Laurence Baskin, MD

A newborn baby boy had a tough forceps delivery. Exhibit 1 (above) is a gross photograph of his genitalia.

Sonogram of the right kidney and retroperitoneum

Exhibits 2 and 3 are two representative sonographic images of right kidney.

Continue to the next page for the answer.

Adrenal hemorrhage (with scrotal ecchymosis)

Forceps delivery can be associated with trauma. In this baby, a scrotal sonogram is indicated secondary to the color changes on the physical exam to rule out testicular fracture/hematoma/torsion.

On critical evaluation of the genital photograph, (Exhibit 1) the scrotum looks symmetric except for color. Some swelling and changes in the size of the testis would be expected in the case of in utero torsion.

Forceps deliveries are also associated with adrenal trauma, the adrenal gland being relatively large in the fetus and neonate. Therefore, a sonogram of the kidneys is also indicated, which in this case shows the typical complex adrenal hemorrhage (Exhibits 2 and 3). This complex mass can be confused with tumors, the most common being neuroblastoma. However, common things happen more commonly, and adrenal hemorrhage in the setting of a traumatic delivery is the most likely diagnosis.

MRI showing a suprarenal complex mass above the right kidney consistent with adrenal hemorrhage

Obtaining 24-hour urine for VMA and OVA and potentially an MRI (Exhibit 4) will rule out neuroblastoma. A better choice is follow-up with physical exam, sonogram, and acutely serial hematocrits, which will show resolution of the scrotal ecchymosis and adrenal hemorrhage, ruling out a duplex system with an ectopic ureter, tumor, and segmental renal dysplasia.

 professor of pediatric urology at the University of California, San Francisco, is section editor for 

Hemiscrotal hematoma in the newborn-don't forget adrenal hemorrhage. 

Adrenal haemorrhage in term neonates: a retrospective study from the period 2001-2013. 

J Matern Fetal Neonatal Med 2015; 28:2062-5.

Neonatal adrenal hematoma: various modes of presentation. 

Articles

Laurence Baskin, MD, presents the case of a baby boy who had a tough forceps delivery, with accompanying sonographic images.

Quiz: Baby boy with difficult forceps delivery

	Exhibit 1. CT scan of the pelvis with a urinary catheter in place.

	A 16-year-old female presents with irritative voiding symptoms progressing to “urinary retention.” Evaluation with a computed tomography scan is shown in Exhibits 1 and 2.

	Exhibit 3. The pelvic aspect of the sonogram is shown in Exhibit 3 after injecting 150 ccs of saline into the urinary catheter.

	Evaluation with a renal, bladder, pelvic sonogram shows two normal kidneys. 

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Continue to the next page for the answers.

	The differential diagnosis of a large, fluid-filled pelvic mass in a female should always include Mullerian structures. In this case, the large mass is a simple ovarian cyst (causing all of the irritative symptoms), and the urinary catheter is in the decompressed bladder off to the patient’s left. The patient has two normal kidneys (which would be a key fact to sort out early and not shown in the imaging), which rules out a duplicated hemivagina with one side obstructed and an ipsilateral pelvic renal nubbin (OHVIRA syndrome) as well as a duplicated system with an upper pole obstructed ureter from ureteral ectopy and/or a ureterocele. In addition, obstructed vaginas tend to have old blood and not simple fluid as shown in this case. In males, an enlarged prostatic utricle and seminal vesicle cyst should be considered in the differential diagnosis.

	The urinary catheter irrigated without issues and could be seen on real-time sonogram as separate from the large ovarian cyst. The cyst was drained and removed laparoscopically (Exhibits 4 and 5), along with the effaced right ovary with resolution of her symptoms.

	Exhibit 4. Laparoscopic image of large simple ovarian cyst about to be drained.

	Exhibit 5. Laparoscopic image collapsed simple ovarian cyst after percutaneous drainage.

 professor of pediatric urology at the University of California, San Francisco, is section editor for 

	Ovarian pathology for the pediatric urologist. 

	Evaluation, Treatment and Follow-up Results of Ovarian Cysts in Childhood and Adolescence: A Multicenter, Retrospective Study of 100 Patients. 

A 16-year-old female presents with irritative voiding symptoms progressing to “urinary retention.” Evaluation with a renal, bladder, pelvic sonogram shows two normal kidneys. What is the differential diagnosis?

[Quiz]: Voiding symptoms progressing to retention

	A 3-month old baby born with atypical genitalia (Exhibit 1) has an XY karyotype.

	The baby undergoes diagnostic laparoscopy. Exhibit 2 is an overview of the patient’s internal pelvic anatomy.

	Exhibit 3 is a high-power image of the left gonad. Exhibit 4 is a high-power image of the right gonad. The baby is doing clinically well without medication.

