Etiology

Petechiae are probably caused by a sudden increase in intrathoracic and venous pressures during passage of the chest through the birth canal. An infant born with the cord tightly wound around the neck may have petechiae only above the neck.

Differential Diagnosis

Petechiae may be a manifestation of an underlying hemorrhagic disorder. The birth history, early appearance of the petechiae, and absence of bleeding from other sites help to differentiate petechiae caused by increased tissue pressure or trauma from petechiae caused by hemorrhagic disorders (see Chapter 88). The localized distribution of the petechiae, absence of subsequent crops of new lesions, and a normal platelet count exclude neonatal thrombocytopenia. The platelet count also may be low because of infection or disseminated intravascular coagulation. Infections may be clinically distinguished from traumatic petechiae by the presence of other signs and symptoms. Disseminated intravascular coagulation usually is associated with excessive and persistent bleeding from a variety of sites. Petechiae usually are distributed over the entire body when associated with systemic disease.

Treatment

If the petechiae are caused by trauma, neither corticosteroids nor heparin should be used. No specific treatment is necessary.

Prognosis

Traumatic petechiae usually fade within 2 or 3 days.

Treatment

No local therapy is necessary. The rise in serum bilirubin that follows severe bruising may be decreased by the use of phototherapy (see Chapter 100). Ecchymoses rarely result in significant anemia.

Prognosis

The ecchymoses usually resolve spontaneously within 1 week.

Etiology

Although subcutaneous fat necrosis can occur without any obvious cause, it is most commonly seen in association with perinatal asphyxia. Other etiologic factors that have been implicated include cold exposure, localized skin trauma, obstetric trauma, preeclampsia, gestational diabetes, maternal or fetal risk of thrombosis, maternal cocaine use, hypothermia, prostaglandin E administration, brown fat deficiency, meconium aspiration, sepsis, and intrapartum calcium channel blocker administration. It can also occur as a complication of therapeutic hypothermia for perinatal asphyxia or in newborns undergoing surgical procedures.44,93 Many affected infants are large and have been delivered by forceps or after a prolonged, difficult labor involving vigorous fetal manipulation. The distribution of the lesions usually is related to the site of trauma, which explains the frequent involvement of shoulders and buttocks. One suggested mechanism of pathogenesis proposes that diminished in utero circulation and mechanical pressure during labor and delivery result in vascular compromise to specific areas, which eventually causes localized fat necrosis.¹⁷ In maternal cocaine use during pregnancy, it has been postulated that cocaine may decrease placental perfusion with subsequent hypoxemia and alteration of the maternal and fetal pituitary-adrenal axes.¹⁴

Pathology

Histopathologic studies reveal initial endothelial swelling and perivascular inflammation in the subcutaneous tissues. This is followed by necrosis of fat and a dense granulomatous inflammatory infiltrate containing foreign body–type giant cells with needle-shaped crystals resembling cholesterol.

Clinical Manifestations

Necrotic areas usually appear between 6 and 10 days of age, but may be noted as early as the second day or as late as the sixth week. They occur on the cheeks, neck, back, shoulders, arms, buttocks, thighs, and feet, with relative sparing of the chest and abdomen. The lesions vary in size from 1 to 10 cm; rarely, they may be more extensive. They are irregularly shaped, hard, plaquelike, and nonpitting (Figure 30-2). The overlying skin may be colorless, red, or purple. The affected areas may be slightly elevated above the adjacent skin; small lesions may be easily movable in all directions. There is no local tenderness or increase in skin temperature.

This condition may be associated with hypoglycemia, hypertriglyceridemia, hypercalcemia, anemia, and thrombocytopenia. Marked symptomatic hypercalcemia may develop in infants with subcutaneous fat necrosis at 3 to 4 weeks of age; this has been characterized by vomiting, weight loss, anorexia, fever, somnolence, and irritability, with serum calcium levels as high as 17.3 mg/dL.19,55 The treatment includes intravenous hydration, calcium wasting diuretics such as furosemide, potassium citrate to inhibit renal stone formation, and corticosteroids. Successful short-term treatment with bisphosphonates (e.g., pamidronate or etidronate) has been reported to control hypercalcemia.⁶⁰ Investigators have suggested extra renal production of 1,25-dihydroxyvitamin D by the granulomatous cells of fat necrosis as a possible mechanism for the hypercalcemia.^18,46

Differential Diagnosis

The differential diagnosis includes lipogranulomatosis and sclerema neonatorum, which carry a serious prognosis, and nodular nonsuppurative panniculitis, which is usually associated with fever, hepatosplenomegaly, and tender skin nodules.

