|Year : 2020 | Volume
| Issue : 4 | Page : 280-285
Challenges in the management of omphalocele in Ile-Ife, Nigeria
Ademola Olusegun Talabi, Oludayo Adedapo Sowande, Olusanya Adejuyigbe
Department of Surgery, Division of Paediatric Surgery, Obafemi Awolowo University Teaching Hospitals Complex, Obafemi Awolowo University, Ile-Ife, Osun State, Nigeria
|Date of Submission||11-Jul-2020|
|Date of Acceptance||13-Aug-2020|
|Date of Web Publication||01-Oct-2020|
Dr. Ademola Olusegun Talabi
Department of Surgery, Division of Paediatric Surgery, Obafemi Awolowo University Teaching Hospitals Complex, Obafemi Awolowo University, Ile-Ife, Osun State
Source of Support: None, Conflict of Interest: None
Introduction: The management of omphalocele poses serious challenges in developing countries with significant morbidity and mortality. We highlighted the challenges and outcomes of management in a teaching hospital. Materials and Methods: This was a retrospective review of neonates that presented with omphalocele between January 2010 and December 2019 at a tertiary hospital in Nigeria. Information on bio data of patients and their parents, the gestational age, state of the membrane, presence of concomitant congenital anomalies, and treatment outcome were obtained from the patients' case notes. Patients were stratified into three groups as follows: Group A represented intact omphalocele minor, Group B were intact omphalocele major, whereas Group C were ruptured omphalocele. Data were analyzed using descriptive and Chi-square analysis. Results: There were 20 males and 30 females with a male: female ratio of 1:1.5. Their ages ranged between a few hours and 21 days, with a median of 1 day. Thirty-five (70%) neonates presented within the 1st day of life. The mean maternal age was 31.4 ± 6.1 years. Only 14 (28%) children had one or more prenatal ultrasonography with no prenatal ultrasound diagnosis. Six children presented with a ruptured omphalocele major. Thirty-six (72%) neonates were managed conservatively. Group C had the highest mortality rate 6 (100%), compared with 6 (0%) and 23 (39.5%) in Groups A and B, respectively, (P < 0.0001). We had an overall mortality rate of 42%. Conclusion: The mortality rate is still high in our institution, especially among those with ruptured omphalocele.
Keywords: Mortality rate, omphalocele, prenatal diagnosis, ruptured
|How to cite this article:|
Talabi AO, Sowande OA, Adejuyigbe O. Challenges in the management of omphalocele in Ile-Ife, Nigeria. J Clin Neonatol 2020;9:280-5
| Introduction|| |
Omphalocele is a congenital anterior abdominal wall defect covered by a membranous sac. The defect can be anywhere in the midline ranging from 2 cm to 12 cm in its widest diameter. The contents are usually bowel loops, liver, and sometimes other abdominal viscera. It has an incidence of 1 in 4000 to 6000 live births in developed nations with the mothers of affected children usually in their fourth decade of life. The incidence remains unknown in developing countries despite its significant contribution to the overall neonatal morbidity and mortality.,,,,,,
It is frequently associated with other structural anomalies in 30%–88% of cases with cardiac and gastrointestinal tract anomalies being the most common, as well as syndromes such as Beckwith-Wiedemann, Pentalogy of Cantrell, and omphalocele, exstrophy of the bladder/cloaca, imperforate anus and spinal Bifida anomalies.,,,,
Chromosomal anomalies, most commonly trisomies 13, 15, 18, and 21, are seen in about a third of patients, particularly in infants with small omphaloceles that do not contain the liver. In contrast, many patients with large omphalocele have associated pulmonary hypoplasia. These associated anomalies are the primary determinants of outcome for infants with omphalocele.,,, Literature reports in the West-African sub-region noted a high mortality rate ranging from 22.9% to 45%,,,, whereas mortality has reduced from 90% in 1960 to 10% in the last quarter of the nineteenth century in developed countries. The burden of this abnormality seems to overwhelm the limited existing facilities in developing countries; hence, survival among these patients seems to depend largely on the decision taken by the pediatric surgeons on either to operate or to manage conservatively as well as other pre- and post-operative factors.
