46,XX male disorders of sex development are rare. Approximately 80% of cases of testicular tissue differentiation may be due to translocation of SRY to the X chromosome or an autosome. SRY-negative 46,XX males show overexpression of pro-testis genes, such as SOX9 and SOX3, or failure of pro-ovarian genes, such as WNT4 and RSPO1, which induces testis differentiation, however, almost all testicles exhibit dysgenesis. Following inadequate exposure to androgens during the embryo stage, remnants of the Mullerian duct and incomplete closure of the urogenital sinus lead to enlargement of prostatic utricles. This condition is associated with proximal hypospadias and disorders of sex development. Many cases are asymptomatic, but show increased rates of postoperative complications and surgical failure.
A 5-year-old Chinese boy with scrotal hypospadias and bilateral cryptorchidism with prostatic utricles was presented. Gonadal histology showed ovo-testicular tissue on the right side and testicular tissue on the left side; all testicular tissue exhibited dysgenesis. Furthermore, chromosome karyotype analysis revealed 46,XX and, the presence of SRY was ruled out by polymerase chain reaction analysis. Whole-genome analysis showed the boy has a 1.4-Mb duplication in the Xq27.1q27.2 region (arr[hg19]Xq27.1q27.2:139585794-140996652) involving SOX3. No SOX3 duplication was observed in the parents, who had a normal phenotype.
We report the first case of an SRY-negative 46 XX male with prostatic utricle caused by SOX3 duplication. SOX3 duplication may cause sex reversal, and all 46,XX SRY-negative males should be screened for SOX3 mutations. Gonadal biopsy is recommended to evaluate ovarian and testicular tissue development. Testicular dysgenesis and low exposure to male hormones during fetal development can lead to enlarged prostatic utricles. Thus endoscopic examination should be performed preoperatively to detect prostatic utricles in SRY-negative 46,XX males to determine the surgical plan and reduce postoperative complications.

Prostatic utricle cyst is an embryological remnant of the Muller duct system, resulting from an incomplete regression of these structures during embryological development. Most of the time such congenital alteration is asymptomatic, but may sometimes present with various signs and symptoms, from urinary tract infections to episodes of haematospermia in a small percentage of cases.
We reported the case of a 42-years-old man who suffered from recurrent episodes of haematospermia for about 20 years; the instrumental examinations, first of all TRUS and then MRI evaluation, demonstrated the presence of a cystic formation, located in the posterior median site of the prostate closely to the prostatic urethra.
The cytological examination on the liquid taken by transperineal US-guided fine-needle aspiration, confirmed the presence of seminal fluid and normal prostatic tissue consistent with the diagnostic hypothesis of prostatic utricle cyst.
It\'s important to recognize the imaging characteristics of the prostatic utricle cyst since it may be one of the possible benign causes of hematospermia in the young adult subject.

Background: A prostatic utricle (PU) is an unusual pathology with most patients being asymptomatic. However, approximately 29% of patients may show lower urinary tract symptoms, recurrent urinary tract infections (UTI), postvoid dribbling, urethral discharge, epididymo-orchitis, stones, and secondary incontinence caused by urine trapping in the pouch and urinary retention. The standard treatment is through surgical resection, but it is only offered to patients with symptoms. Case summary: We report a case involving a six-year-old boy with congenital hypothyroidism and penoscrotal hypospadias who had previously undergone onlay urethroplasty for the proximal shaft, chordee release, orchidopexy for bilateral undescended testis, and laparoscopic herniorrhaphy for left inguinal hernia. However, the patient later evolved the repetition of UTI and right epididymo-orchitis. Cyclic voiding cystourethrography confirmed the presence of a cystic lesion communicating with the prostatic urethra from the utricle. The PU was then excised laparoscopically. The utricle was identified posterior to the bladder, and insertions of the vas deferens crossover into the utricle were detected by laparoscopy. The post-procedure course was uneventful. Conclusions: Laparoscopic resection of PUs offers a better exposure field, improved wound appearance, complete resection, and reduces the incidence of complications. During laparoscopy, the PU was clearly distinguished from the bladder or other pelvic organs. An incidental finding of vas deferens crossover has rarely been reported. A combined cystoscopy and laparoscopy for PU resection is executable, safe, and valid in this patient population.

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We describe herein the case of a patient whose primary complaints were episodic vertigo and \"depersonalization,\" a sensation of detachment from his own body.
This case study aims to further clinical knowledge and insight into the clinical evaluation of vertiginous patients with complaints of depersonalization.
This is a case study.
A retrospective chart review of vestibular function testing done on a vertiginous patient with complaints of depersonalization was performed.
Vestibular function testing revealed absent cervical and ocular vestibular evoked myogenic potentials on the left side with normal vHIT or video Head Impulse Test, videonystagmography, and rotational chair results, suggesting peripheral vestibular impairment isolated to the left saccule and utricle.
The otolith end organ impairment explains the patient\'s postural deviation to the left side during attempts to ambulate. We recommend that clinicians should be attentive to patient complaints of depersonalization and perform vestibular evoked myogenic potential testing to determine whether evidence of at least a unilateral peripheral otolith end organ impairment exists.