	The diagnosis is ovotesticular syndrome, one of the rarest forms of disorders of sex development, characterized by the presence of both ovarian and testicular tissue.

	The overview pelvic laparascopy image (Exhibit 2) shows retained Mullerian structures suggesting an absence of MIS production or function. The diagnosis is ultimately based on histologic analysis of what appears to be a homogeneous streak gonad on the patient’s left (Exhibit 3) and inhomogeneous streak on the right with an area of bulging gonadal tissue in the superior aspect of the laparoscopic image of the right gonad different from the classic streak appearance at the bottom aspect of the laparoscopic image (Exhibit 4). Histologically, the right gonad has both ovarian tissue and testicular tissue (the source of virilization in this XY baby).

	Patients with XO karyotype (Turner’s syndrome) would have bilateral streak gonads (such as on the left, Exhibit 3) without the external genitalia virilization and the XY karyotype. Patients with Gonadal dysgenesis (Swyers’ syndrome) would have an XY karyotype but without the external genitalia virilization; ie, a female phenotype. The streak gonads in patients with gonadal dysgenesis are at risk for cancer and should be removed since they are non-functional in respect to both hormonal function and future germ cell function.

	The external genitalia are not consistent with congenital adrenal hyperplasia (CAH). Note the open vaginal area inside of the expected common urogenital sinus (Exhibit 1). In addition, in CAH, you would expect normal ovaries internally, not streak gonads or an ovotestis.

	Exogenous testosterone exposure from the mother to the fetus could cause the atypical genitalia phenotype, especially if this occurred later in gestation but again, you would expect normal ovaries.

	In severe hypospadias, you would expect the gonads to look like testes, which is not the case.

	The majority of patients with ovotestes syndrome have an XY karyotype but can also have an XX karyotype. The key to suspecting the diagnosis is the inhomogeneous streak on the right compared to the left and the need for an explanation of virilization. Fertility in XY patients with ovotesticular syndrome has not been reported. Patients with ovotesticular syndrome are often raised as females. Future gender identity is difficult to predict, however, and patients and families will need long-term medical support and counseling.

	Fertility in disorders of sex development: A review. 

	Clinical review of 95 patients with 46, XX disorders of sex development based on the new Chicago classification. 

	Surgery in disorders of sex development (DSD) with a gender issue: If (why), when, and how? 

	To get weekly news from the leading news source for urologists, 

A 3-month old baby born with atypical genitalia has an XY karyotype. The baby undergoes diagnostic laparoscopy. The baby is doing clinically well without medication.

[Quiz]: Baby born with atypical genitalia, XY karyotype

	A 5-year-old girl is evaluated for recurrent urinary tract infections and lower abdomen pain. Her past surgical history is notable for endoscopic correction of left grade IV reflux at 2 years of age with postoperative confirmation of reflux resolution based on a nuclear cystogram and no hydronephrosis based on a sonogram. A KUB x-ray and computed tomography are obtained (Exhibit 1 and 2, respectively).

	What is the key finding(s) on the imaging studies?

. Calcifications within the left distal ureter

 Calcifications along the course of the left distal ureter

	This patient has calcifications after endoscopic treatment of vesicoureteral reflux along the course of the left ureter. The calcifications are most likely outside of the urinary tract based on lack of hydronephrosis on sonogram. A finite number of patients who undergo endoscopic VUR treatment end up with calcifications along the course of the previously injected endoscopic material. Long-term significance remains unknown, but it is generally thought to be benign since calcifications outside the urinary tract rarely, if ever, cause issues.

	The calcification therefore, is unlikely to be the cause of recurrent UTI. The recurrent UTI should be worked up with concern for bladder/bowel dysfunction with a focus on normal voiding and treating constipation.

Suggested reading                   

	Distal ureteral calcification secondary to deflux injection: a reality or myth? Palagiri AV, Dangle PP. 

	Radiologic features of implants after endoscopic treatment of vesicoureteral reflux in children. Cerwinka WH, Kaye JD, Scherz HC, Kirsch AJ, Grattan-Smith JD. 

	Calcification in a Deflux bleb thought to be a ureteral calculus in a child. Noe HN. 

	The fate of the iatrogenic retroperitoneal stone. Evans CP, Stoller ML. 

A 5-year-old girl is evaluated for recurrent urinary tract infections and lower abdomen pain. A kidney, ureter, and bladder x-ray and computed tomography are obtained.

Author | Laurence Baskin, MD

Articles

Quiz: Baby boy with difficult forceps delivery

[Quiz]: Voiding symptoms progressing to retention

[Quiz]: Baby born with atypical genitalia, XY karyotype

Quiz: Pediatric recurrent UTIs, abdomen pain