The treatment of omphalocele is evolving and is centered around conservative or surgical approach. Although the ultimate goal of therapy is to return herniated intestinal loops to the abdomen and to close up the skin and fascia without causing excessive intra-abdominal tension, the treatment options depend on the size of the fascial defect and the presence of concomitant anomalies.,
In this study, we presented a 10-year retrospective review in a tertiary hospital in Nigeria with a view to identifying the challenges encountered in the management of patients with omphalocele.
| Materials and Methods|| |
A retrospective review of all infants that presented with omplalocele from January 2010 to December 2019 in our hospital. Consent to embark on the study was obtained from the Ethics and Research Committee of our teaching hospital.
The extracted data included their bio data, antenatal care, place/mode of delivery, gestational age at delivery, size of omphalocele, state of the sac, the clinical state of the baby on arrival, complications, associated anomalies and syndromes, socioeconomic status of the parents, and outcome. The babies were assessed and resuscitated under incubator with intravenous fluid, broad-spectrum antibiotics, nasogastric tube aspiration, and urinary catheterization. In addition, parenteral Vitamin K was administered, and blood sample was taken for random blood sugar. They were classified and managed as follows:
- Group A: Size of defect <5 cm ± syndrome with the membrane intact managed by primary fascia closure or where necessary conservatively
- Group B: Size of defect >5 cm ± syndrome with the membrane intact, had initial conservative management and fascial closure when the peritoneal cavity was assessed as adequate
- Group C: Any size of defect ± syndrome with the ruptured membrane, had initial primary fascial or improvised silo closure.
Conservative or nonoperative management involved hospitalization for 2–4 weeks. The membranous sac was dressed daily with 70% alcohol in the majority of our babies and in some cases sufra-tulle gauze, honey, silver sulfadiazine, and povidone-iodine. Afterward, they were discharged to an outpatient clinic for follow-up. Epithelization occurred between 4 and 8 weeks with a resultant ventral hernia, which allowed time for the assessment of intra-abdominal volume adequacy on a monthly basis for fascial closure. Hernia repair was accomplished by simple fascial closure or Mayo's repair under general anesthesia. Sometimes we reinforced our repair with a mesh. An increase in intra-abdominal pressure/diaphragmatic splinting was observed as increased resistance to ventilation. Wound was inspected on the 4th–5th postoperative days, and skin sutures were removed on the 10th–14th day. Babies were thereafter discharged to follow-up clinic. For the purpose of this study, we defined abnormal temperature as temperature <36.5°C or >37.4°C.
The data obtained were analyzed using SPSS version 21 (IBM, Chicago, IL, USA). Descriptive and Chi-square analysis was done with continuous variables and expressed as means and standard deviation, while categorical variables were presented as tables. A value of P < 0.05 was taken as significant.
| Results|| |
During the study period, 54 neonates were admitted. Of these, 50 children's records were analyzed, while four children were discharged against medical advice. There were 20 (40%) males and 30 (60%) females, giving a male: female ratio of 1:1.5. Their ages ranged between few hours and 21 days with a median age of 1 day. Thirty-five (70%) neonates presented within the 1st day of life. Their birth weights were between 1.4 kg and 3.8 kg, with a mean of 2.8 ± 0.5 kg. Thirty-two (64%) patients were admitted with abnormal temperature. Their mothers' age range was 19–47 years with a mean of 31.4 ± 6.1 years [Table 1]. Most (82%) mothers belonged to the low socioeconomic class. Nearly all children (98%) were delivered at term. Most (94%) babies were delivered through spontaneous vertex deliveries, one (2%) baby through cesarean section, and two babies (4%) through breech deliveries. Fourteen (28%) children had one or more prenatal ultrasonography, but none was diagnosed prenatally. Three (6%) babies were twins, with the second twins being normal. The birth order varies from 1 to 6, with a majority 30 (60%) belonging to the third birth order and above. The places of birth are shown in [Table 1].
