.
Raccoon eyes should be distinguished from amyloid eyes (see Chapter 7).
Periorbital hemorrhages and scalp bruising have been reported in a child with head trauma as an unusual manifestation of scurvy, not accompanied by typical clinical findings but responding dramatically to vitamin C (Ahuja and Karande, 2002).
Because head injury is frequently associated with cervical spine injury, the presence of any of these signs reinforces the importance of protecting the cervical spine until injury to that region can be ruled out.
Head trauma should be taken seriously, even in the absence of these dramatic signs. Children have died or suffered serious head injury in witnessed falls from a height of as little as 2 ft (Plunkett, 2001). The mode of injury in 26 cases of acute infantile subdural hematoma included falls onto a Japanese mat while sitting or standing (Aoki and Masuzawa, 1984). A study in infant cadavers showed that free fall from a height of 82 cm can produce skull fractures (Weber, 1984), even if the fall is onto a softly cushioned surface (Weber, 1985). Until late infancy, there are areas of susceptibility especially to fractures in the parietal bone and elsewhere (Weber, 1987). Deterioration can occur after a lucid interval. Be sure to examine the fundi (see Chapter 10) and check the pupils carefully.A history of head trauma, even if remote, may be pertinent, as symptoms may be long delayed. Rapid acceleration/deceleration may cause a pressure wave of sufficient magnitude to damage the organ of Corti or cause hemorrhage into the labyrinth, resulting in hydrops. The outcome could be symptoms of Meniere disease, even years later (DiBiase and Arriaga, 1997).
The neurologic assessment of patients with head trauma is covered in Chapter 26.
 FIGURE 9-1 A: Battle sign. B: Raccoon eyes. This gentleman returned home late one night after a symposium, only to awaken the next morning amnesic for the events of the evening and be unable to recognize his wife. The further discovery that his wallet was missing suggested that he had been set upon by villains, a suspicion that was heightened with the appearance of these skin signs. The herpetic outbreak (which is bilateral) was posttraumatic. Additionally, there was blood behind the left tympanic membrane (ipsilateral to Battle sign) and left papilledema, presumably because of hemorrhage down the optic nerve sheath. The initial CAT scan revealed bifrontal intracerebral hemorrhages and a right temporal hemorrhage but missed his basal skull fracture, which was later seen on a repeat skull film taken after the Battle sign was recognized. |
A Comment on Contemporary Terminology
These days, when the head is described at all, it is usually called “normocephalic.” (Actually, the head is “normal.” It is the patient who is normocephalic!) However, if everyone is “normocephalic,” it seems superfluous to say so. Note that normocephaly, like having ten fingers, is 100% sensitive for almost all diseases (see Chapter 1). However, not quite everyone is normocephalic, at least if a careful examination of the head is actually done.
“Normocephalic” was previously used as a descriptor that meant that the patient was not brachycephalic or dolichocephalic, anthropologic terms defined by specific measurements of the cranial dimensions, previously thought to be of diagnostic value. Such distortions in the shape of the skull of infants are caused by the premature union of one or more cranial sutures.
Later, “normocephalic” was used to mean that the skull did not have burr holes or frontal bossing. (Burr holes were formerly placed as a diagnostic for subdural hematomas, a procedure made obsolete by the computerized axial tomographic [CAT] scan.)
Frontal bossing, or an unusually prominent forehead, is a sign of congenital heart disease, syphilis, rickets, or, most commonly, hydrocephalus (Fig. 9-2). In the elderly, it may signify Paget disease. Unfortunately, such diagnostically useful bumps have been thrown out with the bath water of phrenology.
Microcephaly (Fig. 9-3) can be a familial trait or a part of the congenital TORCH syndrome (due to toxoplasmosis, rubella, cytomegalovirus, or herpes).
Flattening of the back of the skull is caused by prolonged recumbency; this usually disappears spontaneously once the child is able to sit.
Marked cranial and facial asymmetry may be caused by neurofibromatosis.
In infants and children, head circumference should be measured and plotted on a head circumference/growth graph to check for developing hydrocephalus.
A Historic Interlude
The story of phrenology reminds us that a scientific proposition is not validated by being the focus of prestigious journals or professional societies; becoming part of “professionally accepted standards of care”; gaining acceptance by political authorities, influential scientists, or the vast majority of the populace; or being denounced by the Roman Catholic Church. Phrenology gained such a degree of popularity that in 1832, there were 29 phrenologic societies in
Great Britain and several journals devoted to phrenology in Great Britain and America, including one (the Phrenological Journal) that went through 20 volumes (Encyclopaedia Britannica, 1911). It is said that Clara Barton entered nursing on the advice of her phrenologist and that General McClellan recruited spies for the Union Army on the basis of the phrenologic profiles, insisting on a prominent secretiveness faculty. Horace Greeley, editor of the New York Tribune, thought that accidents could be reduced if railroad engineers were selected based on the shapes of their heads. Presidents James Garfield and John Tyler both had their heads examined.