OBJECTIVE: Like other vestibular schwannomas developing in the internal auditory canal, intralabyrinthine schwannomas (ILS) may present with similar symptoms as in endolymphatic hydrops. Two different studies have described MR saccular hydrops in ~ 30% of internal auditory canal vestibular schwannomas, but this association has never been studied in ILS before. The aim of this work is to study the prevalence of a saccular dilation in ILS, on a T2-weigthed sequence at 3 T, compared to a control group.
METHODS: All patients presenting with typical ILS between January 2008 and October 2018 were included (n = 28, two patients with bilateral tumors) and compared to a control group (n = 53). All underwent a high-resolution T2-weighted 3D sequence (FIESTA-C). The height and width of the saccule were measured on a coronal plane by two radiologists.
RESULTS: The saccule was dilated on the side of the schwannoma in 47% of the cases (p = 0.0006 for the height, p = 0.0487 for the width). Bilateral saccular dilation was observed in 37% of the cases. There was a statistically significant correlation between the presence of a saccular hydrops and balance disorders (p = 0.02) as 50% of the patients with an intralabyrinthine schwannoma who presented with such symptoms had a saccular dilation.
CONCLUSIONS: Forty-seven percent of ILS are associated with homolateral saccular dilation, which is an MR sign of endolymphatic hydrops (bilateral in 37%) and it appears related to the presence of balance disorders. This opens new therapeutic potentialities with the possible use of anti-vertiginous drugs, which could have a beneficial effect on their clinical symptomatology.

OBJECTIVE: Vestibular schwannomas (VS) may present with similar symptoms endolymphatic hydrops. Association between hydrops and internal auditory canal VS has been described by Naganawa et al. (Neuroradiology 53:1009-1015, 2011), but has never been confirmed since. The aim of this work was to study the prevalence of a saccular dilation on a T2-weighted sequence at 3 T MRI in VS compared to a control group.
METHODS: All patients presenting with typical VS between May 2009 and July 2018 were included (n = 183) and compared to a control group (n = 53). All underwent a high-resolution T2-weighted 3D sequence (FIESTA-C). The height and width of the saccule were measured on a coronal plane by two radiologists.
RESULTS: The saccule was dilated on the side of the schwannoma in 28% of the cases (p = 2.81 × 10- 5), with 15.7% of bilateral dilation. Saccular dilation was correlated to sensorineural hearing loss (OR 3.26, p = 0.02). There was also a significant correlation between saccular hydrops on the normal contralateral side of patients with VS and vertigo (p = 0.049), and between saccular hydrops on the side of the tumour and tinnitus (p = 0.006).
CONCLUSIONS: A third (29%) of VS are associated with a saccular dilation on the side of the tumour, which is an MR sign of endolymphatic hydrops (bilateral in 15.7% of the cases) and it appears related to sensorineural hearing loss and tinnitus, as well as vertigo if a contralateral dilation is present. This opens new therapeutic potentialities with the use of anti-vertiginous drugs, which could have a beneficial effect on the clinical symptoms.

We report the case of a 54-year-old woman who presented for evaluation of deterioration in her voice and swallowing function, which had begun after she had undergone a medialization laryngoplasty for unilateral vocal fold paralysis. Findings on examination and imaging revealed that a mass had developed adjacent to the Silastic implant that had been placed during the laryngoplasty. The superior extent of the implant reached above the laryngeal ventricle. Endolaryngeal surgical resection of the mass was accomplished without the need to remove the implant. Pathologic analysis identified the mass as a laryngeal saccular cyst. Although laryngeal saccular cysts are uncommon, medialization laryngoplasties are not. This case represents a rare complication of medialization laryngoplasty in which an implant compressed the laryngeal saccule and led to formation of the cyst.

OBJECTIVE: This is the first report, to our knowledge, of a child with torticollis due to saccular dysfunction.
METHODS: An 18-month-old infant with torticollis was referred for postural imbalance and observed rotary nystagmus. The infant had undergone physical therapy treatment of left torticollis for nearly 15 months. Cervical vestibular evoked myogenic potentials (cVEMPs) were recorded to assess saccular function and caloric stimulation and positional and rotational testing were performed to evaluate other vestibular receptors. The child demonstrated abnormal cVEMP findings, with a low-amplitude response on the left, which indicated left-sided saccular dysfunction. The patient\'s rotary-torsional nystagmus suggested positional vertigo secondary to abnormal saccular function.
CONCLUSIONS: This case highlights that saccular dysfunction should be considered when timely resolution of congenital torticollis is not obtained with physical therapy. Early detection of abnormal saccular function in infants and young children with CPT is necessary to ensure appropriate intervention. Further study needs to be done to confirm our findings.

Sound-induced vestibular evoked myogenic potentials (VEMP) on the surface of the sternocleidomastoid muscle constitute the basis of the saccular function test. In order to assess the saccular function, VEMP test was performed in a patient having unilateral Meniere\'s disease with profound sensorineural hearing loss in the contralateral ear. The saccular response was absent on the affected side with a normal caloric response. However, VEMP response was normal in the contralateral ear with profound sensorineural hearing loss and canal paresis. There may be saccular dysfunction in Meniere\'s disease.