Pediatric emergency medicine trisk 641

not uncommon and is thought to occur as a result of birth trauma. Clinical
manifestations depend on the degree of hemorrhage, and typically include signs
of anemia, and rarely adrenal insufficiency (poor feeding, vomiting, diarrhea,
obstipation, dehydration, irritability, hypoglycemia, uremia, and shock).
Treatment is largely supportive, and may include corticosteroids in the setting of
adrenal insufficiency. Rarely, surgical intervention for vessel ligation or
adrenalectomy is warranted. Neuroblastomas are the most common cause of
neonatal tumors, and occur most commonly in the adrenal gland. These can be
distinguished from adrenal hemorrhage based on US, and confirmed with urine
homovanillic acid (HVA) and vanillylmandelic acid (VMA) levels.
Pelvic Masses
Ovarian cysts can result from maternal hormonal stimulation in utero and often
are diagnosed in the fetal period, where the vast majority resolves prior to
delivery. For those that persist into the neonatal period, expectant management is
warranted as again most will resolve spontaneously. Large or complex cysts may
be at risk for torsion, which should be treated surgically. Additional
complications are rare but can include rupture with resultant hemoperitoneum or
bowel obstruction. Smaller cysts will typically resolve with expectant
management alone.

Genitourinary Anomalies
Goals of Treatment
Most genitourinary anomalies in the newborn are benign lesions or lesions that
can be monitored by the pediatrician in an outpatient setting. The goal of
treatment is to recognize the anomalies that require urgent evaluation and
treatment.
CLINICAL PEARLS AND PITFALLS
Vaginal discharge in the female neonate is typically benign, and
represents withdrawal of maternal hormones.
Patients with ambiguous genitalia, particularly infants with
masculinization of female-appearing genitalia, should be evaluated for

congenital adrenal hyperplasia, which can result in a life-threatening
adrenal crisis if untreated.
Circumcision


Complications from circumcision are rare, and in the newborn, typically occur 2
to 5 times per 1,000 procedures. Most common complications include bleeding,
particularly if there is an underlying coagulopathy. For minor bleeding, treatment
includes manual pressure and a compression dressing. Circumferential dressings
need to be monitored for penile ischemia or urinary retention. If bleeding
continues and a specific bleeding vessel can be identified, treatment includes
topical administration of lidocaine with epinephrine and/or sutures. If bleeding is
generalized and persistent, the infant should be evaluated for a coagulopathy with
complete blood count, and coagulation studies.
Wound infection is another infrequent complication of circumcision. It is
typically localized, with evidence of local redness, irritation, or drainage. If
localized, treatment includes topical antibiotic ointment, although caregivers
should be educated on generalized symptoms of fever, irritability, or lethargy. If
present, infants with generalized symptoms should be evaluated for sepsis with
blood, urine, and CSF cultures and parenteral antibiotics.
Surgical complications of the procedure can include injury to the urethra or
glans. If injury is suspected, urgent pediatric urology consultation should be
obtained. If the circumcision has not been completed, any prepuce or redundant
skin should be left intact as this tissue may be used in the repair. Similarly, if
there is evidence of hypospadias, circumcision should be deferred and referred to
pediatric urology, for repair. Urethrocutaneous fistulas can also result from
circumcision, although these can be corrected as an outpatient procedure several
months after the initial procedures. If the glans is injured or amputated during the
procedure, the severed tissue should be wrapped in saline-soaked gauze, placed
indirectly on ice. An emergent pediatric urology consult should be obtained, as

there have been reports of successful reattachment if it is performed within an
hour of injury. Additional glans injury can occur if the Plastibell ring, which
normally sits over the glans for several days until the skin sloughs and ring falls
off, is displaced. If the ring cannot be removed easily, it may need to be removed
used ring cutters, particularly in the setting of constriction or ischemia.
Vaginal Discharge
Newborn females may present with either a milky white or blood-tinged vaginal
discharge. Both can be attributed to withdrawal from maternal hormones, and
need nothing more than conservative management, observance, and reassurance
to the parents. Occasionally, a girl with prolapsed urethra will present with “blood
in the diaper.” The prolapsed urethra is recognizable as an annular, beefy red or
purple mass between the labia.