Treatment

These lesions require only observation. Surgical excision is not indicated.

Prognosis

The lesions slowly soften after 6 to 8 weeks and completely regress within several months. Occasionally minimal residual atrophy, with or without small calcified areas, is observed. Affected infants should be followed closely during the first 6 weeks for potential development of hypercalcemia. It is important to treat this complication without delay to prevent central nervous system (CNS) and renal sequelae.¹⁹

Etiology.

A cephalhematoma is caused during labor or delivery by a rupture of diploic blood vessels that traverse from skull to periosteum. Repeated buffeting of the fetal skull against the maternal pelvis during a prolonged or difficult labor and mechanical trauma caused by use of forceps and vacuum suction devices in delivery have been implicated. Petrikovsky and associates⁷³ described seven infants in whom cephalhematoma or caput succedaneum was identified prenatally before onset of labor. Occurrence of premature rupture of membranes in five of the pregnancies suggests an etiology of fetal head compression by the uterine wall resulting from oligohydramnios subsequent to the ruptured membranes.

Clinical Manifestations.

The bleeding is sharply limited by periosteal attachments to the surface of one cranial bone; there is no extension across suture lines. The bleeding usually occurs over one or both parietal bones. Less often, it involves the occipital bones and, very rarely, the frontal bones. The overlying scalp is not discolored. Because subperiosteal bleeding is slow, the swelling may not be apparent for several hours or days after birth. The swelling is often larger on the second or third day, when sharply demarcated boundaries are palpable. The cephalhematoma may feel fluctuant and often is bordered by a slightly elevated ridge of organizing tissue that gives the false sensation of a central bony depression. In 1974, Zelson and co-workers¹⁰² noted an underlying skull fracture in 5.4% of cephalhematomas. These fractures are almost always linear and nondepressed.

Radiographic Manifestations.

Radiographic manifestations vary with the age of the cephalhematoma. During the first 2 weeks, bloody fluid results in a shadow of water density. At the end of the second week, bone begins to form under the elevated pericranium at the margins of the hematoma; the entire lesion is progressively overlaid with a complete shell of bone.

Differential Diagnosis.

Cephalhematoma must be distinguished from other birth complications such as subgaleal hematoma, caput succedaneum, vacuum caput, leptomeningeal cyst, or congenital anomalies such as meningoceles. It may be differentiated from caput succedaneum by (1) its sharp periosteal limitations to one bone, (2) the absence of overlying discoloration, (3) the later initial appearance of the swelling, and (4) the longer time before resolution. Cranial meningocele is differentiated from cephalhematoma by pulsations, an increase in pressure during crying, and the demonstration of a bony defect on a radiograph. An occipital cephalhematoma may be confused initially with an occipital meningocele and with cranium bifidum because all occupy the midline position.

Treatment.

No therapy is indicated for the uncomplicated cephalhematoma, as more than 80% resolve by gradual hemolysis and resorption in 3 to 4 weeks. When the hematoma does not resolve spontaneously, it may get organized, and calcification may be seen. It may still get absorbed slowly and often disappears over 3 to 6 months. Persistent calcification that is not resolved by time may be an indication for surgical excision.³⁷ Rarely, a massive cephalhematoma may result in blood loss severe enough to require transfusion. Significant hyperkalemia⁵³ and hyperbilirubinemia may result from resolving hematoma, necessitating appropriate treatment. The most common associated complications are skull fracture and intracranial hemorrhage. Linear fractures do not require specific therapy, but radiographs should be taken at 4 to 6 weeks to ensure closure and exclude formation of leptomeningeal cysts; depressed fractures require immediate neurosurgical consultation. Routine incision or aspiration of a cephalhematoma is contraindicated because of the risk for introducing infection. Rarely, bacterial infections of cephalhematomas occur, usually in association with septicemia and meningitis. Focal infection should be suspected when a sudden enlargement of a static cephalhematoma occurs during the course of a systemic infection, with a relapse of meningitis or sepsis after treatment with antibiotics, or with the development of local signs of infection over the cephalhematoma (Figure 30-3). Diagnostic aspiration may be indicated. If a local infection is present, surgical drainage and specific antibiotic therapy should be instituted. Osteomyelitis of the underlying skull may be a rare concurrent problem.^65,68 The diagnosis may be suggested by periosteal elevation and overlying soft tissue swelling on skull radiographs. Additional rare complications that may accompany an infected cephalhematoma and osteomyelitis include venous sinus thrombosis and cerebellar hemorrhage.¹⁵ Magnetic resonance imaging (MRI) may be used to detect these two intracranial complications, whereas computed tomography (CT) is the best imaging modality to identify the permeative bone erosion and destruction of osteomyelitis.¹⁵

Prognosis.

