|
 
 |
| ORIGINAL ARTICLE |
|
| Year : 2021 | Volume
: 10
| Issue : 2 | Page : 88-94 |
|
Outcome of nonurinary surgical malformations predicted by fetal abdominal signs on prenatal ultrasound
Amit Gupta1, Leonard J Ezung1, Manisha Kumar2, Vipan Kumar1, Subhasis Roy Choudhury1, Partap S Yadav1
1 Department of Pediatric Surgery, Kalawati Saran Children's Hospital, Lady Hardinge Medical College, New Delhi, India
2 Department of Obstetrics and Gynecology, Lady Hardinge Medical College, New Delhi, India
| Date of Submission | 19-Nov-2020 |
| Date of Acceptance | 13-Jan-2021 |
| Date of Web Publication | 15-May-2021 |
Correspondence Address:
Amit Gupta
Room No. 330, 3rd Floor, Kalawati Saran Children's Hospital, Bangla Sahib Road, New Delhi - 110 001
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/jcn.JCN_185_20
Aim: The aim of our study was to study the outcome of “nonurinary” surgical malformations predicted by fetal abdominal signs on prenatal ultrasound (US). Methods: This prospective observational study was done over a 3-year period. Results: Out of 66 cases, 15 different malformations were detected. The accuracy of prenatal US was 83.3%. There were four still births, two medical termination of pregnancy, and sixty live births (91%). Postnatal surgery was necessary in 35 neonates (62.5%) with postoperative survival of 71.4%. Sixteen neonates (26.7%), 7 with normal postnatal US and 9 asymptomatic lesions, were managed conservatively. Overall 1-year survival rate of fetuses was 62.1%. The most common malformations confirmed postnatally were duodenal atresia (n = 14, 23.3%), followed by gastroschisis (n = 9; 15%), esophageal atresia (n = 8; 13.3%), meconium peritonitis (n = 4; 6.7%), and ovarian cyst (n = 4; 6.7%). Prematurity (n = 34; 56.7%) and low birth weight (n = 44; 66.7%) had no significant effect on survival due to in utero transfer to tertiary care. Salient factors adversely affecting the survival were: (1) type of anomaly-omphalocele major and fetal ascites (FA) had no survivors, (2) postoperative complications, and (3) associated cardiac anomalies. It was also notable that, among fetal signs of meconium peritonitis, isolated intra-abdominal calcification had a good prognosis with 75% survival, whereas FA had no survivors. Conclusions: Results of this study will be helpful in realistic prognostication and postnatal management of these anomalies in the appropriate hospital setting.
Keywords: Congenital, fetal, prenatal, surgical malformations
How to cite this article:
Gupta A, Ezung LJ, Kumar M, Kumar V, Choudhury SR, Yadav PS. Outcome of nonurinary surgical malformations predicted by fetal abdominal signs on prenatal ultrasound. J Clin Neonatol 2021;10:88-94 |
How to cite this URL:
Gupta A, Ezung LJ, Kumar M, Kumar V, Choudhury SR, Yadav PS. Outcome of nonurinary surgical malformations predicted by fetal abdominal signs on prenatal ultrasound. J Clin Neonatol [serial online] 2021 [cited 2023 Mar 24];10:88-94. Available from: https://www.jcnonweb.com/text.asp?2021/10/2/88/316179 |
| Introduction | |  |
Prenatal surgical malformations detected by ultrasound (US) signs in fetal abdomen may be broadly classified as renal/genitourinary, gastrointestinal (GI), abdominal wall defects, and others which are predominantly simple/complex cystic lesions. Digestive system anomalies comprise more than 7% of all major congenital malformations (MCM).[1],[2] It's incidence has been reported from the most common to the fourth most common MCM.[3],[4],[5] As per the EUROCAT registry combined, the prevalence of digestive system anomalies and abdominal wall defects is the third most common anomaly after the urinary and nervous system.[5] Prevalence in India for GI anomalies is reported as 50.19/10,000 births.[4],[6] Most of these malformations are isolated with 96.99% survival reported until 1 year of age.[7] Among intra-abdominal cystic lesions, ovarian cyst is the most common followed by cysts related to digestive system, with an overall excellent outcome.[8] Expectant parents should be apprised of favorable outcome associated with these malformations.[9],[10] Considering the significant burden of these “nonurinary” surgical malformations predicted by US signs in fetal abdomen, we designed a prospective study to assess their outcome at our tertiary care center with limited resources.
