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Single institution experience of cloacal malformation

      Highlights

      • Limited long-term outcome data following surgical correction of cloaca malformation in the published literature. The most recent systematic review of functional outcomes was published in 2013.
      • We report the long term renal and colorectal outcomes of twenty-one cloaca malformation patients managed at a single institution in the UK.
      • We emphasise the principle long term issues are preservation of renal function.

      Abstract

      Introduction

      The aim of this study is to report on the outcomes of patients born with cloacal malformation, managed at a single institution more than the last 28 years. The focus of this study is the long term renal and colorectal outcomes.

      Methods

      Patients were identified from the departmental database from 1994 to 2021. The medical records and operative notes were retrospectively reviewed.

      Results

      Twenty-one patients fulfilled the inclusion criteria. Eleven long common channel (LCC) and ten short common channel (SCC) cloacae patients were identified. Median age at the time of primary reconstruction was 11 months in both groups. In the LCC group, seven (63.6%) patients underwent a Total Urogenital Mobilisation (TUM), and 4 (36.4%) required a vaginal replacement. 6/11 (54.5%) of patients required drainage of a hydrocolpos. In the SCC group, four patients required a TUM, two patients underwent mobilisation of the rectum and vagina alone, and three underwent rectal mobilisation alone. Two patients have required renal transplant for congenital renal dysplasia, and two have developed chronic renal failure associated with the sequalae of vesicoureteric reflux. Eleven (52.3%) of the patients manage their bowels with an antegrade continent enema (ACE), and two of the LCC cloaca are defunctioned with a colostomy. Clean intermittent catheterisation is performed by 12 (57%) of the patients, either per urethra or via a Mitrofanoff channel.

      Conclusion

      The urinary and faecal continence are the main challenges in the management of cloaca patients. Many require surgical intervention to achieve social continence.

      Level of evidence

      Level IV.

      Keywords

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      References

        • Peña A.
        • Bischoff A.
        • Breech L.
        • Louden E.
        • Levitt M.A.
        Posterior cloaca-further experience and guidelines for the treatment of an unusual anorectal malformation.
        J Pediatr Surg. Jun 2010; 45: 1234-1240
        • Rintala R.J.
        Congenital cloaca: long-term follow-up results with emphasis on outcomes beyond childhood.
        Semin Pediatr Surg. Apr 1, 2016; 25: 112-116
        • deVries P.A.
        • Peña A.
        Posterior sagittal anorectoplasty.
        J Pediatr Surg. Oct 1982; 17: 638-643
        • Peña A.
        Total urogenital mobilization–an easier way to repair cloacas.
        J Pediatr Surg. Feb 1997; 32 (discussion 267-8): 263-267
        • Peña A.
        • Levitt M.
        Surgical management of cloacal malformations.
        Semin Neonatol. Jun 2003; 8: 249-257
        • Levitt M.A.
        • Peña A.
        Cloacal malformations: lessons learned from 490 cases.
        Semin Pediatr Surg. May 2010; 19: 128-138
        • Lane V.A.
        • Levitt M.A.
        Clinical quiz: reconstructive Strategies in a 5-month-old infant with a Cloaca.
        Eur J Pediatr Surg Rep. Jan 2020; 8: e45-e47
        • Wood R.J.
        • Reck-Burneo C.A.
        • Levitt M.A.
        Cloacal malformations: technical aspects of the reconstruction and factors which predict surgical complexity.
        Front Pediatr. 2019; 7: 240
        • Skerritt C.
        • Wood R.J.
        • Jayanthi V.R.
        • Levitt M.A.
        • Ching C.B.
        • DaJusta D.G.
        • et al.
        Does a standardized operative approach in cloacal reconstruction allow for preservation of a patent urethra?.
        J Pediatr Surg. Dec 2021; 56: 2295-2298
        • Wood R.J.
        • Reck-Burneo C.A.
        • Dajusta D.
        • Ching C.
        • Jayanthi R.
        • Bates D.G.
        • et al.
        Cloaca reconstruction: a new algorithm which considers the role of urethral length in determining surgical planning.
        J Pediatr Surg. Oct 12, 2017;
        • Halleran D.R.
        • Thompson B.
        • Fuchs M.
        • Vilanova-Sanchez A.
        • Rentea R.M.
        • Bates D.G.
        • et al.
        Urethral length in female infants and its relevance in the repair of cloaca.
        J Pediatr Surg. Feb 2019; 54: 303-306
        • Vilanova-Sanchez A.
        • Halleran D.R.
        • Reck C.A.
        • McCracken K.
        • Hewitt G.
        • Gasior A.C.
        • et al.
        Factors predicting the need for vaginal replacement at the time of primary reconstruction of a cloacal malformation.
        J Pediatr Surg. Jan 2020; 55: 71-74
      1. Bischoff A. The surgical treatment of cloaca. Semin Pediatr Surg. 2016 Apr;25(2):102–7.