Most cephalhematomas are resorbed within 2 weeks to 3 months, depending on their size. In a few patients, calcium is deposited (Figure 30-4), causing a bony swelling that may persist for several months and, rarely, up to several years. Radiographic findings persist after the disappearance of clinical signs. The outer table remains thickened as a flat, irregular hyperostosis for several months. Widening of the space between the new shell of bone and the inner table may persist for years; the space originally occupied by the hematoma usually develops into normal diploic bone, but cystlike defects may persist at the sites of the hematoma for months or years. Rarely, a neonatal cephalhematoma may persist into adult life as a symptomless mass, the cephalhematoma deformans of Schüller.

Etiology.

The most common predisposing factor is difficult operative vaginal delivery, particularly midforceps delivery and vacuum extraction.⁹⁷ Plauche reported that operative vaginal delivery was the most common factor in 64% of cases of subgaleal hemorrhage.⁷⁵ The risk for subgaleal hemorrhage may be reduced by use of softer silicone vacuum cups instead of the original rigid metallic ones.⁹ The major risk factors include coagulopathies,^18,83 prematurity, macrosomia, fetal dystocia, precipitous labor, intrapartum hypoxia, male sex, cephalopelvic disproportion, prolonged labor, and nulliparity.^75,97

Mechanism of Injury.

When vacuum is used, the mechanism of injury is thought to be the vacuum traction pulling the scalp away from stationary bony calvarium, thus avulsing open the subgaleal space and causing the bridging vessels to tear and bleed into the subgaleal space. The loose connective tissue of the subgaleal space is extremely expansive and extends over the entire area of the scalp. The space can accommodate the entire neonatal blood volume (250 mL or more in a term baby), leading to hypovolemic shock, disseminated intravascular coagulation, and multiorgan failure, resulting in death in 25% of the cases.75,98

Clinical Manifestations.

Early manifestations may be limited to pallor, hypotonia, and diffuse swelling of the scalp. The development of a fluctuating mass straddling cranial sutures, fontanelles, or both is highly suggestive of the diagnosis. Because blood accumulates beneath the aponeurotic layer, ecchymotic discoloration of the scalp is a later finding.³⁵ This is often associated with pitting edema and progressive posterior spread toward the neck and lateral spread around the ears, frequently displacing the ears anteriorly (Figure 30-6). Periorbital swelling and ecchymosis also are commonly observed.⁷⁵ Eventually hypovolemic shock, multiorgan failure, and signs of cerebral irritation develop. Massive lesions can cause extracranial cerebral compression, which may lead to rapid neurologic decompensation.⁵ The clinician should be aware of occasional “silent presentation,” in which a fluctuant mass is not apparent initially despite serial clinical examinations.⁷⁰ Subgaleal hemorrhage should be considered in infants who show signs of hypoperfusion and falling hematocrit after attempted or successful vacuum delivery even in the absence of a detectable fluctuant mass. Close monitoring is particularly important in those infants who are considered stable enough to allow admission to the normal newborn nursery.

Radiographic Manifestations.

Standard radiographs of the skull may identify possible associated fractures. Computed tomography scanning may demonstrate abundant epicranial blood, parieto-occipital bone dehiscence, bone fragmentation, and posterior cerebral interhemispheric densities compatible with subarachnoid hemorrhage.³⁵

Differential Diagnosis.

In contrast with cephalhematoma, subgaleal hemorrhage is characterized by its more diffuse distribution, more rapid course, significant anemia, signs of CNS trauma (e.g., hypotonia, lethargy, seizures), and frequent lethal outcome.

Treatment.

Prompt restoration of blood volume with fresh frozen plasma or blood is essential. In the presence of continued deterioration, neurosurgery may be considered as a last resort. A bicoronal incision allows for exposure of the subgaleal space. Bipolar cauterization of any bleeding points can then be accomplished, and a drain can be left in the subgaleal space. One report⁵ described a successful outcome after finger disimpaction of a large clot before insertion of a drain that released an additional 200 mL of blood over 2 days.