| Methods | | |
This was a prospective observational study done at our institute from November 1, 2016, to October 31, 2019, after obtaining ethical clearance. We included all the fetuses with “nonurinary” surgical malformations predicted by prenatal US findings in fetal abdomen. Other associated major malformations were also noted. We included esophageal atresia, as a small stomach bubble in the fetal abdomen associated with polyhydramnios predicts this anomaly. We excluded congenital diaphragmatic hernia because of its abdominothoracic nature. The expectant parents were counseled by a team of obstetrician and pediatric surgeon regarding future clinical course and management. The option of medical termination of pregnancy (MTP) was discussed if there were multiple major anomalies detected before 20 weeks of gestation. Postnatally, patients were managed by a preinformed team of pediatricians and surgeons, prepared for anticipated problems specific to the suspected anomaly. All the babies were followed up for at least 1 year. Primary outcome was percentage of pregnancies continued and delivered live birth and types of malformations with accuracy of prenatal diagnosis. Secondary outcome was postnatal survival rate of newborns with these malformations.
| Results | | |
Sixty-six fetuses were registered, and prenatal US findings could be broadly categorized as GI tract obstructions, abdominal wall defects, intra-abdominal cysts, and solid organ lesions. [Table 1] describes the 15 different malformations recorded; duodenal atresia (DA) (22.7%) being the most common followed by esophageal atresia (13.6%) and gastroschisis (13.6%). MTP was done in two fetuses with omphalocele having multiple other anomalies. There were four still births with median gestational age (GA) 32 weeks (31–33 weeks). The accuracy of prenatal diagnosis was 83.3%. | Table 1: Prenatal diagnoses, medical termination of pregnancy, stillbirth, and discordant postnatal diagnoses
Click here to view |
[Table 2] describes the 15 different malformations detected in sixty live births (91%). Cesarean section was done in only 23.33% (n = 14/60) of cases for indications not related to the fetal anomaly. The sex distribution was equal (male:female = 1:1.14). Two-third babies (n = 41; 68.3%) were low birth weight (LBW) and 56.7% (n = 34) of babies were preterm (PT). Newborn intensive care unit (NICU) admission was required in 73.3% (n = 44) of live births. DA (23.3%) was the most common anomaly, followed by esophageal atresia (13.6%) and gastroschisis (13.6%). Surgery was necessary in 35/56 (62.5%) neonates. Four out of the 16 neonates on close follow-up have been operated during late infancy, one each with following diagnosis – duplication cyst, Hirschsprung's disease, choledochal cys, t and retroperitoneal teratoma. Postnatal diagnosis differed in 16.7% of cases with no abnormality on US in 10.6% of babies and a different pathology in 6.1% of babies [Table 1]. One child with antenatally dilated bowel loops was asymptomatic at birth with normal US but developed symptoms in infancy and was confirmed as Hirschsprung's disease. Associated malformations were noted with GI and abdominal wall malformations: cardiac anomalies (6), down phenotype (2), VACTERL (2), bilateral congenital talipes equino varus (1), and small bowel atresia in two gastroschisis babies.
