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CT scan depicting an inflammatory Tubo-ovarian mass situated between the bowel on the right and bladder which has been deviated on the left
Source publication
Purpose
We assessed the surgical outcome of cloacal malformation (CM) with emphasis on need and timing of vaginal replacement.
Methods
An ambispective study of CM was carried out including prospective cases from April 2014 to December 2017 and retrospective cases that came for routine follow-up. Early vaginal replacement was defined as that done a...
Citations
... Complications associated with colon vaginoplasty include introital stenosis, neovaginal prolapse, wound dehiscence, hematoma, and infection [4,5]. Ulcerative and diversion colitis can occur and typically presents with bloody vaginal discharge [6,7]. ...
Vaginoplasty with colon is a common technique for vaginal replacement in patients with cloaca. Malignancy in the neovagina is a rare outcome and typically presents decades after reconstruction. We present a case of an adolescent female with history of cloaca, ulcerative colitis, and high-grade dysplasia of the sigmoid neovagina.
... Cloacal malformation (CM) is an extremely rare disease with an incidence of approximately 0.002%, [1][2][3][4][5][6][7] which represents the most severe classification of urogenital and anorectal deformity. [6] The CM patients are characterized by the convergence of the urinary, vagina, rectum, and tract into a single shared channel that is open at the urethral meatus. ...
... [13] CM is commonly found in female newborns. [2,3,5,6,11,13,14,17] As far as we know, only 2 male neonates have been reported in the literature. [4,8] Depending on the different stages of development stop, it will lead to various spectrum malformations, ranging from urogenital sinus to cloacal dysgenesis. ...
... With appropriate management, the patients could achieve an excellent anatomical repair. [7] Sharma and Gupta [2] reported that the initial diversion surgery in CM was a transverse colostomy. Further treatment depends on the length of the common channel, which needs a comprehensive evaluation before considering pull through. ...
Rationale:
Cloacal malformation (CM) is a serious type of anorectal and urogenital tract malformation. However, prenatal ultrasound (US) detection of CM is challenging. In this paper, we reported a rare case of CM prenatally diagnosed by US and magnetic resonance imaging (MRI), as well as reviewed the prenatal US and MRI characteristics of CM in the literature.
Patient concerns:
A 30-year-old pregnant woman complained of cystic mass in the fetal abdomen detected by prenatal US.
Diagnosis:
Fetus CM.
Interventions:
The fetus was diagnosed as fetal CM by US and MRI, then the pregnant woman received a drug-induced labor treatment. After the neonate was delivered, the measurement was performed on the weight, length, head circumference, abdomen circumference, and bilateral thigh circumference.
Outcomes:
A female dead neonate was delivered from the vagina of the gravida, showing congenital anus absence. Prenatal ultrasound demonstrated right kidney duplication, hydronephrosis, and right ureteral dilatation. Meanwhile, prenatal MRI showed a cystic cavity, double collecting systems of right kidney, right ureteral dilatation, and right rectum dilatation. In addition, general parameters are as follows: weight: 2280 g; length: 39 cm; head circumference: 26.3 cm; abdomen circumference: 31 cm; right thigh circumference: 17 cm, and left thigh circumference: 18 cm.
Lessons:
US combined with MRI can not only provide reliable evidence for fetal CM in the third trimester but also offer crucial information to the pregnant women to establish clinic treatment programs as early as possible.
Anorectal malformations (ARM) and Hirschsprung disease (HD) are managed with placement of normal intestine within the anal sphincter complex. Long-term complications specific to ARM include fistula remnants, recurrence, urinary reflux with associated chronic renal insufficiency, sexual dysfunction, and fertility difficulties. Complications specific to HD include enterocolitis, persistent or acquired aganglionosis, and internal sphincter achalasia. ARM and HD patients are both at risk of stricture, bowel dysfunction and incontinence, which can have a severe impact on quality of life. Bowel management strategies should be tailored to the patient's specific category of bowel dysfunction.
People with cloacal malformation and 46,XX cloacal exstrophy are at risk of developing Müllerian outflow tract obstruction (OTO). Management of OTO requires expertise of many medical and surgical specialties. The primary presenting symptom associated with OTO is cyclical and later continuous pain and can be initially quelled with hormonal suppression as a temporizing measure to allow for patient maturation. The decision for timing and method of definitive treatment to establish a patent outflow tract that can also be used for penetrative sexual activity and potential fertility is a complicated one and incredibly variable based on patient age alone. To understand the management approach to OTO, we put forth five phases with associated recommendations: (1) caregiver and patient education and evaluation before obstruction; (2) presentation, diagnosis, and symptom temporization; (3) readiness assessment; (4) peri-procedural management; (5) long-term surveillance. This review will emphasize the importance of interdisciplinary team management of the complex shared medical, surgical, and psychological decision making required to successfully guide developing patients with outflow obstruction secondary to cloacal malformations and cloacal exstrophy through adolescence.