Prognosis.

Although nearly 25% of infants with subgaleal hemorrhage die, long-term prognosis for survivors is generally good.48,75 More experience with aggressive and timely neurosurgical intervention may help to improve outcomes.

Etiology.

Skull fractures usually follow a forceps delivery or a prolonged, difficult labor with repeated forceful contact of the fetal skull against the maternal symphysis pubis, sacral promontory, fifth lumbar vertebrae, or ischial spine. They have also been described after vacuum-assisted vaginal delivery.⁴³ Most of the fractures are linear. Depressed fractures are associated with forceps application. However, they may occur spontaneously after cesarean section^26,31 or vaginal delivery without forceps. Factors that also have been implicated include pressure on the fetal skull by a maternal bony prominence (e.g., sacral promontory) or uterine fibroid, a fetal hand or foot, or the body part of a twin. Occipital bone fractures usually occur in breech deliveries as a consequence of traction on the hyperextended spine of the infant when the head is fixed in the maternal pelvis.

Clinical Manifestations.

Linear fractures over the convexity of the skull frequently are accompanied by soft tissue changes and cephalhematoma. Usually the infant’s behavior is normal unless there is an associated concussion or hemorrhage into the subdural or subarachnoid space. Fractures at the base of the skull with separation of the basal and squamous portions of the occipital bone almost always result in severe hemorrhage caused by disruption of the underlying venous sinuses. The infant may then exhibit shock, neurologic abnormalities, and drainage of bloody cerebrospinal fluid from the ears or nose.

Depressed fractures are visible, palpable indentations in the smooth contour of the skull, similar to dents in a ping-pong ball (Figure 30-7). The infant may be entirely free of symptoms unless there is an associated intracranial injury.

Radiographic Manifestations.

The diagnosis of a simple linear or fissure fracture is seldom made without radiographs in which fractures appear as lines and strips of decreased density. Depressed fractures appear as lines of increased density. On some views they are manifested by an inward buckling of bone with or without an actual break in continuity. Either type of fracture may be seen on only one view.

Differential Diagnosis.

Occasionally the fragments of a linear fracture may be widely separated and may simulate an open suture. Conversely, parietal foramina, the interparietal fontanelle, mendosal sutures, and innominate synchondroses may be mistaken for fractures. In addition, normal vascular grooves, “ripple lines” that represent soft tissue folds of the scalp, and lacunar skull may be mistaken for fractures.

Treatment.

Uncomplicated linear fractures over the convexity of the skull usually do not require treatment. Fractures at the base of the skull often necessitate blood replacement for severe hemorrhage and shock in addition to other supportive measures. If cerebrospinal fluid rhinorrhea or otorrhea is present, antimicrobial coverage is indicated to prevent secondary infection of the meninges.

Small (<2 cm) “ping-pong” fractures may be observed without surgical treatment. Loeser and associates⁵⁹ reported three infants with depressed skull fractures in whom spontaneous elevation of the fractures occurred within 1 day to months of age. Follow-up at 1 to years revealed normal neurologic development in all three.

Several nonsurgical methods have been described for elevation of depressed skull fractures in certain infants:

Because these methods are technically easier and less traumatic, they may be preferable to surgical intervention in a symptom-free infant with an isolated lesion.

Comminuted or large fractures associated with neurologic signs or symptoms should be treated by immediate surgical elevation of the indented segment to prevent underlying cortical injury from pressure. Other indications for surgical elevation include manifestations of cerebrospinal fluid beneath the galea and failure to elevate the fracture by nonsurgical manipulation.

Prognosis.

Simple linear fractures usually heal within several months without sequelae.

Basal fractures carry a poor prognosis. When separation of the basal and squamous portions of the occipital bone occurs, the outcome is almost always fatal; surviving infants have an extremely high incidence of neurologic sequelae.

The prognosis for a depressed fracture is usually good when treatment is early and adequate. When therapy is delayed, especially with a large depression, death may occur from pressure on vital areas of the brain. Because the natural history of depressed skull fractures in neonates has not been clearly elucidated, the outcome is uncertain for infants with smaller lesions managed either by simple observation or by surgery after significant delays.

Etiology.