The details regarding mortalities are represented in [Table 3]. Neonates with associated malformations had a mortality rate of 54.5% (n = 6/10); five had cardiac anomalies and one had Down phenotype. Major cardiac anomaly was associated with one-third of all the mortalities. Furthermore, one-third of mortalities (n = 5/15) were preoperative; median GA being 35 weeks (32 weeks–39 weeks) at delivery; 3 of them were PT and LBW. Among the postoperative mortalities, the median GA was 35.3 weeks for survivors and 34.5 weeks among nonsurvivors. One baby with meconium peritonitis succumbed to refractory shock in immediate postoperative period. All other had postoperative complications manifesting in 2nd week-anastomotic leak being the most common followed by prolonged intestinal failure. The postsurgical survival for PT and LBW babies was 64% and 72%, respectively. Chi-square test showed no significant difference in the proportion of PT (P = 0.739) and LBW (P = 0.629) babies between survivors and nonsurvivors among the operated neonates [Table 4]. | Table 3: Comorbidities associated with the mortalities in live-born patients
Click here to view |
DA was the most common malformation (14/60). Down phenotype and cardiac anomaly were present in two cases each (14.3%). The median GA was 36 weeks 4 days (33 weeks–40 weeks). The postsurgical mortality was 35.7% – anastomotic leak in 3 and intestinal failure in 2 PT babies [Table 3]. Survival rate for LBW babies (4/6, 66.7%) was apparently better than those with normal BW (n = 4/8, 50%) [Table 4]. Of the 15 fetuses with abdominal wall defects, six had omphalocele; 50% major subtype. None of the omphalocele major cases survived – two MTP and one postnatal preoperative death [Table 1] and [Table 3]. All three omphalocele minor (50%) were isolated anomaly and did well. All the nine fetuses with gastroschisis were live births, LBW (median 1.81 kg), and small for GA; seven (77.8%) were PT (median GA 34 weeks). Eight patients were treated surgically with 87.6% survival. The only mortality occurred due to intestinal failure and fungal sepsis at around 6 weeks. Esophageal atresia (n = 8) was the third most common anomaly. Nearly 25% were pure esophageal atresia; they underwent cervical esophagostomy and feeding gastrostomy and survived; one of them had hemivertebrae with low ARM. Of the seven patients operated, 71.4% survived. Associated malformations were present in 3 (42.9%), five patients were LBW (71.4%), and 4 were PT (50%). The two postoperative deaths had anastomotic leak; both were type C, PT, and LBW and had associated major cardiac anomaly [Table 3].
Meconium peritonitis (15/66; 22.7%) was suspected by four different signs present as isolated findings without any combination of any of these. In fetuses with intra-abdominal calcification (IAC), 75% turned out to be normal and only one had meconium peritonitis in postnatal period [Table 1] and [Table 3]. There were five cases of fetal ascites (FA) with no survivors; two were stillborn (GA 32 weeks and 34 weeks), rest three were PT live births (median GA: 33 weeks) but died in the neonatal period due to refractory shock [Table 3]. Antenatal aspiration and postnatal peritoneal tap in the three live births were bilious. One child had a large right-sided loculated collection suggestive of giant meconium pseudocyst. Nearly 50% of fetuses with dilated bowel loops and echogenic bowel had a normal postnatal US. One baby with dilated bowel was a PT with small bowel atresia and one baby with echogenic bowel was still born at GA of 32 weeks 5 days [Table 1] and [Table 2].
Intra-abdominal cysts were noted in ten cases (15.2%), ovarian cysts comprising 40% (n = 4) of the lesions. Five cases (50%) have been operated [Table 2]. One ovarian cyst required surgery in neonatal period for a large abdominal mass ithw intestinal obstruction and the other four during follow-up in infancy [Figure 1]. Two were complex cysts near adrenal gland suggestive of neuroblastoma, however, postnatally diagnosed as benign lesions – subdiaphragmatic extrapulmonary sequestration (small nonprogressive and asymptomatic) and retroperitoneal teratoma (successfully operated).
Figure 1 depicts the summary of the observations in our study. Overall survival at 1-year follow-up was 62.1% (41/66); live-birth survival was 73.2% (41/56) and postoperative survival in neonates was 71.4%.
