Cloacal malformation: what it is and how it is treated
A cloacal malformation is a rare congenital condition in girls. The rectum, vagina and urinary tract do not open separately to the outside but join in one common channel with a single visible opening. Treatment is carefully planned in several stages and depends on the child's individual anatomy.
What is a cloacal malformation?
During early fetal development, the bowel, vagina and urinary tract normally separate. In a cloacal malformation, they retain a common outlet. There is no anus, and only one opening is visible at the perineum instead of three separate openings. The length of the common channel and the internal anatomy vary considerably.
The term "persistent cloaca" is also widely used. A cloacal malformation is not the same as cloacal exstrophy, in which parts of the bladder and bowel are exposed at the abdominal wall.
Signs, symptoms and diagnosis after birth
When a cloacal malformation is suspected, the team promptly checks whether urine and stool can pass safely. Ultrasound is used to examine the kidneys, bladder and pelvic organs. The spine, spinal cord, heart and other organs are also assessed for associated congenital conditions.
Initial treatment aims to stabilize the baby and relieve any obstruction. A temporary colostomy is usually created. If fluid has collected in the vagina or uterus, drainage may also be needed. If necessary, urinary drainage is also established.
How is surgery planned?
The exact anatomy must be understood before reconstruction. The assessment usually includes ultrasound, endoscopy of the urinary tract and vagina, and, depending on the findings, contrast studies or further imaging. Important details include the length of the common channel, the length and function of the urethra, and the position of the rectum and vagina.
Specialists in pediatric colorectal surgery, pediatric urology, pediatric gynecology, radiology and other relevant fields review these findings together. Based on this assessment, they determine the timing and surgical approach.
Experience with cloacal malformations
According to his own clinical records, PD Dr. Reck-Burneo treats approximately five to ten patients with cloacal malformation each year. In his professional assessment, this makes him one of the most experienced surgeons in Europe in this field.
How is a cloacal malformation treated?
The aim of reconstructive surgery is to separate the rectum, vagina and urinary tract functionally and create individual outlets. The rectum is positioned in the center of the sphincter muscles. Reconstruction of the vagina and urinary tract depends on the child's anatomy.
When the anatomy is suitable, reconstruction may be performed through a posterior sagittal approach. A longer or more complex common channel may also require laparoscopic or open abdominal surgery. A frequently used term for comprehensive reconstruction is PSARVUP, posterior sagittal anorectal vaginal urethroplasty. Once healing is complete, a previously created colostomy can be closed.
There is no single operation plan for every child. The number and sequence of procedures are decided individually and discussed with the family.
Long-term follow-up
Care does not end after reconstruction. Bowel control, bladder emptying, kidney function and gynecologic development are monitored over time. Some children need structured bowel management, medication, enemas or support with bladder emptying. Pediatric gynecology follow-up is particularly important before and during puberty.
Future bowel and bladder control can only be estimated from the individual anatomy, associated conditions and clinical course. Long-term multidisciplinary care into adulthood helps identify and treat problems early.
Frequently asked questions
Is a cloacal malformation a type of imperforate anus?
It belongs to the spectrum of anorectal malformations but also involves the vagina and urinary tract. Its treatment is therefore more complex than that of many other forms of imperforate anus.
Can a cloacal malformation be detected before birth?
Prenatal ultrasound sometimes shows unusual fluid collections or changes in the pelvis. However, the diagnosis is often made after birth by examining the perineum.
Can a cloacal malformation be treated?
Yes. Treatment is surgical and usually takes place in several stages. The exact plan depends on the child's anatomy and associated congenital conditions.
Will my child have bowel and bladder control?
This cannot be predicted in general terms. The outlook depends on factors including the common channel, urinary tract, sacrum and spinal cord. Continence programs can support bowel and bladder control when needed.
Request an appointment or second opinion
- Phone: +49 3381 411270
- Email: [email protected]
Please bring or send available ultrasound images, medical reports, operative reports and other records. More information is available on the second opinion page.
