To describe the spectrum of congenital renal anomalies and emphasize the critical role of comprehensive autopsy examination in identifying CAKUT, especially of lower urinary tract malformations correlating with prenatal imaging methods. Retrospective analyses of CAKUT diagnosed at fetal autopsy were analyzed over a 7-y period and correlated with prenatal imaging findings. Among the 255 fetal autopsies, 45 cases were detected with CAKUT. Isolated (27%), syndromic CAKUT (51%), and CAKUT associated with other system anomalies (22%) were found. Hydronephrosis, followed by cystic renal diseases and agenesis were the common renal malformations. The gastrointestinal tract (GIT) was the commonest system associated with CAKUT. Among the syndromic CAKUT, the urorectal septum malformation (URSM) was the most frequent one, followed by VACTER-L, acrorenal syndrome, and OEIS complex. When correlating prenatal USG and autopsy findings, a significant change in final diagnosis was observed in 60% of cases. Extrarenal malformations and syndromic associations of CAKUT predominated over isolated ones. Detection of lower urinary tract anomalies in CAKUT is difficult through antenatal imaging methods if associated with oligohydramnios. In these circumstances, the perinatal autopsy has a significant role in arriving at the final diagnosis which guides the clinician in predicting the recurrence risk and the need for genetic workup.

[This retracts the article on p. 207 in vol. 30, PMID: 33013074.].

Introduction  Acrorenal syndrome is autosomal recessive inherited disorder commonly associated with congenital renal disorders and ipsilateral hand/foot anomalies. The hand and foot deformities corrections are challenging to achieve a good functional and cosmetic result. We described a case of acrorenal syndrome with suppressed radial elements and absent thumb in whom we were able to reconstruct the hand. Case Report  A 5-year-old girl presented to us with right renal agenesis and right hand/foot deformity. The hand showed a cleft hand with central deficiency, index finger hypoplastic, and syndactylyzed to middle finger, absent thumb. The hand was nonfunctional because of absent thumb. To improve the functions, it was decided to proceed with thumb reconstruction. The middle finger was pollicized to regain tripod grip and thereby the functions was enhanced. Discussion  The cleft hand belongs to "failure of finger ray induction group" in classification by "International Federation of Societies for Surgery of the Hand (IFSSH)." Absence of thumb and first web space makes it a strong indication for surgical reconstruction. In our case, thumb was addressed by pollicization of middle finger and we were able to provide a good tripod grip.

A primiparous woman in her late 30s at 28+1 weeks' gestation presented with a 3-day history of abdominal pain, loss of appetite, nausea and vomiting and was diagnosed with starvation ketoacidosis. A routine admission swab returned positive for COVID-19. She had been diagnosed with acrorenal syndrome from birth. Three days post admission, she deteriorated rapidly into respiratory failure requiring intubation and ventilation. She was treated with dexamethasone, prophylactic enoxaparin, a course of piperacillin/tazobactam followed by meropenem and fluconazole and 8 cycles of proning. An emergency caesarean section was performed on day 12 of hospital admission at 29+5 weeks' gestation to improve maternal oxygenation and ventilation. The baby had deformities consistent with acrorenal syndrome but no evidence of COVID-19. She spent 23 days in the intensive care unit. Our case describes an unusual presentation of COVID-19, the challenges in managing critically ill pregnant patients along with a rare background history of acrorenal syndrome.