There were 44 (88%) patients whose defects were greater than 5 cm (omphalocele major) while 6 (12%) patients had minor omphalocele (defects <5 cm). Six (12%) children presented with ruptured omphalocele major: the sac ruptured during breech delivery at a maternity home in one patient, two at the time of care at home while the remaining three ruptured during transfer to our hospital. The eviscerated viscera consisted mainly of the liver, intestine, and stomach. They were managed surgically by housing the viscera in a silastic bag [Figure 1]a and [Figure 1]b. Majority 36 (72%) of our patients were brought with the membrane wrapped in the clothing of their mothers with some of the membranous sacs being infected. The various associated congenital anomalies seen are shown in [Table 2] [Figure 1]c. The liver was part of the content of the defect in 42 (84%) patients on postnatal ultrasound.
|Figure 1: (a) Ruptured omphalocele major with eviscerated stomach and intestine. (b) Ruptured omphalocele major with improvised silastic bag (urinary bag). (c) Intact omphalocele major with multiple associated congenital anomalies; Microcephaly, bilateral cleft lip, patent ductus arteriosus, and ventricular septa defect. (d) Healed ventral hernia in a patient with Omphaloce major|
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There were 6 (12%) neonates in Group A, 38 (76%) were included in Group B, and 6 (12%) in Group C. Two children in Group B with adequate intra-abdominal volume had primary fascial and skin closure, while others in this group either because of large size of fascial defect, the liver, or comorbidities were managed nonoperatively [Table 3]. The sacs of most patients in Group B were dressed with 70% alcohol and sometimes with any of the following agents; honey, povidone-iodine, silver sulfadiazine and sufratulle. Epithelization and skin coverage were achieved between 1 and 2 months in those who survived. All patients in Group C had inadequate intra-abdominal volume and were treated with improvised silastic bag.
Of the 21 (42%) children that eventually had ventral hernias [Figure 1]d, 11 (52.4%) had simple herniorrhaphy or mesh hernioplasty between 1 and 6 years of age.
Group C had the worst mortality rate 6 (100%), compared with 6 (0%) and 15 (39.5%) in Groups A and B, respectively, (P < 0.0001) [Table 3]. We had an overall mortality rate of 42%. The causes of death were mainly sepsis, pulmonary hypoplasia, and possibly congenital cardiac defects due to central cyanosis seen in some of the babies.
| Discussion|| |
This study reported that mortality was unacceptably high in our hospital with ruptured omphalocele having the worst prognosis. The etiology of omphalocele has not been unraveled. Some authors proposed genetic or chromosomal abnormalities, failure of return of the mid-gut following physiologic herniation, while others suggested the failure of the lateral embryonic folds to fuse in the midline.,,,, These theories were supported by the high level of congenital anomalies and syndromes associated with this condition. In this study, some of our patients demonstrated these associated anomalies. Unfortunately, intrinsic cardiac defects, which constitute a significant proportion of anomalies associated with omphalocele could not be diagnosed in this series because dedicated pediatric cardiologists and echocardiographic facility for children were not available in our institution in most of the early years of this study. This constitutes a limitation to our study. Notwithstanding, three out of the five patients that eventually had echocardiography had either patent ductus arteriosus, ventricular septa defect or combination of both. Literature reports have shown that cardiac defects and other life-threatening anomalies are responsible for the high morbidity and mortality in most cases of omphalocele.,,
In developed societies, prenatal ultrasound diagnosis, counseling, supervision of delivery and adequate care of the defect is the norm, whereas in many nations in sub-Saharan Africa prenatal diagnosis is lacking, deliveries are not properly supervised, and most children are referred to tertiary hospitals without adequate care and covering of the defect with sterile, moist and warm gauze.,,,,, In our study, when the babies were first seen by pediatricians or surgeons, many of them were wrapped in their mothers' clothing, with many having elevated or sub-clinical temperatures at admission. Transportation in warm environment was lacking, and many of the babies arrived at the referral centers suffering from hypothermia and dehydration. In addition, the membranes were heavily contaminated if not infected. It was not surprising that ruptured membrane and sepsis due to improper handling of babies who had traveled several kilometers before arrival at the referral hospital occurred in this series. Our finding is consistent with most reports from other centers in Nigeria and other developing countries.,, Prenatal screening, access to care, and surgical safety need to be improved in our settings so that these cases can be detected and referred in-utero to well-equipped centers for proper management. Adequate nutritional supplementation during pregnancy had been recommended as the use of multivitamins during antenatal care has been shown to reduce the incidence of nonsyndromic ompalocele by 60%. The fact that the membranes covering the content of the defect ruptured either at home during delivery, time of care, or transportation to the hospital lay credence to the burden of poor access to safe health-care services in our region.