Sources and further information
- German Society of Pediatric Surgery guideline on anorectal malformations: AWMF guideline 006-002
- Nationwide Children's Hospital: Cloacal Malformation
- NCBI Bookshelf clinical review: Cloacal Malformations
- SoMA e.V., support organization for people with anorectal malformations: www.soma-ev.de
Publications
2025
- Anorektale Fehlbildungen
- Complications After Surgery for Anorectal Malformations: An ARM-Net Consortium Registry Study
- Excision of a rectal duplication cyst in proximity to the glomus coccygeum - an unusual cause of intraoperative bleeding: a case report
- Late presentation of a Type IV sacrococcygeal teratoma in a 16-year-old female: a case report
- Pediatric pseudo-Meigs' syndrome secondary to a Sertoli-Leydig cell tumor with elevated cancer antigen 125 and alpha-fetoprotein: a case report
- Sphincter Preserving Anorectoplasty (SPARP) With Anterior Approach: A Revisited and Modified Technique in the Treatment of Anorectal Malformations
2023
2022
- Clinical Differentiation between a Normal Anus, Anterior Anus, Congenital Anal Stenosis, and Perineal Fistula: Definitions and Consequences - The ARM-Net Consortium Consensus
- Cysto-Vaginoscopy of a 3D-Printed Cloaca Model: A Step toward Personalized Noninvasive Preoperative Assessment in Patients with Complex Anorectal Malformations
- The Use of an Inanimate Simulation Model for the Correction of an Anorectal Malformation in the Training of Colorectal Pediatric Surgery
- Validation of a Newly Developed Competency Assessment Tool for the Posterior Sagittal Anorectoplasty
2021
- A technique to reconstruct the anal sphincters following iatrogenic stretching related to a pull-through for Hirschsprung disease
- Are routine postoperative dilations necessary after primary posterior sagittal anorectoplasty? A randomized controlled trial
- Assessment of sacral ratio in patients with anorectal malformations: can magnetic resonance imaging replace conventional radiograph?
- Laparoscopic Approach in Anorectal Malformations
- Laparoscopic Approach to Hirschsprung Disease
- One-year impact of a bowel management program in treating fecal incontinence in patients with anorectal malformations
- Quality outcomes for pediatric colorectal surgery treated during short-term international medical service trips at a dedicated site in Honduras
2020
- Factors predicting the need for vaginal replacement at the time of primary reconstruction of a cloacal malformation
- Joint Attention in a Laparoscopic Simulation-Based Training: A Pilot Study on Camera Work, Gaze Behavior, and Surgical Performance in Laparoscopic Surgery
- Organizing the care of a patient with a cloacal malformation: key steps and decision making for pre-, intra-, and post-operative repair
- Re: Cloaca Reconstruction: A New Algorithm Which Considers the Role of Urethral Length in Determining Surgical Planning
- Re: The Use of Rotational Fluoroscopy and 3-D Reconstruction in the Diagnosis and Surgical Planning for Complex Cloacal Malformations
- The Effect of Pediatric Colorectal Short-Term Medical Service Trips on Self-Reported Confidence in Patient Care in Volunteers in the Home Country
- Total Colonic Hirschsprung's Disease: The Hypermotility and Skin Rash Protocol
- Validation of a newly developed competency assessment tool for the Posterior Sagittal Anorectoplasty
2019
- Cloacal Malformations: Technical Aspects of the Reconstruction and Factors Which Predict Surgical Complexity
- Diagnosis and management of a remnant of the original fistula (ROOF) in males following surgery for anorectal malformations
- Obstetrical Outcomes in Adult Patients Born with Complex Anorectal Malformations and Cloacal Anomalies: A Literature Review
- The use of rotational fluoroscopy and 3-D reconstruction in the diagnosis and surgical planning for complex cloacal malformations
- What do pediatric surgeons think about sexual issues in dealing with patients with anorectal malformations? The ARM-Net consortium members' opinion
2018
- A descriptive model for a multidisciplinary unit for colorectal and pelvic malformations
- A structured bowel management program for patients with severe functional constipation can help decrease emergency department visits, hospital admissions, and healthcare costs
- Are Senna based laxatives safe when used as long term treatment for constipation in children?