Traumatic facial nerve palsy most often follows compression of the peripheral portion of the nerve, either near the stylomastoid foramen through which it emerges, or where the nerve traverses the ramus of the mandible. The neonate is vulnerable to these injuries because of the superficial course of the extracranial facial nerves. The nerve may be compressed by forceps, especially when the fetal head has been grasped obliquely. The condition also occurs after spontaneous deliveries in which prolonged pressure was applied by the maternal sacral promontory. Less frequently injury is sustained in utero, often in association with a mandibular deformity, by the persistent position of the fetal foot against the superior ramus of the mandible. An extremely rare cause is the pressure of a uterine tumor on the nerve.

This condition may occur rarely with simultaneous ipsilateral brachial plexus palsy, most likely secondary to compressive forces during delivery.²² Contributing factors include prolonged second stage of labor and midforceps delivery.

Traumatic facial nerve palsy may follow a contralateral injury to the CNS such as a temporal bone fracture, or hemorrhage, tissue destruction, or both to structures within the posterior fossa. This CNS injury is less frequent than peripheral nerve injury.

Clinical Manifestations.

Paralysis is usually apparent on the first or second day but may be present at birth. It usually does not increase in severity unless considerable edema occurs over the area of nerve trauma. The type and distribution of paralysis are different in central facial paralysis compared with peripheral paralysis.

Central paralysis is a spastic paralysis limited to the lower half or two thirds of the contralateral side of the face. The paralyzed side is smooth and full and often appears swollen. The nasolabial fold is obliterated, and the corner of the mouth droops. When the infant cries, the mouth is drawn to the normal side, the wrinkles are deeper on the normal side, and movement of the forehead and eyelid is unaffected. Usually other manifestations of intracranial injury appear, most often a sixth cranial nerve palsy.

Peripheral paralysis is flaccid and, when complete, involves the entire side of the face. When the infant is at rest, the only sign may be a persistently open eyelid on the affected side, caused by paralysis of the orbicular muscle of the eye. With crying, the findings are the same as in a central facial nerve injury, with the addition of a smooth forehead on the involved side. Because the tongue is not involved, feeding is not affected.

A small branch of the nerve may be injured, with involvement of only one group of facial muscles. Paralysis is then limited to the forehead, eyelid, or mouth. Peripheral paralysis caused by nerve injury distal to the geniculate ganglion may be accompanied by a hematotympanum on the same side.

Differential Diagnosis.

Central and peripheral facial nerve palsies must be distinguished from nuclear agenesis (Möbius syndrome). The latter frequently results in bilateral facial nerve palsy; the face is expressionless and immobile, suggesting muscle fibrosis. Other cranial nerve palsies and deformities of the ear, palate, tongue, mandible, and other bones may be associated with Möbius syndrome. Congenital absence or hypoplasia of the depressor muscle of the angle of the mouth also may simulate congenital facial palsy and has been associated with an increased incidence of other congenital anomalies.

Treatment.

No specific therapy is indicated for most facial palsies. If the paralysis is peripheral and complete, initial treatment should be directed at protecting the cornea with an eye pad and instilling 1% methylcellulose drops every 4 hours. The functional state of the nerve should be followed closely. Falco and colleagues²⁷ proposed the following comprehensive approach:

Prognosis.

Most facial palsies resolve spontaneously within several days; total recovery may require several weeks or months. Electrodiagnostic testing is beneficial in predicting recovery; repeatedly normal nerve excitability indicates a good prognosis, but decreased or absent excitability early in the course suggests a poor outlook. The subsequent appearance of muscle fibrillation potentials indicates nerve degeneration. The prognosis in surgically treated infants worsens with increasing age at treatment.

Fractures and Dislocations of Facial Bones

Facial bone fractures may occur during passage through the birth canal, during forceps application and delivery, and during obstetric manipulation (most often the Mauriceau maneuver for delivery of the fetal head in a breech presentation). Manipulation may result in mandibular fractures and mandibular joint damage, but is rarely severe enough to cause separation of the symphysis of the mandible. Fracture of the nose may result in early respiratory distress and feeding difficulties. The most frequent nasal injury is dislocation of the cartilaginous part of the septum from the vomerine groove and columella. This may result from intrauterine factors such as a uterine tumor or persistent pressure on the nose by fetal small parts or during delivery from pressure on the nose by the symphysis pubis, sacral promontory, or perineum. The presence of nasal septal dislocation may be differentiated from the more common normal variant of a misshapen nose by a simple compression test in which the tip of the nose is compressed (Figure 30-8).²⁰ In the presence of septal dislocation, the nostrils collapse, and the deviated septum becomes more apparent; in the normal nose, no nasal deviation occurs with compression.

