Long-term urologic and gynecologic follow-up and the importance of collaboration for patients with anorectal malformations

Skerritt, Clare; DaJusta, Daniel; Fuchs, Molly; Pohl, Hans G.; Gómez-Lobo, Veronica; Hewitt, Geri

doi:10.1016/j.sempedsurg.2020.150987

Cited by 19 publications

(11 citation statements)

References 74 publications

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“…A patient with an anorectal anomaly seeking medical assistance and treatment for the first time in adulthood is a rare occurrence and we could not find any similar cases presenting with fourth degree perineal tear and secondary infertility. ARM include a number of congenital defects and various types of urinary and/or sexual malformations [7]. The recto-vestibular fistula in our patient is the most commonly observed anorectal defect in female pediatric patients with anorectal malformations while the presentation and complications of fourth degree perineal tear and secondary infertility are the rarest of the lesions.…”

Section: Discussionmentioning

confidence: 60%

“…In these patients, concomitant anomalies should be considered and the necessary tests should be performed. The management of such complex lesions needs attention of the multidisciplinary expertise and the other departments, including mainly pediatric surgery, gynecology, and urology, should be involved in the systemic evaluation of adult patients with ARM [7].…”

Section: Discussionmentioning

confidence: 99%

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Successful Resolution of Secondary Infertility following Treatment of Fourth-Degree Perineal Tear through Neglected Recto-Vestibular Fistula in an Adult Female with Anorectal Malformation

Gorasia¹,

Govani²,

Govani³

et al. 2022

MPOG

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Section: Discussionmentioning

confidence: 60%

Section: Discussionmentioning

confidence: 99%

Successful Resolution of Secondary Infertility following Treatment of Fourth-Degree Perineal Tear through Neglected Recto-Vestibular Fistula in an Adult Female with Anorectal Malformation

Gorasia¹,

Govani²,

Govani³

et al. 2022

MPOG

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“…Therefore, it is essential not to miss the point when physical activity in patients with ARM/ HD decreases compared to peers, especially in girls in order to initiate specialist care. Guidelines recommend a life-long follow-up even in asymptomatic patients with HD [27] and ARM [28]. However, many adolescents with ARM/ HD are lost to regular follow-up over time, particularly during the transition to adult care.…”

Section: Discussionmentioning

confidence: 99%

Physical self-concept and ability to swim in patients born with anorectal malformation and Hirschsprung’s disease: a case control study

2022

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Background Children with anorectal malformation (ARM) and Hirschsprung’s Disease (HD) live with permanent urinary and bowel symptoms, possibly impairing motor development in early childhood. Not being able to swim adds an unnecessary health risk. The aim of this study was to determine the ability to swim and physical self-concept in patients with ARM and HD. Methods We performed an anonymous survey among the members of the national patient organization SoMA e.V. (6 through 25 years). A control group was recruited from our department. Ability to swim, symptom load according to Rintala Score and physical self-concept were recorded using validated questionnaires. Patients were matched with controls according to gender and age. Mean scores and 95%-confidence intervals (95%-CI) were calculated, χ2-test and multiple linear regression models were used as appropriate. Results Totally, 83 match-control-pairs were included. Patients learned to swim at a similar age and rate (6.5 years, 95%-CI: 6.1–6.9, 74.7% swimmers) compared to controls (6.4 years, 95%-CI: 6.1–6.8, 79.5% swimmers, p = 0.46). VACTERL patients had a significantly lower swimmer rate (59.1%, p = 0.048). Swimmers had a significantly higher mean Rintala Score (12.5, 95%-CI: 11.6–13.2) compared to non-swimmers (10.4, 95%-CI: 8.1–12.1, p = 0,049). In prepubertal children (6 through 12 years), no difference in physical self-concept was shown compared to controls. Adolescents and young adults with ARM/HD, especially females, had a significantly lower mean score for the subscales of flexibility, speed, endurance and sports competence, independent of bowel symptom load according to Rintala Score. Conclusions Patients with ARM/HD have normal swimming skills and a normal physical self-concept in childhood that decreases with age compared to peers. In adolescence, parents and health care professionals should actively promote physical activity in ARM/HD patients.

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“…If the Mullerian structures are not inspected during colostomy or vaginostomy creation, they may be evaluated during definitive repair, or if any future abdominal surgery is needed (such as colostomy closure or laparoscopy for a Malone appendicostomy). Greater than 50% of cloaca patients have Mullerian anomalies, and require longitudinal follow-up with a gynecologist [ 30 , 44 , 45 ]. Forty percent of patients develop obstructive menses; therefore, gynecologic consultation and sonographic evaluation around puberty and thelarche are essential [ 44 , 45 ].…”

Section: Post-operative Course and Follow-upmentioning

confidence: 99%

“…Greater than 50% of cloaca patients have Mullerian anomalies, and require longitudinal follow-up with a gynecologist [ 30 , 44 , 45 ]. Forty percent of patients develop obstructive menses; therefore, gynecologic consultation and sonographic evaluation around puberty and thelarche are essential [ 44 , 45 ]. Establishing a relationship early with a reconstructive gynecologist with experience in patients with cloaca is optimal.…”

Section: Post-operative Course and Follow-upmentioning

confidence: 99%

Fetal and Newborn Management of Cloacal Malformations

et al. 2022

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Cloaca is a rare, complex malformation encompassing the genitourinary and anorectal tract of the female in which these tracts fail to separate in utero, resulting in a single perineal orifice. Prenatal sonography detects a few cases with findings such as renal and urinary tract malformations, intraluminal calcifications, dilated bowel, ambiguous genitalia, a cystic pelvic mass, or identification of other associated anomalies prompting further imaging. Multi-disciplinary collaboration between neonatology, pediatric surgery, urology, and gynecology is paramount to achieving safe outcomes. Perinatal evaluation and management may include treatment of cardiopulmonary and renal anomalies, administration of prophylactic antibiotics, ensuring egress of urine and evaluation of hydronephrosis, drainage of a hydrocolpos, and creation of a colostomy for stool diversion. Additional imaging of the spinal cord and sacrum are obtained to plan possible neurosurgical intervention as well as prognostication of future bladder and bowel control. Endoscopic evaluation and cloacagram, followed by primary reconstruction, are performed by a multidisciplinary team outside of the neonatal period. Long-term multidisciplinary follow-up is essential given the increased rates of renal disease, neuropathic bladder, tethered cord syndrome, and stooling issues. Patients and families will also require support through the functional and psychosocial changes in puberty, adolescence, and young adulthood.

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Long-term urologic and gynecologic follow-up and the importance of collaboration for patients with anorectal malformations

Cited by 19 publications

References 74 publications

Successful Resolution of Secondary Infertility following Treatment of Fourth-Degree Perineal Tear through Neglected Recto-Vestibular Fistula in an Adult Female with Anorectal Malformation

Successful Resolution of Secondary Infertility following Treatment of Fourth-Degree Perineal Tear through Neglected Recto-Vestibular Fistula in an Adult Female with Anorectal Malformation

Physical self-concept and ability to swim in patients born with anorectal malformation and Hirschsprung’s disease: a case control study

Fetal and Newborn Management of Cloacal Malformations

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