- Assessment of the Heineke-Mikulicz anoplasty for skin level postoperative anal strictures and congenital anal stenosis
- Can sacral development as a marker for caudal regression help identify associated urologic anomalies in patients with anorectal malformation?
- Current concepts in the management of anorectal malformations
- Die häufigsten proktologischen Erkrankungen bei Kindern
- Erratum to “Cloaca reconstruction: a new algorithm which considers the role of urethral length in determining surgical planning”
- Health literacy and health-related quality of life in patients with anorectal malformations: a comparison between a charity hospital in Honduras and a tertiary care center in the United States
- Imaging in anorectal and cloacal malformations
- Impact on Patient Care of a Multidisciplinary Center Specializing in Colorectal and Pelvic Reconstruction
- Late Presentation of a Duodenal Obstruction in a Child with Failure to Thrive and Abdominal Pain
- Presacral masses and sacrococcygeal teratomas in patients with and without anorectal malformations: a single institution comparative study
- Reply to letter to the editor
- Surgical management of functional constipation: an intermediate report of a new approach using a laparoscopic sigmoid resection combined with Malone appendicostomy
- The Impact of Manuscript Learning vs. Video Learning on a Surgeon's Confidence in Performing a Difficult Procedure
- The Mullerian Black Box: predicting and defining Mullerian anatomy in patients with cloacal abnormalities and the need for longitudinal assessment
- Transcending Dimensions: a Comparative Analysis of Cloaca Imaging in Advancing the Surgeon's Understanding of Complex Anatomy
- Urinary Outcomes in Patients with Down's Syndrome and Hirschsprung's Disease
2017
- A Retrospective Cohort Study of Total Colonic Aganglionosis: Is the Appendix a Reliable Diagnostic Tool?
- Cloaca reconstruction: a new algorithm which considers the role of urethral length in determining surgical planning
- Does clinic visit education within a multidisciplinary center improve health literacy scores in caregivers of children with complex colorectal conditions?
- Gynecologic anatomic abnormalities following anorectal malformations repair
- Indications for Perineoplasty With Perineal Body Reconstruction for Gynecological Complications in Patients With Previously Repaired Anorectal Malformations
- Intraoperative and Radiologic Assessment of Mullerian and Ovarian Anatomy in Patients With Cloacal Anomalies - In What Proportion of Patients are we Sure? A Call for Longitudinal Multi-Disciplinary Assessment
- Pediatric Colorectal and Pelvic Surgery: Case Studies
- Perineal Fistula and Vaginal Agenesis Video Case Report
- Primary or Redo Posterior Sagittal Anorectoplasty without a Stoma: To Feed or Not to Feed?
- Sociodemographics and the impact of a colostomy to indigent families and children with colorectal disorders in Honduras
- Surgical Management of Functional Constipation: Preliminary Report of a New Approach Using a Laparoscopic Sigmoid Resection Combined with a Malone Appendicostomy
2016
- A standardized approach for the assessment and treatment of internationally adopted children with a previously repaired anorectal malformation (ARM)
- Anorectal Malformation, Rectoperineal Fistula with Vaginal Agenesis (operative video)
- Rectal atresia and anal stenosis: the difference in the operative technique for these two distinct congenital anorectal malformations
- Use of a Heineke-Mikulicz like stricturoplasty for intractable skin level anal strictures following anoplasty in children with anorectal malformations
2013
2012
- Esophageal replacement in children and its long-term consequences
- Interleukin 8 correlates with intestinal involvement in surgically treated infants with necrotizing enterocolitis
- MRI graduation of osseous reaction and drill hole consolidation after arthroscopic Bankart repair with PLLA anchors and the clinical relevance
2011
2010
2009
2008
2007
- The efficacy of cervical mediastinotomy in children
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