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| CORRESPONDENCE |
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| Year : 2021 | Volume
: 4
| Issue : 1 | Page : 40-41 |
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Singleton newborn with bilateral renal and ureteric agenesis, without features of potter sequence
WA Lasanthi Kumari Weerasooriya1, Shenal Thalgahagoda2, Dinesh Rangana1, Mathula Hettiarachci3
1 Teaching Hospital, Jaffna, Sri Lanka
2 Department of Paeditric, Faculty of Medicine, Teaching Hospital, University of Peradeniya, Kandy, Sri Lanka
3 Department of Surgery, Faculty of Medicine, University of Peradeniya; Sirimavo Bandaranaike Specialised Children Hospital, Kandy, Sri Lanka
| Date of Submission | 12-Sep-2020 |
| Date of Decision | 29-Jan-2021 |
| Date of Acceptance | 19-Mar-2021 |
| Date of Web Publication | 30-Jun-2021 |
Correspondence Address:
W A Lasanthi Kumari Weerasooriya
Teaching Hospital, Jaffna
Sri Lanka
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/AJPN.AJPN_30_20
How to cite this article:
Lasanthi Kumari Weerasooriya W A, Thalgahagoda S, Rangana D, Hettiarachci M. Singleton newborn with bilateral renal and ureteric agenesis, without features of potter sequence. Asian J Pediatr Nephrol 2021;4:40-1 |
How to cite this URL:
Lasanthi Kumari Weerasooriya W A, Thalgahagoda S, Rangana D, Hettiarachci M. Singleton newborn with bilateral renal and ureteric agenesis, without features of potter sequence. Asian J Pediatr Nephrol [serial online] 2021 [cited 2021 Dec 2];4:40-1. Available from: https://www.ajpn-online.org/text.asp?2021/4/1/40/320184 |
Sir
Bilateral renal agenesis is a rare condition that is associated with severe oligohydramnios, pulmonary hypoplasia, and facial compression or Potter sequence. In contrast, esophageal atresia is associated with polyhydramnios. We discuss a singleton neonate who had both these malformations but did not have Potter sequence or lung hypoplasia.
A boy born at term gestation and with low birth weight (1700 g) showed features of imperforate anus, tracheoesophageal fistula, esophageal atresia, and bilateral renal agenesis. There was no parental consanguinity. A prior pregnancy was associated with intrauterine growth retardation (IUGR), respiratory distress since birth, and early neonatal death; autopsy was not performed. The second pregnancy produced a healthy boy, and the third was a second trimester miscarriage.
Antenatally, gestational diabetes mellitus in the present pregnancy was managed with dietary changes. On antenatal ultrasonography at 27 weeks of gestation, fetal kidneys could not be identified; however, liquor volume was adequate. Cesarean section was performed at 37 weeks of gestation in view of moderate oligohydramnios, severe IUGR, and unfavorable obstetric history.
At birth, Apgar scores were satisfactory and there was no facial dysmorphism [Figure 1]a. The anus was imperforate and genitals appeared normal. Chest radiography indicated well-expanded lung fields. While gastric air bubble was present, the nasogastric tube coiled in the esophagus, indicating esophageal atresia with tracheoesophageal fistula [Figure 1]b. There was no urine output on urethral catheterization, and ultrasonography indicated an empty bladder and bilaterally absent kidneys. Absence of both the kidneys and ureters was confirmed on noncontrast computed tomography [Figure 1]c. In the presence of 4 of 7 criteria (vertebral anomalies, anorectal malformations, cardiovascular anomalies, tracheoesophageal fistula, esophageal atresia, renal and/or radial anomalies, and limb defects), a diagnosis of VACTERL association was made. | Figure 1: Images showing (a) facial appearance; (b) chest radiograph indicating bilateral lung expansion as well as coiling of the nasogastric tube in the esophagus; (c) computed tomography of the abdomen indicating the absence of kidneys
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Serum creatinine rose from a baseline value of 70–479 μmol/L on day 10. Following parental counselling regarding complications, prognosis, costs of care, and expected outcomes, repair of anomalies was not attempted and kidney replacement therapy was not initiated. The neonate succumbed on day 11 due to end-stage kidney disease. Autopsy confirmed the diagnosis of complete agenesis of bilateral kidneys and ureters, the presence of intact urinary bladder and well-developed lungs, and esophageal atresia, tracheoesophageal fistula, and imperforate anus.
Kidney development begins in the fourth week of gestation when the outgrowing ureteric bud of the mesonephric duct interacts with the metanephric mesenchyme, and urine is produced beginning in the 13th week.[1],[2] Renal agenesis refers to lack of formation of mature kidney from the renal pelvis, collecting ducts, and renal mesenchyme, while ureteric agenesis results from failure of the ureteric bud to mold into the ureter. During the first half of pregnancy, both fetal and maternal compartments contribute to the formation of amniotic fluid; the fetal urine becomes the chief source after 16 weeks of gestation.[3] Amniotic fluid volume is decided by the dynamic balance between amniotic fluid synthesis by the kidneys and its removal by the fetal gastrointestinal tract. Kidney agenesis results in oligohydramnios or anhydramnios, and subsequently, lung hypoplasia and Potter sequence. In contrast, gastrointestinal obstruction proximal to the ligament of Treitz reduces effective removal of amniotic fluid, leading to polyhydramnios. While case reports describe normal amniotic fluid volume and pulmonary function in monoamniotic twins discordant for bilateral renal agenesis,[4] few reports describe singleton pregnancy with renal agenesis and duodenal atresia presenting without pulmonary hypoplasia.[5] Amnioinfusion has been used for fetuses with bilateral renal agenesis and shows the lengthiest recorded survival in an infant with bilateral renal agenesis.[6] It is possible that the liquor volume and lung function in the index patient were preserved despite bilateral renal agenesis as a result of associated esophageal atresia. Since fetuses with this combination of anomalies may not have lung hypoplasia and Potter sequence and the newborn may be managed with ambulatory peritoneal dialysis until kidney transplantation, termination of pregnancy may not always be required in such circumstances.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Acknowledgments
We would like to thank the all members of the medical team who involved in management this patient.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | |  |
| 1. | Rehman S, Ahmed D. Embryology, Kidney, Bladder, and Ureter. In StatPearls 2019 Sep 21. StatPearls Publishing. Rehman S, Ahmed D. Embryology, kidney, bladder, and ureter. In: StatPearls. Treasure Island (FL): StatPearls Publishing; 2021. Available from: https://www.ncbi.nlm.nih.gov/books/NBK547747/. [Last accessed on 2020 Mar 19].  |
| 2. | Ludwig KS, Landmann L. Early development of the human mesonephros. Anat Embryol (Berl) 2005;209:439-47. |
| 3. | Abramovich DR. Fetal factors influencing the volume and composition of liquor amnii. J Obstet Gynaecol Br Commonw 1970;77:865-77. |
| 4. | Perez-Brayfield MR, Kirsch AJ, Smith EA. Monoamniotic twin discordant for bilateral renal agenesis with normal pulmonary function. Urology 2004;64:589. |
| 5. | George L, Manimtim W, Sharma J. A singleton infant with bilateral renal agenesis and normal pulmonary function. Case reports in pediatrics 2017;2017. |
| 6. | Whittaker N, Leonardi M. Five-month survival of neonate after serial amnioinfusions for fetal bilateral renal agenesis [18D]. Obstet Gynecol 2016;127:39S. |
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