Infants who sustain nasal trauma during the birth process may demonstrate stridor and cyanosis, even in the absence of septal dislocation. Miller and co-workers⁶⁶ noted high nasal resistance in three such infants, of whom only one was found to have septal dislocation. The authors postulated the presence of edema and narrowed nasal passages from compression forces on the midface during delivery. The problem may be exaggerated by repeated nasal suctioning or transnasal bronchoscopy. These procedures and oral feeding should be avoided until the infant re-establishes normal nasal ventilation. Pulse oximetry measurements are useful in monitoring these infants.

Eyelids

Edema, suffusion, and ecchymoses of the eyelids are common, especially after face and brow presentations or forceps deliveries. Severely swollen lids should be forced open by an ophthalmologist for examination of the eyeball; retractors may be necessary. These findings usually resolve within a week without treatment, although an infant has been reported with totally everted upper eyelids that required suturing for 4 days before they would remain in the normal position.⁸⁰ Some believe that these injuries represent a possible cause of congenital ptosis.

A less common injury is laceration, including disruption of the lacrimal canaliculus. This has been associated with multiple upper-eyelid lacerations, including a full-thickness vertical wound lateral to the punctum and a full-thickness laceration through the lower eyelid with transection of the canaliculus after a low forceps delivery. Microsurgical repair of the lacrimal system and eyelids, including lacrimal intubation with a silicone stent, has been successful. Follow-up at 14 months revealed normal tear drainage with no amblyopia or residual deformity.⁴²

An infant has been reported with superficial eyelid lacerations caused by an internal fetal monitoring spiral electrode.⁵⁷ At delivery, the electrode was attached to the eyelid. Marked facial edema related to brow presentation apparently obscured the lacerations until 14 hours of age, when much of the edema had resolved. Periorbital edema was believed to have protected the infant from more serious injury to the eyelid and globe.

Lagophthalmos, the inability to close an eyelid, is an occasional finding thought to result from facial nerve injury by forceps pressure. It usually is unilateral. The exposed cornea should be protected by an eye pad and frequent use of methylcellulose drops. The condition usually resolves within a week.

Orbit

Orbital hemorrhage and fracture may follow direct pressure by the apex of one forceps blade, most often in high forceps extractions. In most instances, death occurs immediately. Surviving infants demonstrate traumatic eyelid changes, disturbances of extraocular muscle movements, and exophthalmos. The presence of the latter two findings warrants immediate ophthalmologic consultation. Subsequent management also may require neurosurgical and plastic surgery consultations.

Sympathetic Nervous System

Horner syndrome, resulting from cervical sympathetic nerve trauma, frequently accompanies lower brachial plexus injury. The syndrome consists of miosis, partial ptosis, slight enophthalmos, and anhidrosis of the ipsilateral side of the face. Although small, the pupil reacts to light. The presence of neurologic signs indicating brachial plexus injury helps distinguish this syndrome from intracranial hemorrhage as a cause of anisocoria. Pigmentation of the ipsilateral iris is frequently delayed to several months of age; occasionally, pigmentation never occurs. Resolution of other signs of the syndrome depends on whether the injury to the nerve is transient or permanent.

Subconjunctival Hemorrhage

Subconjunctival hemorrhage, characterized by bright red patches on the bulbar conjunctiva, is a relatively common finding in the neonate. It may be found after a difficult delivery but often is noted after easy, completely uncomplicated deliveries. This finding is considered to result from increased venous pressure in the infant’s head and neck, produced by obstruction to venous return consequent to compression of the fetal thorax or abdomen by uterine contractions during labor. If the infant is otherwise well, management consists of reassuring the parents. The blood is usually absorbed within 1 to 2 weeks. As the blood pigments break down and are absorbed, the color changes from bright red to orange and yellow.

External Ocular Muscles

Injury involving the external ocular muscles may result from direct trauma to the cranial nerve (in the form of compression or surrounding hemorrhages) or from hemorrhage into the muscle sheath, with subsequent fibrosis. The sixth cranial nerve (abducens) is the most frequently injured cranial nerve because of its long intracranial course; the result is paralysis of the lateral rectus muscle. This injury may follow a tentorial laceration with extravasation of a small amount of blood around the intracranial portion of the nerve. The involvement may be mild and transient; internal strabismus noted at birth may resolve gradually within 1 to 2 months. The seventh cranial nerve may be injured simultaneously with the sixth nerve by compression with forceps. Improvement in lateral gaze of the affected eye may appear within 1 to 2 months. Alternate patching of either eye in the severely affected infant maintains visual acuity until, with time, maximal improvement has occurred. At 6 months, the degree of nerve regeneration may be evaluated. Some infants subsequently require surgical repair of the strabismus.