Ambiguous Genitalia
A normal genital examination in newborn girls includes a fully opening vagina
(without posterior labial fusion), clitoris width of 2 to 6 mm, and absence of
gonads in the labia majora or inguinal region. In boys, the urethral opening
should be at the tip of the glans, with a stretched penile length of 2.5 to 5 cm, and
bilateral testes in the scrotal sacs. Patients with male-appearing genitalia but
micropenis, moderate to severe hypospadias or bilateral cryptorchidism, or
patient with female-appearing genitalia but posterior labial fusion, clitoromegaly,
or a labial/inguinal mass, require further evaluation for an intersex disorder.
Diagnostic evaluation includes peripheral blood karyotype, serum testosterone
and 17-OH progesterone levels, and pelvic US. The most immediate concern is
when ambiguous genitalia results from CAH. Typically, adrenal insufficiency will
present between 3 and 21 days. Females with CAH are more easily recognized by
abnormal masculinization of the external genitalia. Early signs of adrenal
insufficiency may include lethargy, decrease oral intake, increased emesis, failure
to thrive, and weight loss. In severe cases, patients will present with obtundation,

hypotension, gray appearance, cardiac arrhythmias (due to hyperkalemia), or
seizures (due to hyponatremia or hypoglycemia). For patients with suspected
CAH, serum electrolytes and glucose should be checked, and any abnormalities
corrected, and cortisol replacement should be instituted. In the salt-wasting form
of CAH, patients may also require mineralocorticoid replacement and salt
supplementation.

NEONATAL NEUROLOGIC EMERGENCIES
Goals of Treatment
Neurologic findings in neonates are subtle. Seizures are easily missed. Irritability
or lethargy may be the only signs of systemic infection. The goals of treatment
are to recognize these subtle findings and to aggressively treat underlying causes,
such as hypoglycemia, hyponatremia, and infections.
RELATED CHAPTERS
Signs and Symptoms
Apnea: Chapter 14
Medical, Surgical, and Trauma Emergencies
Neurologic Emergencies: Chapter 97


Seizures
CLINICAL PEARLS AND PITFALLS
Clinical seizures in the neonate are very subtle due to incomplete
myelination of the motor pathways; infants can present with tongue
thrusting, lip smacking, bicycling, or intermittent repetitive motions of a
single extremity or trunk; generalized clonic–tonic movements are
unlikely manifestations.
Neonatal seizures can be due to hypoglycemia and/or electrolyte
abnormalities, particularly from incorrect formula preparation.
Neonatal seizures may benefit from pyridoxine.

Current Evidence
Because of rapid, continued development of the neonatal brain, both clinical and
electroencephalographic (EEG) seizures of the newborn, vary dramatically from
the older child, so that recognizing the seizing infant remains a major challenge to
clinicians. Therefore, the true incidence of neonatal seizures remains unclear,
with reported ranges between 0.5% in term infants and 22% in preterm infants.
The effect of neonatal seizures on outcome is largely influenced by the
underlying disease process. However, regardless of etiology, it is believed that
seizures in the neonate can disrupt normal biochemical pathways responsible for
the development and maturation of the CNS. Prolonged neonatal seizures can
lower the seizure threshold in later life and are associated with learning and
memory impairments in adults with seizure disorders. Complicating outcome
studies of neonatal seizures is that many antiepileptic medications may contribute
to medication-induced brain injury and/or teratogenic effects on the brain
development, particularly with prolonged use.
Goals of Treatment
The primary goal of treatment is early recognition of neonatal seizures,
identification of the etiology, and control of the seizure as quickly as possible.
True seizure activity should be distinguished from more benign, nonepileptic
behaviors of newborns, such as tremors, jitteriness, or benign sleep myoclonus. If
the seizure is due to hypoglycemia or electrolyte anomalies, initial treatment
should be correction of the underlying metabolic abnormality. If antiepileptic
medications are used to treat seizures, it is important to also initiate EEG