Division of Urology

Urology Case Studies

A scrotal mass

Presenting concern

An 11-month old boy presented to the Emergency Department at a local hospital with his parents. Scrotal Mass
Scrotal Mass
EnlargeHis parents reported a two-day history of “not acting like himself.” He was less interactive than usual and had a decreased appetite. On this day, he woke up from a nap crying and grabbing the right side of his scrotum. He was taken to a local emergency room and found to have a fever of 101.3 degrees Fahrenheit and WBC of 28. His physical exam was notable for bilateral hydroceles, which had reportedly been present since birth, and echogenic bowel within the right hemiscrotum. There was no evidence of testicular torsion. He was transferred to The Children’s Hospital of Philadelphia (CHOP) to rule out an incarcerated hernia.

Evaluation

Upon arriving at CHOP, his vital signs were stable and his temperature had come down to 100.4 degrees Fahrenheit. He continued to be crying and fussy with one hand over the right hemiscrotum at all times. His abdomen was soft and non-tender with no masses appreciated. His scrotal exam revealed a left descended testicle in normal lie within the scrotum and no palpable masses. The right testicle was palpable and descended. A discretely separate marble-size paratesticular mass could be palpated along the right hemiscrotum. No hernia was detected.

A KUB revealed a heterogeneous cluster of calcifications projecting over the partially visualized right scrotum, but no abnormal intra-abdominal calcifications were identified. In addition to the known bilateral hydroceles, a scrotal ultrasound showed two and possibly three circular, heterogeneous, but primarily hyperechoic partially calcified masses within the right scrotum, completely separate from the testis. No hernia was visualized.

The differential diagnoses included benign etiologies such as granulomas and meconium periorchitis, and more aggressive tumors-like rhabdomyosarcoma, metastatic neuroblastoma and lymphoma.

Treatment

The child was admitted to the hospital and given intravenous antibiotics. He was taken to the operating room for a right inguinal and scrotal exploration. Scrotal Mass
Removed Scrotal Mass
EnlargeA completely detached 1.5 cm marble-like mass fell out of the tunica vaginalis sac once it was opened. The processus vaginalis was closed. Frozen sections of the mass were sent to pathology and read as likely meconium with no evidence of malignancy. The final surgical pathology confirmed meconium periorchitis. A thin, fibrous-walled cyst filled with a fibromyxoid material and numerous foci of dystrophic calcification was described. Pigment laden macrophages, fine hairs and rare degenerating squamous cells were also identified in the sample tissue.

Following surgery, the child did well and he was discharged from our clinic.

Discussion

Meconium periorchitis is a very rare mass-like lesion that results from the infiltration of meconium peritonitis into the scrotal sac through a patent processus vaginalis. The meconium leaks into the scrotum and quickly turns into a fibrous solid mass. While initially soft, the mass develops calcifications over time due to chronic inflammation.

Approximately 60 cases are described in the literature, including a few which were diagnosed prenatally. Although about 25 percent of boys with meconium orchitis are reported to have no scrotal anomaly at birth, many are reported to have had reducible hydroceles, often on both sides, at birth.

References

Jeanty C, Bircher A, Turner C (2009). Prenatal diagnosis of meconium periorchitis and review of the literature. J Ultrasound Med 28(12): 1729-34.

Krause H, Hass HJ, von Rohden L, Kroker S, Buhtz P, Jaekel A, Meyer F (2009). Meconium-induced periorchitis. BMJ Case Reports. doi: 10.1136/bcr.042009.1801.

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Disorders of sex development (DSD) case

At The Children’s Hospital of Philadelphia’s Division of Urology, we believe a multidisciplinary team is critical to providing the best care for children with disorders of sex development (DSD).

The following cases represent the kinds of children referred to us for evaluation, and their management exemplifies the team approach we take in coming to an accurate diagnosis for children with DSD.

Our team, led by Attending Urologist Thomas Kolon, MD, is comprised of colleagues in urology, endocrinology and genetics, and our pediatric psychologist, Aileen Schast, Ph.D., is available to families during the diagnostic process and throughout a child’s care at CHOP. We work together to ensure each child’s evaluation is efficient and thorough.

Case study – patient 1

Patient 1 is a 16-year, 6-month old female who was originally referred to the Division of Endocrinology at CHOP for primary amenhorrea.

Of note, the patient has always been tall. She grew 2 inches in the past year and is at the 99th percentile for her age.

The patient was started on estrogen therapy and referred to Urology for further workup.

Work-up and diagnosis

The working diagnoses for patient 1 following her initial workup were probable complete androgen insensitivity vs. early testicular/gonadal failure (dysgenesis).

We recommended an MRI with gadolinium of her abdomen and pelvis to further delineate her anatomy. The MRI showed an infantile uterus with no discernible gonads. During a laparoscopic evaluation, two dysplastic gonads attached to small fallopian tubes and a rudimentary uterus were visualized. Given these findings and her karyotype analysis, it was determined that gonadectomies would be performed due to the increased risk of cancer. A fallopian tube was identified on the right and a streak gonad containing a microscopic gonadoblastoma (3 mm) was identified on the left.

Based on these findings, a final diagnosis of pure gonadal dysgenesis (Swyer Syndrome) was confirmed. Patient 1 has continued follow-up with endocrinology for estrogen management, with Dr. Schast for counseling related to coming to terms with her diagnosis, and she is scheduled for consultation with oncology regarding the gonadoblastoma.

Case study – patient 2

Patient 2 is a 2-day-old infant who was transferred to CHOP from an outside hospital with a referral concern of ambiguous genitalia. The child was born at 41 weeks gestation with no complications and no other obvious medical concerns.

Work-up and diagnosis

For patient 2 an MRI at 9 weeks of age revealed a left inguinal gonad, no discernible right gonad, and a possible right hemiuterus. Bloodwork was done at the time of the MRI to assess gonadotropins and androgen production at the expected normal gonadotropin surge at 60-90d of life.

We recommended an exam under anesthesia with exploration of the pelvic structures. Although the parents had been referring to their baby as a girl and had named her and dressed her accordingly, they requested that we not do anything that would prevent the gender of rearing from being male if that is what they later decided. Thus, it was decided that if the palpable gonad was found to be a testis, it would be brought into the labioscrotal folds. The parents wanted no genital reconstruction at that time.

On cystoscopy, a vaginal canal and abnormal cervix were visible. During the inguinal exploration, a dysgenetic testis with a completely detached epididymis was seen on the left and a streak gonad was found on the right. These findings confirmed the diagnosis of mixed gonadal dysgenesis. The left testis was brought to the labioscrotal folds and the streak gonad on the right was removed. In follow-up discussions with the parents, they decided to remove the left severely dysgenetic testis but deferred all other genital reconstruction.

We will continue to follow patient 2 and her family throughout her childhood, in conjunction with our colleagues in endocrinology.

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