Fourth cranial nerve (trochlear) palsy occurs infrequently. It may follow small brainstem hemorrhages with nuclear damage. The affected muscle is the superior oblique, which mainly turns the eye inferiorly and medially. This condition is difficult to identify in the newborn infant. Surgical correction may be necessary later.

Third cranial nerve (oculomotor) palsy, when complete, causes paralysis of the inferior oblique and medial, superior, and inferior rectus muscles. This results in ptosis, a dilated fixed pupil, and outward and downward deviation of the eye, with inability to adduct or elevate up and in, or up and out, or to depress down and out. This palsy also may occur in partial form, with or without pupillary involvement. Partial palsies may recover function spontaneously within several months, whereas complete palsies usually require surgical intervention.

Optic Nerve

The optic nerve may be injured directly by a fracture in the region of the optic canal or from a shearing force on the nerve, with resultant hemorrhage into the nerve sheath. The latter injury seldom is recognized because of the more apparent and severe changes in the sensorium. Occasionally, a fracture through the optic foramen results in formation of callus, which slowly compresses the nerve. A difficult forceps delivery is a frequent preceding event.

Cornea

A diffuse or streaky haziness of the cornea is relatively common. This is usually caused by edema related to the birth process, but also may follow use of a silver nitrate solution more concentrated than 1%. The haziness usually disappears in 7 to 10 days. When it persists, a rupture of the Descemet membrane has probably occurred, usually because of malpositioning of forceps at delivery. The consequence of a ruptured Descemet membrane is a leukoma or diffuse white opacity of the cornea. This results from interstitial damage of the substantia propria by fluids entering through the tear in the membrane. These leukomas are often permanent and, despite patching of the contralateral eye and use of glasses, are accompanied by a high incidence of amblyopia and strabismus.

A ruptured Descemet membrane has been reported after a prolonged delivery in which low forceps were used after unsuccessful attempts at vacuum extraction.⁹² Because of significant corneal astigmatism at 2 months of age, a gas-permeable hard contact lens was applied. Patching of the contralateral eye was continued. Assessment of visual acuity at 13 months, with the use of spatial frequency sweep visual-evoked potentials, demonstrated an excellent visual result.

Intraocular Hemorrhage

Trauma at birth may result in retinal hemorrhage, hyphema, or vitreous hemorrhage, with retinal hemorrhage the most common. The cause is most likely compression of the fetal head, resulting in venous congestion. The fetal head is compressed two to four times more forcefully than other fetal parts during the second stage of labor. Retinal hemorrhage is more common in primiparous deliveries and after forceps or vacuum extraction; it is rare after cesarean section. It may occur in normal deliveries. The most common lesion is the flame-shaped or streak hemorrhage found mainly near the disk and sparing the macula and extreme periphery; it usually disappears within 1 to 3 days (occasionally 5 days) with no residual effects. Rarely, hemorrhages may take as long as 21 days to resolve. It is critical to identify birth-related retinal hemorrhages and document their presence on the infant’s chart, to avoid subsequent suspicion of child abuse. Retinal hemorrhages may reduce the resolving power of the macula, either bilaterally to produce nystagmus or unilaterally to produce amblyopia, which may not always respond to prolonged covering of the fixing eye with improvement of the amblyopic eye.

Hyphemas and vitreous hemorrhages usually result from misplacement of forceps and often are associated with ruptures of the Descemet membrane. One infant has been described in whom a hyphema developed in one eye after spontaneous delivery.⁷⁶ The hyphema usually is clear of gross blood within 5 days; during this time the infant should be handled gently and fed frequently to minimize crying and agitation. If blood persists or secondary hemorrhage occurs, systemic administration of acetazolamide (Diamox) and surgical removal of blood may be necessary.

Vitreous hemorrhage is manifested by large vitreous floaters, blood pigment seen with the slit lamp, and an absent red reflex. The prognosis is guarded; if resolution does not occur in 6 to 12 months, surgical correction should be considered.