        • Versteegh H.P.
        • Sloots C.E.J.
        • de Jong J.R.
        • Sleeboom C.
        • Rassouli R.
        • van Heurn L.W.E.
        • et al.
        Early versus late reconstruction of cloacal malformations: the effects on postoperative complications and long-term colorectal outcome.
        J Pediatr Surg. 2014; 49: 556-559
        • Versteegh H.P.
        • van Rooij I.A.L.M.
        • Levitt M.A.
        • Sloots C.E.J.
        • Wijnen R.M.H.
        • de Blaauw I.
        Long-term follow-up of functional outcome in patients with a cloacal malformation: a systematic review.
        J Pediatr Surg. 2013; 48: 2343-2350
        • Versteegh H.P.
        • Sloots C.E.J.
        • Wolffenbuttel K.P.
        • de Jong J.R.
        • Sleeboom C.
        • Feitz W.F.
        • et al.
        Urogenital function after cloacal reconstruction, two techniques evaluated.
        J Pediatr Urol. Dec 1, 2014; 10: 1160-1164
        • Ruiz J.
        • Tessi C.
        • Szklarz T.
        • Vazquez M.
        • Siffredi J.
        • Imizcoz F.L.
        • et al.
        Long-term urological assessment and management of cloaca patients: a single tertiary institution experience.
        J Pediatr Surg. May 2021; 56: 984-987
        • Blyth U.E.B.
        • Lall A.
        • Jaffray B.
        • Choudhary M.
        Transition care from adolescence to adulthood: a 10-year service review of the gynecological implications for young women and girls born with cloacal anomalies.
        J Pediatr Adolesc Gynecol. Jun 2021; 34: 412-414
      2. Warne S.A., Wilcox D.T., Creighton S., Ransley P.G. Long-term gynecological outcome of patients with persistent cloaca. In: J Urol. Lippincott Williams and Wilkins; 2003. p. 1493–6.

        • Caldwell B.T.
        • Wilcox D.T.
        Long-term urological outcomes in cloacal anomalies.
        Semin Pediatr Surg. Apr 1, 2016; 25: 108-111
        • Davies M.C.
        • Creighton S.M.
        • Wilcox D.T.
        Long-term outcomes of anorectal malformations.
        Pediatr Surg Int. Aug 2004; 20: 567-572
        • Fernando M.A.
        • Creighton S.M.
        • Wood D.
        The long-term management and outcomes of cloacal anomalies.
        Pediatr Nephrol. May 2015; 30: 759-765
        • Warne S.A.
        • Wilcox D.T.
        • Ledermann S.E.
        • Ransley P.G.
        Renal outcome in patients with cloaca.
        J Urol. Jun 2002; 167 (discussion 2551): 2548-2551
        • Fernando M.A.
        • Creighton S.M.
        • Wood D.
        The long-term management and outcomes of cloacal anomalies.
        Pediatric Nephrol. May 13, 2015; 30: 759-765
        • Braga L.H.P.
        • Lorenzo A.J.
        • Dave S.
        • Del-Valle M.H.
        • Khoury A.E.
        • Pippi-Salle J.L.
        Long-term renal function and continence status in patients with cloacal malformation.
        Can Urol Assoc J. Nov 2007; 1: 371-376
        • Chalmers D.J.
        • Rove K.O.
        • Wiedel C.A.
        • Tong S.
        • Siparsky G.L.
        • Wilcox D.T.
        Clean intermittent catheterization as an initial management strategy provides for adequate preservation of renal function in newborns with persistent cloaca.
        J Pediatr Urol. Aug 2015; 11 (211.e1-4)
        • Levitt M.A.
        • Stein D.M.
        • Peña A.
        Gynecologic concerns in the treatment of teenagers with cloaca.
        J Pediatr Surg. 1998; 33: 188-193
        • Couchman A.
        • Creighton S.M.
        • Wood D.
        Adolescent and adult outcomes in women following childhood vaginal reconstruction for cloacal anomaly.
        J Urol. 2015; 193: 1819-1822
        • Vilanova-Sanchez A.
        • McCracken K.
        • Halleran D.R.
        • Wood R.J.
        • Reck-Burneo C.A.
        • Levitt M.A.
        • et al.
        Obstetrical outcomes in adult patients born with complex anorectal malformations and cloacal anomalies: a literature review.
        J Pediatr Adolesc Gynecol. 2019; 32: 7-14