Dominantly-inherited lop ears.

We describe a four-generation Chinese family that included five members who had an isolated bilateral lop ear anomaly. The presentation suggested a dominant mode of inheritance. The absence of male-to-male transmission does not exclude an X-linked dominant mode of inheritance. Since the phenotypic anomaly of the male proband was no more severe than the affected female members, an autosomal dominant mode of inheritance is most likely.

Febrile seizures.

Febrile seizures are the most frequent of seizure disorders in childhood. Febrile seizures are most common in children between 6 months and 3 years of age, with a peak incidence at about 18 months. Approximately 30% to 40% of children who experience a febrile seizure will have a recurrence. The majority of febrile seizures occur within 24 hours of the onset of the fever. Febrile seizures can be simple or complex. Diagnostic studies are usually not necessary. Febrile seizures usually are self-limited, and intervention to stop the seizure often is unnecessary. When possible, the cause of the fever should be treated. Continuous preventative anticonvulsant therapy is not recommended for children with either simple or complex febrile seizures. The use of intermittent anticonvulsant therapy is not routinely indicated. Parental educational and counseling is important. The prognosis is excellent.

Tuberous sclerosis complex: a review.

Tuberous sclerosis complex (TSC) is an inherited neurocutaneous disorder characterized by the potential for hamartoma formation in almost every organ. The inheritance is autosomal dominant with almost complete penetrance but variable expressivity. The two gene loci that code for TSC are TSC1, located on chromosome 9q34, and TSC2 on 16p13.3. TSC complex may affect the skin, central nervous system, kidneys, heart, eyes, blood vessels, lungs, bone, and gastrointestinal tract. The diagnosis of TSC is based on the identification of hamartomas in more than one organ system. Treatment should be symptomatic and organ specific. A multidisciplinary management approach is necessary.

A giant congenital nevus spilus in an 8-year-old girl.

Congenital nevus spilus is rare, and the lesion is usually small. This report describes an 8-year-old girl with a giant congenital nevus spilus that involved the left side of her abdomen, perineal area, and left upper thigh. The patient also had a Mongolian spot in the lumbosacral area.

An approach to daytime wetting in children.

Daytime wetting is a common problem with various causes that can usually be identified through a careful history, thorough physical examination, and urinalysis. Conservative approaches to therapy have a successful outcome in most children. Invasive diagnostic imaging studies and pharmacologic or surgical intervention are necessary only for carefully selected children.

Herpes zoster in childhood.

Herpes zoster is caused by reactivation of latent varicella-zoster virus that resides in a dorsal root ganglion. Herpes zoster can develop any time after a primary infection. Because varicella vaccine is a live attenuated virus, herpes zoster can develop in a vaccine recipient. The incidence of herpes zoster among vaccine recipients is about 14 cases per 100,000 person-years. In young children, herpes zoster has a predilection for areas supplied by the cervical and sacral dermatomes. The most common complications are secondary bacterial infection, depigmentation, and scarring. Although the diagnosis of herpes zoster is based on a distinct clinical appearance, viral DNA analysis of the lesion by polymerase chain reaction or restriction fragment length polymorphism is necessary to differentiate wild from vaccine-type viruses. Acyclovir is the treatment of choice for herpes zoster.

Treatment of labial fusion with topical estrogen therapy.

Twenty girls with labial fusion that covered at least 50% of the vaginal opening were treated with a topical estrogen cream. The cream was precisely applied to the fused area twice a day until the adhesions were totally lysed. After separation of the adhesions, a petroleum ointment (Vaseline) was applied twice a day to the labia minora for at least 1 month. The introital area was carefully rinsed twice each day before application of either the estrogen cream or the petroleum ointment. The mean age of the studied population was 13.2 months (range, 2 to 38 months). The mean duration of estrogen treatment was 2.4 months (range, 1 to 3.5 months). All patients were successfully treated. Five patients developed vulval pigmentation, which lasted for a mean of 1.5 months. One patient developed breast enlargement, which lasted for 1 month. The mean duration of follow-up was 3.1 months, and there was no recurrence of labial fusion during the period of follow-up. We conclude that treatment of labial fusion with topical estrogen therapy is safe and effective.

Current status of cryptorchidism.

Cryptorchidism is present at birth in 3% to 5% of term male infants. The incidence is higher in preterm and low birth weight infants. The two most important possible sequelae are infertility and testicular tumor. Diagnostic studies are usually not necessary if the undescended testis is palpable. Laparoscopy has replaced imaging studies for localization of a nonpalpable testis and might also obviate the need for exploration of the groin. The recommended age for treatment has progressively decreased as our understanding of the problem has improved. Current information suggests that the placement of the testis in the scrotum should be accomplished by 1 year of age to maximize the potential for fertility. Treatment options include surgical relocation or hormonal therapy. The lower the pretreatment position of the testis, the better the success rate for either method.

Childhood cervical lymphadenopathy.

Cervical lymphadenopathy is a common problem in children. The condition most commonly represents a transient response to a benign local or generalized infection, but occasionally it might herald the presence of a more serious disorder. Acute bilateral cervical lymphadenopathy usually is caused by a viral upper respiratory tract infection or streptococcal pharyngitis. Acute unilateral cervical lymphadenitis is caused by streptococcal or staphylococcal infection in 40% to 80% of cases. The most common causes of subacute or chronic lymphadenitis are cat scratch disease, mycobacterial infection, and toxoplasmosis. Supraclavicular or posterior cervical lymphadenopathy carries a much higher risk for malignancies than does anterior cervical lymphadenopathy. Generalized lymphadenopathy is often caused by a viral infection, and less frequently by malignancies, collagen vascular diseases, and medications. Laboratory tests are not necessary in the majority of children with cervical lymphadenopathy. Most cases of lymphadenopathy are self-limited and require no treatment. The treatment of acute bacterial cervical lymphadenitis without a known primary source should provide adequate coverage for both Staphylococcus aureus and group A beta hemolytic streptococci.