| Discussion | | |
Our study is the first of its kind from India analyzing the detailed outcome of fetal abdominal findings predicting nonurinary surgical malformations. Out of 66 fetuses, 91% were delivered as live births. The most common anomalies noted were bowel atresia, esophageal atresia, and abdominal wall defects. Our findings are similar to two other studies conducted in different regions; >12% of neonatal MCM were of GI tract.[2],[3] However, they have not reported other anomalies as in our study. DA is by far the most common GI anomaly with high prenatal detection rate.[12],[13]
The detection rate before 20-week gestation was better (31.8%) in comparison to a previous study (13.6%) by Kumar et al. (one of our coauthors) which included all types of anomalies.[11] Our finding is comparable to Rydberg and Tunón (39%)[14] and much better than Kaur et al. (9.4%),[15] but much less than Stefos et al. (85.7%)[13] where prenatal US was done by highly trained and experienced person. The highest accuracy for prenatal detection is for central nervous system, musculoskeletal system, renal malformations, abdominal wall defects, and DA among GI obstructive anomalies.[12],[13],[14],[16],[17],[18],[19] The overall accuracy of prenatal diagnosis, on including those detected beyond 20 weeks of gestation, in our study was 83.3%. It is consistent with the findings in an Indian study by Kaur et al.[15] which included renal malformations and better than reported in other studies ranging from 16% to 53.7%.[13],[17],[18],[19] The high may be better due to a higher proportion of anomalies such as DA, abdominal wall defects, and ovarian cysts with high prenatal detection rate. Thus, the variation in overall detection rate of the malformations noted in a study depends on following factors – the quality of US equipment, training and expertise in performing US, prenatal detection rate for individual anomaly and its proportion, timing, and frequency of prenatal US as malformations such as GI obstruction may evolve later in gestation and even postnatally.[17],[18],[19]
None of the malformations was an indication for cesarean section. Normal delivery occurred in most of the abdominal malformation (n = 46; 76.7%). Selected cases such as large abdominal wall defects with liver herniation or a large tumor with risk of dystocia during labor are exceptions.[20]
In our study, two-third of cases (n = 39/56; 69.6%) required surgery which is twice that 35% noticed by Sharma et al.,[10] implying the increased need for surgery in “nonurinary” malformations detected by US findings in fetal abdomen. We could not find any such study like ours to compare our observation. GI obstructions (n = 29/60; 48.3%) and abdominal wall malformations (n = 13/60; 21.7%) comprised the majority of surgical cases with a substantial number of cystic lesions (n = 10/60; 16.8%) [Table 2].
NICU level-4 is an essential adjunct as 73.3% of live-born babies needed intensive care, as noted in two other studies.[2],[3] It negated the adverse effects of prematurity and LBW on survival, making postoperative complications more apparent cause for mortality.
Overall mortality rate of 26.8% (n = 15/56; including preoperative deaths) is comparable with similar studies.[2],[3] Those with associated anomalies usually have a poor outcome.[21] Associated cardiac anomaly was present in 50% mortalities similar to observations by Kumar and Singh[2] Asindi et al. attributed 75% of fatality to postoperative infection and 25% to multiple anomalies.[3] In both these studies, they have analyzed only malformations related to hollow viscera in GI tract, we included other nonurinary surgical anomalies too. Survival beyond 90% has been reported for digestive tract anomalies.[7],[22],[24] This is much better than our live-birth survival rate of 73.2% but appears to be satisfactory in a developing country with limited resource settings. The cardiac anomalies are the most frequent associated malformation adversely affecting the overall prognosis. Nonavailability of cardiothoracic surgical services and the need for even better intensive care to manage sick neonates with postsurgical anastomotic complications and prevent nosocomial sepsis in patients on prolonged parenteral nutrition awaiting normal bowel function are potentially modifiable factors to improve survival.
Haeusler et al. have reported MTP in 15% of prenatally diagnosed GI obstructive anomalies.[23] We attribute our low MTP rate (3%) to late detection (>20 weeks) and higher proportion of isolated anomalies. Anomaly-specific counseling and information regarding reasonable postsurgical outcome in isolated malformations was very reassuring to the anxious parents.
Most of the GI obstructions manifested beyond 20-week gestation as mentioned in different studies.[12],[18],[19],[23] Our prenatal diagnostic accuracy of 93.3% (14/15) for DA is similar to other studies.[18] Although MTP is an option for DA associated with multiple associated anomalies,[19],[24],[25]both (n = 2/15; 13.3%) the fetuses detected before 20 weeks in our study had isolated anomaly. Higher postoperative complications have been reported in prenatally diagnosed esophageal atresia.[26] LBW was associated with longer gap length and higher mortality in an earlier study from our center.[27] We achieved good survival rate notably in abdominal wall malformations and esophageal atresia by ensuring in utero transfer to a tertiary care with timely counseling, thereby avoiding detrimental effects of postnatal transfer from periphery. The high survival rate of our prenatally diagnosed gastroschisis (87.6%) patients is exemplary for such condition matching that of developed countries.[7],[22]
Among the prenatally suspected meconium peritonitis, 75% of cases with IAC had a normal postnatal ultrasonography, however, none of the fetuses with FA survived [Table 1] and [Table 2]. IAC was present as isolated finding without any combination with other signs. As per scoring system by Zangheri et al., fetuses with isolated IAC had a favorable outcome, whereas associated additional findings increased the chances of postnatal surgery and such fetuses should be referred in-utero to a tertiary care.[28],[29],[30] FA was the most common finding in our study with very poor prognosis similar to observations of Kamata et al.[30] Hence, this knowledge is extremely helpful in antenatal counseling and planning management of such patients.
