A term newborn is undergoing routine evaluation in the nursery. The boy was born 12 hours ago via spontaneous vaginal delivery to a 24-year-old woman. His mother had appropriate prenatal care, and her pregnancy was uncomplicated. Delivery was unremarkable, and the patient has had a normal nursery course. He received the hepatitis B vaccine, erythromycin eye ointment, and intramuscular vitamin K after delivery. Family history reveals factor VIII deficiency in the patient's paternal grandfather. Vital signs are normal. Physical examination shows a sleeping newborn with normal tone. A 1/6 systolic murmur is best heard at the left sternal border. Peripheral pulses are strong. Genitourinary examination shows a penis of normal length with no curvature. The urethral meatus extends to the corona on the ventral aspect of the penis, and the foreskin has a dorsal hood appearance. The testes are present bilaterally within the scrotum. The patient urinates through the urethral meatus during examination. The father requests that a circumcision be performed. Which of the following is the best next step in management of this patient?

Question

A term newborn is undergoing routine evaluation in the nursery.  The boy was born 12 hours ago via spontaneous vaginal delivery to a 24-year-old woman.  His mother had appropriate prenatal care, and her pregnancy was uncomplicated.  Delivery was unremarkable, and the patient has had a normal nursery course.  He received the hepatitis B vaccine, erythromycin eye ointment, and intramuscular vitamin K after delivery.  Family history reveals factor VIII deficiency in the patient's paternal grandfather.  Vital signs are normal.  Physical examination shows a sleeping newborn with normal tone.  A 1/6 systolic murmur is best heard at the left sternal border.  Peripheral pulses are strong.  Genitourinary examination shows a penis of normal length with no curvature.  The urethral meatus extends to the corona on the ventral aspect of the penis, and the foreskin has a dorsal hood appearance.  The testes are present bilaterally within the scrotum.  The patient urinates through the urethral meatus during examination.  The father requests that a circumcision be performed.  Which of the following is the best next step in management of this patient?

Quizplus · Accepted Answer

11ec25c8_d6df_5469_bc81_fdfaf1463ea5_MD0003_00 This newborn boy with a urethral meatus extending to the corona has hypospadias, defined as a ventrally displaced urethral opening. This embryologic defect results from failure of the urethral folds to fuse during early fetal development. In addition, incomplete ventral foreskin closure leads to a dorsal hood appearance (ie, extra dorsal foreskin with deficient ventral foreskin). Penile curvature (ie, chordee) may also be seen in patients with hypospadias. Diagnosis of hypospadias is based on clinical examination. Further workup is not required in cases of isolated hypospadias, as in this patient. The first step in management of hypospadias is urologic evaluation because most cases require surgical correction. Circumcision is deferred until after evaluation because the foreskin may be required for hypospadias repair. In addition, placement of conventional circumcision devices over an asymmetric foreskin may be unsafe. (Choice A) Coagulation studies are indicated for a newborn who may have inherited a coagulopathy. However, a paternal family history of factor VIII deficiency (hemophilia A) does not warrant laboratory evaluation because this disorder is X-linked and passed to boys from the mother. (Choice B) Echocardiogram is performed in newborns with signs of congenital heart disease (eg, tachypnea, cyanosis, weak pulses). This 1-day-old's soft, systolic murmur at the left sternal border is consistent with a physiologic ductus arteriosus, which is a normal finding in the first few days of life before spontaneous closure. (Choice C) Severe hypospadias (eg, scrotal/perineal urethral meatus) with bilateral, nonpalpable gonads may be indicative of a disorder of sex development (DSD) in which a female infant is virilized or a male infant is undervirilized. Karyotype is performed to confirm the genotype in these patients. However, this patient has descended testes, making a DSD unlikely. (Choice D) Renal ultrasound is indicated for patients with hypospadias plus other congenital anomalies (eg, aniridia) indicative of a genetic condition (eg, WAGR syndrome [Wilms tumor, Aniridia, Genitourinary malformations, and mental Retardation]). In contrast, isolated hypospadias, as seen in this patient, is not commonly associated with renal abnormalities because the fetal kidneys develop prior to the formation of external genitalia. Educational objective: Hypospadias is characterized by a ventrally displaced urethral opening and dorsal hooded foreskin. Urologic evaluation is required prior to circumcision because the foreskin may be needed for hypospadias repair, and conventional circumcision techniques may be unsafe. References: Hypospadias, all there is to know. (http://www.ncbi.nlm.nih.gov/pubmed/28190103)

A Term Newborn Is Undergoing Routine Evaluation in the Nursery