The Mullerian Black Box: Predicting and defining Mullerian anatomy in patients with cloacal abnormalities and the need for longitudinal assessment

Pradhan, Shashwati; Vilanova-Sánchez, Alejandra; McCracken, Katherine; Reck, Carlos A.; Halleran, Devin R.; Wood, Richard J.; Levitt, Marc A.; Hewitt, Geri

doi:10.1016/j.jpedsurg.2018.05.009

Cited by 21 publications

(18 citation statements)

References 14 publications

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“…Ultrasound can provide a gross assessment of the obstructive characteristics of the cloaca and determine what treatment options will be needed. 5 Vaginostomy (via image-guided drain placement, or surgically placed vaginostomy) or suprapubic catheters for the drainage of the bladder are sometimes required and can also be placed either with cystoscopic guidance or image guidance in interventional radiology. 1 Magnetic resonance imaging (MRI) can also be beneficial for patients with cloaca as it can provide better detailed information about the genitourinary system and also the presence or absence of Müllerian structures.…”

Section: Noninvasive Imagingmentioning

confidence: 99%

“…6 Patients with cloaca also tend to have hemivaginas, however, one or both of these may be atretic. 5 Characterization of these structures may be possible with MRI and can be correlated with the follow-up cloacagram. Typically MRI is obtained, if deemed necessary, separately from the cloacagram and is usually most valuable preoperatively, if there are unanswered questions from endoscopic and cloacagram imaging modalities about the anatomy.…”

Section: Noninvasive Imagingmentioning

confidence: 99%

“…A) On this lateral view of the 3D reconstructed image, the common channel (4) is more clearly delineated as the confluence of the rectum and the urethra(5) as it opens at the perineum. The bladder (1), hemi-vaginas (2), and distal colon/rectum(3)are again noted.…”

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confidence: 99%

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Complex Cloacal Malformations: The Application and Importance of Cloacagrams

2019

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Cloaca is a rare complex anorectal malformation in female patients where the urinary, gynecologic, and gastrointestinal tracts empty through a single pathway, known as the common channel. Previously, cloacas were measured through endoscopic evaluation alone. In recent years, radiographic imaging of the cloaca with a 3D cloacagram, has been a valuable tool for the characterization of the specific anatomical variables in each cloaca, and provides additional information that is beneficial for the preoperative surgical planning. This article reviews the embryology of cloaca, as well as the classification, noninvasive imaging, cloacagram protocol, and surgical intervention.

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Section: Noninvasive Imagingmentioning

confidence: 99%

Section: Noninvasive Imagingmentioning

confidence: 99%

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Complex Cloacal Malformations: The Application and Importance of Cloacagrams

2019

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“…To finish, the development of the urinary system is closely related to the genital tract and anomalies of these organs are often coexisting. Less commonly, MDAs may coexist with developmental anomalies of the distal gastrointestinal tract particularly cloaca anomaly [17]. A retrospective study of female patients treated for imperforate anus revealed that primary vaginal anomalies occurred in 22 of 72 (32%) patients assessed, and uterine anomalies occurred in 18 of 51 (35%) patients assessed.…”

Section: Discussionmentioning

confidence: 99%

Unicornuate uterus with a rudimentary non-communicating cavitary horn in association with VACTERL association: case report

et al. 2019

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Background The unicornuate uterus is caused by abnormal or failed development of one Müllerian duct. Unicornuate uteri with functioning non-communicating rudimentary horns are susceptible to many gynaecologic and obstetric complications such as hematometra, endometriosis and ectopic pregnancy and thus surgical resection is usually recommended.. Case presentation We report a rare case of a unicornuate right uterus with rudimentary non-communicating (functional) cavitary left horn (class U4a) in a 17-year-old girl who was diagnosed with VACTERL association. She was presented to our centre with 3 years history of secondary sever dysmenorrhea. Pelvic magnetic resonance imaging revealed a normal uterus on the right side, a 7 × 8 cm left endometrioma, a tortuous dilated fluid-filled structure in the left hemipelvis, mostly represented left-sided hematosalpinx, and a well-defined lesion with thick enhancing wall in the left hemipelvis measuring 6.7 × 5.7 × 5.6 cm with a similar enhancement to the uterus in the right. She underwent laparotomy that showed a right unicornuate uterus with a normal cervix and a rudimentary non-communicating distended left horn. In addition, there was a left endometrioma and left hematosalpinx. Resection of the left communicating horn, left salpingectomy and left ovarian cystectomy were performed. The right tube and both ovaries were preserved. At 9-months follow up, the patient had a regular period and the pain subsided completely. Conclusion We report yet the second case of VACTERL association and unicornuate uterus with non-communicating functional rudimentary horn, in hope of expanding the knowledge of a rare occurrence. This case also highlights the importance of considering the diagnosis of Müllerian duct anomalies in patients with a history of other anomalies, and/or history of early-age secondary dysmenorrhea.

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“…The presence of a didelphys configuration is the most definitive from a diagnostic point of view, the presence of a single cervix could mean either normal uterine anatomy or perhaps a bicornuate uterus which may not be obvious on imaging. Multi-modal imaging and longitudinal follow up is required, especially around puberty, to fully define the uterine anatomy in many cases (14). In addition, the location of the rectal fistula can be assessed during endoscopy, however, the location is only part of the story.…”

Section: Factors In the Initial Assessment Which Affect Treatmentmentioning

confidence: 99%

Cloacal Malformations: Technical Aspects of the Reconstruction and Factors Which Predict Surgical Complexity

2019

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Cloacal malformations are rare anomalies which occur in one in 50,000 live births. Anatomically these anomalies are defined by the presence of a single perineal orifice. There is however a substantial range in their complexity. Defining these differences is key to surgical planning and timely referral of selected cases to centers with the capabilities to manage the most challenging cases. Traditionally the common channel length as measured during cysto-vaginoscopy has been used to differentiate between patients that can be repaired with a reproducible operation and those requiring a more complex repair. The quality and range of imaging available has advanced and thus a more detailed anatomic picture is now possible to help with pre-operative planning. Cross sectional imaging with 3D reconstruction has enhanced the understanding of the anatomic variations in these patients. In addition, the sacral ratio, previously thought to only have an influence on long term continence predictions, has been shown to not only forecast the presence of urological anomalies, but also the complexity of the malformation. In principle, cloacal malformations have two major components to the reconstruction. First, the rectum needs to be separated from the urogenital tract and second, the urogenital sinus needs to be managed to create a urethral orifice and vaginal introitus. The length of the urethra has been shown to be vital in deciding between the two main surgical maneuvers; a total urogenital mobilization (TUM) and a urogenital separation. The technical demands of a urogenital separation are significant and discussed here in detail. The need for vaginal replacement adds further complexity to the care of these patients and has also been shown to be related to the length of the urethra. Predicting complexity in an accurate and non-invasive way will facilitate the care of the most complex cloacal malformations and improve outcomes.

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The Mullerian Black Box: Predicting and defining Mullerian anatomy in patients with cloacal abnormalities and the need for longitudinal assessment

Cited by 21 publications

References 14 publications

Complex Cloacal Malformations: The Application and Importance of Cloacagrams

Complex Cloacal Malformations: The Application and Importance of Cloacagrams

Unicornuate uterus with a rudimentary non-communicating cavitary horn in association with VACTERL association: case report

Cloacal Malformations: Technical Aspects of the Reconstruction and Factors Which Predict Surgical Complexity

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