Intra-abdominal cysts constituted two-thirds of conservatively managed cases. The overall prenatal diagnostic accuracy for fetal abdominal cysts was 70% [Table 1] and 50% of patients required postnatal surgery. Ovarian cyst was the most common anomaly with 100% detection rate. The outcome is excellent even in those requiring surgery at 1-year follow-up. Different benign pathologies were noted in complex cysts prenatally suspected to be neuroblastoma highlighting the need for postnatal imaging in complex cysts to ascertain the correct diagnosis. All our observations are similar to a large series by Thakkar et al. except for the prenatal diagnostic accuracy of 92% in their study.[8]
Few lacunae of this study were a small number of different malformations in our series; no fruitful statistical analysis of the outcome was possible for individual malformation. A longer duration of study including a larger number of patients will provide a significant disease-related outcome. A better outcome is expected with the availability of expertise to manage comorbidities adversely affecting the survival. Moreover, there are no benchmark studies to compare with our cohort of nonurinary surgical malformations detected by US findings in fetal abdomen.
| Conclusions | | |
Our observations provide a useful information for realistic prognostication during prenatal counseling in nonurinary surgical malformations predicted by fetal abdominal signs: (1) high prenatal detection rate in most of the anomalies, low incidence of MTP in isolated anomalies, high rate of normal delivery and high survival rate with in utero transfer to tertiary care, (2) possibility of late detection beyond 20 weeks in GI obstructions, postnatal normalization of some prenatal US findings, and change of diagnosis, (3) in fetuses with signs of meconium peritonitis, isolated IAC had 75% survivors, whereas FA had none, and (4) availability of expertise for managing associated cardiovascular anomalies and better surgical NICU care can further improve the outcome in a limited resource setting.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Dastgiri S, Stone DH, Le-Ha C, Gilmour WH. Prevalence and secular trend of congenital anomalies in Glasgow, UK. Arch Dis Child 2002;86:257-63.  |
| 2. | Kumar A, Singh K. Major congenital malformations of gastrointestinal tract among the newborns in one of the English Carribean countries, 1993-2012. J Clin Neonatol 2014;3:205-10. [Full text] |
| 3. | Asindi AA, Al-Daama SA, Zayed MS, Fatinni YA. Congenital malformation of the gastrointestinal tract in Aseer region, Saudi Arabia. Saudi Med J 2002;23:1078-82. |
| 4. | Gandhi MK, Chaudhari UR, Thakor N. Incidence and distribution of congenital malformations clinically detected at birth: A prospective study at tertiary care hospital. Int J Res Med Sci 2016;4:1136-9. |
| 5. | |
| 6. | Bhide P, Kar A. A national estimate of the birth prevalence of congenital anomalies in India: Systematic review and metaanalysis. BMC Pediatr 2018;18:175. |
| 7. | Dastgiri S, Gilmour WH, Stone DH. Survival of children born with congenital anomalies. Arch Dis Child 2003;88:391-4. |
| 8. | Thakkar HS, Bradshaw C, Impey L, Lakhoo K. Post-natal outcomes of antenatally diagnosed intra-abdominal cysts: A 22-year single-institution series. Pediatr Surg Int 2015;31:187-90. |
| 9. | Choudhury SR. Fetal malformations and the Pediatric Surgeon. J Indian Assoc Pediatr Surg 2020;25:65-7. [ PUBMED] [Full text] |
| 10. | Sharma S, Bhanot R, Deka D, Bajpai M, Gupta DK. Impact of fetal counseling on outcome of antenatal congenital surgical anomalies. Pediatr Surg Int 2017;33:203-12. |