Great Britain and several journals devoted to phrenology in Great Britain and America, including one (the Phrenological Journal) that went through 20 volumes (Encyclopaedia Britannica, 1911). It is said that Clara Barton entered nursing on the advice of her phrenologist and that General McClellan recruited spies for the Union Army on the basis of the phrenologic profiles, insisting on a prominent secretiveness faculty. Horace Greeley, editor of the New York Tribune, thought that accidents could be reduced if railroad engineers were selected based on the shapes of their heads. Presidents James Garfield and John Tyler both had their heads examined.
 FIGURE 9-2 A patient with frontal bossing due to hydrocephalus since early childhood. (Courtesy of Dr Michael Schlitt of Washington, with permission.) |
 FIGURE 9-3 Microcephaly. The face is of normal size, but the head is small with a sloping forehead. The scalp is redundant and furrowed. |
President Ulysses S. Grant met so often with his phrenologist that Democrats charged that the doctor was dictating domestic policy. In the south of France, the idea was carried to its logical conclusion by binding the heads of young children with tight bandages to squeeze their brains into a desirable shape. The Roman Catholic Church banned phrenology in Austria because the Vatican considered it a heresy. There is, however, an interesting anecdotal finding: the skull of Austrian anatomist Franz Joseph Gall, who introduced the theory of phrenology, was found to be twice as thick as normal at autopsy (Scott, 1998).
 FIGURE 9-4 Down syndrome. A: An infant who has a flattened face; malformed ears with a small lobule; and a short, broad neck with excessive, loose skin over the nape. B: An older child with the stereotypic facies: open mouth, protruding tongue, epicanthal folds, strabismus (see Chapter 10), and small nose with a broad bridge. (From Gellis SS, Feingold M. Atlas of Mental Retardation Syndromes: Visual Diagnosis of Facies and Physical Findings. U.S. Department of Health, Education, and Welfare; 1968, with permission.) |
However, phrenology was a scientific proposition in that it was falsifiable and has indeed been falsified.
Movements of the Head
The bobbing of the head fore and aft during systole is called the de Musset sign (see Chapter 17). It may be seen in aortic insufficiency or any other high stroke volume condition. Lateral systolic head bobbing is a sign of severe tricuspid regurgitation or right ventricular dysfunction (see Chapter 19).
Dyskinetic movements or dystonic posturing of the head is seen in primary neurologic diseases and in both the dystonias and the tardive dyskinesia associated with dopaminergic blockers such as the phenothiazines (see Chapter 26).
Torticollis is discussed in Chapters 25 and 26.
Facies
A Pedagogic Note
The appearance of the face is quite characteristic in a number of congenital disorders and systemic illnesses. Only a few examples can be presented here.
Down Syndrome
Probably, the most frequently seen of the stereotypic facies is that of Down syndrome, shown in Fig. 9-4. The tongue tends to protrude, and the whole face appears flat and round. The appearance is diagnostic.
Scleroderma
Patients with scleroderma (progressive systemic sclerosis or PSS) have a pinched nose, a mouth that will not open far (see Chapter 13),
and shiny, tight skin. The usual wrinkles of time can become obliterated, but because of the pinched nose, women so affected still do not look younger than their age.
and shiny, tight skin. The usual wrinkles of time can become obliterated, but because of the pinched nose, women so affected still do not look younger than their age.
 FIGURE 9-5 The classic facies of myxedema. (Courtesy of Dr Chris Casten and Consultant, the Cliggott Publishing Group of CMP Healthcare Media, Darien, CT, with permission.) |
Morphea or localized scleroderma (to be distinguished from PSS) may present with a vertical patch or scar over the skull. This “dueling scar” looks like a healed saber blow or coup de sabre.
Endocrine Disorders
Myxedema
The patient shown in Fig. 9-5 has myxedema (primary hypothyroidism). In addition to the coarse hair and dry rough skin (see Chapter 7), there is a puffy appearance and a yellow hue (from increased carotene). The nephrotic syndrome has a similar appearance in which the puffiness results from hypoproteinemic edema and the yellow hue from urochromes.
 FIGURE 9-6 A: Typical facies of acromegaly. The features are coarse because the cartilages of the nose, and possibly eyelids and ears, are enlarged and thickened. The lower lip is thick and projecting. The bones of the lower jaw are enlarged to such an extent that prognathism is present. The supraorbital ridges are prominent, so the rest of the forehead seems to retreat, while the skin of the forehead is hypertrophied and thrown into folds. (From Osborne OT. Acromegaly. In: Buck AH, ed. A Reference Handbook of the Medical Sciences. Vol. 1. New York: William Wood and Company; 1900:86-97, with permission.) B: Skull of an acromegalic, showing prognathism and prominent supraorbital ridges. |
Cushing Syndrome
Obese persons with diabetes and hypertension are frequently suspected of having Cushing syndrome, although the vast majority of them do not. Buccal fat pads and moon facies are considered to be reliable signs of Cushing syndrome. To check for buccal fat pads, stand directly in front of the patient and try to see his ears. Facial fat sufficient to obscure normal ears is a positive sign (although the patient still may not have Cushing’s). Protuberant ears can be the cause of a false negative. The specificity of this test is still high because most patients who do not have this sign will also not have the disease. This reemphasizes the importance of the prevalence of disease and shows why specificity is a less-useful concept than predictive value.
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