V07-07: Diagnostic Laparoscopy in Cloacal Exstrophy

Video

INTRODUCTION

Cloacal exstrophy is a rare diagnosis that occurs in 1 out of 250,000 live births. There are often multiple anomalies associated with cloacal exstrophy and is also known as OEIS syndrome because of the features often found together: omphalocele, exstrophy of bladder and gastrointestinal tract, imperforate anus, and spinal abnormalities. The internal anatomy especially the reproductive organs of a cloacal exstrophy patient may be difficult to evaluate by physical exam and imaging modalities. Here we present two cases of cloacal exstrophy female patients undergoing diagnostic laparoscopy to clearly identify the complex anatomy prior to second stage reconstruction.

METHODS

A 5mm laparoscopic port with zero degree camera was placed at the level of planned umbilicoplasty in both patients. A laparoscopic grasper was used to help identify intra-abdominal structures.

RESULTS

In the first patient, we were able to identify bilateral ovaries, bilateral fallopian tubes, hemi-ureteri, and possible hemi-vaginal remnants. We also were able to appreciate dilated ureters prior to second stage reconstruction likely due to non-obstructed, non-refluxing megaureters. The second patient was thought to be a cloacal malformation patient with one perineal opening and a low set umbilicus later diagnosed as a covered cloacal exstrophy patient. Multiple exams under anesthesia, abdominal/pelvic MRI, cystogram, retrograde pyelograms, and mucous fistulograms were performed to identify anatomy. However, anatomy was still unclear. Diagnostic laparoscopy allowed us to identify similar anatomy including bilateral ovaries, bilateral fallopian tubes, hemi-ureteri, and possible hemi-vaginal remnants. We also identified the anatomy of the bladder halves, mucous fistula, and blind-ending hindgut connecting to the perineal opening. What was thought to be the clocacal exstrophy behind the ventral wall defect was simply an umbilical hernia.

CONCLUSION

To our knowledge, this is the first video describing laparoscopy in a cloacal exstrophy patient and a covered cloacal exstrophy patient which offers clear delineation of anatomy prior to complex reconstruction. It also helps identify reproductive organs which would otherwise not be identified. This allows us to properly counsel these patients and their families.

Funding: None