| 11. | Kumar M, Sharma S, Bhagat M, Gupta U, Anand R, Puri A, et al. Postnatal outcome of congenital anomalies in low resource setting. Prenat Diagn 2013;33:983-9. |
| 12. | Phelps S, Fisher R, Partington A, Dykes E. Prenatal ultrasound diagnosis of gastrointestinal malformations. J Pediatr Surg 1997;32:438-40. |
| 13. | Stefos T, Plachouras N, Sotiriadis A, Papadimitriou D, Almoussa N, Navrozoglou I, et al. Routine obstetrical ultrasound at 18-22 weeks: Our experience on 7,236 fetuses. J Matern Fetal Med 1999;8:64-9. |
| 14. | Rydberg C, Tunón K. Detection of fetal abnormalities by second-trimester ultrasound screening in a non-selected population. Acta Obstet Gynecol Scand 2017;96:176-82. |
| 15. | |
| 16. | Romosan G, Henriksson E, Rylander A, Valentin L. Diagnostic performance of routine ultrasound screening for fetal abnormalities in an unselected Swedish population in 2000-2005. Ultrasound Obstet Gynecol 2009;34:526-33. |
| 17. | Stoll C, Tenconi R, Clementi M. Detection of congenital anomalies by fetal ultrasonographic examination across Europe. Community Genet 2001;4:225-32. |
| 18. | Grandjean H, Larroque D, Levi S. The performance of routine ultrasonographic screening of pregnancies in the Eurofetus Study. Am J Obstet Gynecol 1999;181:446-54. |
| 19. | Chitty LS, Hunt GH, Moore J, Lobb MO. Effectiveness of routine ultrasonography in detecting fetal structural abnormalities in a low risk population. BMJ 1991;303:1165-9. |
| 20. | Wataganara T, Grunebaum A, Chervenak F, Wielgos M. Delivery modes in case of fetal malformations. J Perinat Med 2017;45:273-9. |
| 21. | Garne E, Rasmussen L, Husby S. Gastrointestinal malformations in Funen county, Denmark--epidemiology, associated malformations, surgery and mortality. Eur J Pediatr Surg 2002;12:101-6. |
| 22. | Schneuer FJ, Bell JC, Shand AW, Walker K, Badawi N, Nassar N. Five-year survival of infants with major congenital anomalies: A registry based study. Acta Paediatr 2019;108:2008-18. |
| 23. | Haeusler MC, Berghold A, Stoll C, Barisic I, Clementi M, EUROSCAN Study Group. Prenatal ultrasonographic detection of gastrointestinal obstruction: Results from 18 European congenital anomaly registries. Prenat Diagn 2002;22:616-23. |
| 24. | Choudhry MS, Rahman N, Boyd P, Lakhoo K. Duodenal atresia: Associated anomalies, prenatal diagnosis and outcome. Pediatr Surg Int 2009;25:727-30. |
| 25. | Lawrence MJ, Ford WD, Furness ME, Hayward T, Wilson T. Congenital duodenal obstruction: Early antenatal ultrasound diagnosis. Pediatr Surg Int 2000;16:342-5. |
| 26. | Garabedian C, Sfeir R, Langlois C, Bonnard A, Khen-Dunlop N, Gelas T, et al. Does prenatal diagnosis modify neonatal treatment and early outcome of children with esophageal atresia? Am J Obstet Gynecol 2015;212:340.e1-7. |
| 27. | Rassiwala M, Choudhury SR, Yadav PS, Jhanwar P, Agarwal RP, Chadha R, et al. Determinants of gap length in esophageal atresia with tracheoesophageal fistula and the impact of gap length on outcome. J Indian Assoc Pediatr Surg 2016;21:126-30. [ PUBMED] [Full text] |
| 28. | Zangheri G, Andreani M, Ciriello E, Urban G, Incerti M, Vergani P. Fetal intra-abdominal calcifications from meconium peritonitis: Sonographic predictors of postnatal surgery. Prenat Diagn 2007;27:960-3. |
| 29. | Zerhouni S, Mayer C, Skarsgard ED. Can we select fetuses with intra-abdominal calcification for delivery in neonatal surgical centres? J Pediatr Surg 2013;48:946-50. |
| 30. | Kamata S, Nose K, Ishikawa S, Usui N, Sawai T, Kitayama Y, et al. Meconium peritonitis in utero. Pediatr Surg Int 2000;16:377-9. |
[Figure 1]
[Table 1], [Table 2], [Table 3], [Table 4]
|
Search Pubmed for