The mean age of mothers in this series is in tandem with previous reports that indicated that most mothers of neonates with omphalocele are usually advanced in age with multiple parities and increase the risk of congenital anomalies.,, There is a need for the government of African countries to overhaul antenatal services to best practices so that all pregnant women will not only have an ultrasound scan for the purpose of fetal monitoring but also for prenatal diagnosis of congenital anomalies with special attention giving to mothers in their late 30 s.
The management of omphalocele is quite challenging in our clime due to a number of reasons. first, there is the dearth of neonatal intensive care unit (NICU) where neonatal ventilator support and other monitoring devices can be giving to children with respiratory embarrassment from associated pulmonary hypoplasia or where positive pressure ventilation and muscle paralysis is required during staged closure with silastic bag. The six patients that had membrane rupture could have benefitted from these life monitoring and supporting devices if they were available. Second, total parenteral nutrition was not available in many hospitals, making survival difficult for some of these children who may require high-calorie diet. Even when available, affordability of total parental nutrition remains a big issue as most of the parents were poor who had to pay for medical services out of pockets. Third, sepsis from gross contamination of membrane or infected membrane during transportation or admission was one of the major determinants of survival. To make things worse, these children were often nursed in open wards where other pediatric conditions were being managed.,, In addition, major omphalocele often contains the liver as part of the content of the herniated viscera., This implies that the surgeon must ensure that the peritoneal cavity is capacious enough and care must be taken during reduction of the protruded viscera and closure of the anterior abdominal wall so as not to inadvertently kink the hepatic veins and other great vessels which may compromise venous return to the heart. In our study, the liver was part of the content of the defect in most of the omphalocele major. Because the peritoneal cavity is not adequate in most cases of omphalocele major, the attempt at primary closure will cause abdominal compart syndrome characterized by impaired diaphragmatic movement resulting in reduced lung extrinsic compliance, reduced venous return to the heart and mesenteric ischemia. For these reasons, the introduction of viscera into the peritoneal cavity can lead to complications that may preclude primary closure for omphalocele major.,
Against this backdrop, pediatric surgeons working in developing countries need to make a concrete decisions pertaining to the mode of treatment, whether conservative or surgical management. This is because the mortality rate depends to some extent on the decision taken by the attending pediatric surgeon. In our set up, we have learnt to manage large omphalocele greater than 5 cm conservatively except for few cases in which the peritoneal cavity was adjudged to be adequate to accommodate the returning bowel without, compromising ventilation and circulation. Primary surgical repair was reserved for minor omphalocele irrespective of the status of the sac provided that there was no anomaly that precluded surgical intervention.
The management of ruptured omphalocele (Group A) continues to be problematic in our environment, and the mortality remains high. In our setting and in a similar environment,, this cohort of babies was treated surgically with improvised silo made up of sterile urinary or infusion bags with sequential reduction of the external peritoneal viscera over some days without ventilator support and parenteral nutrition. Sadly, some of these improvised materials separated from the fascia within a few days of application, thereby necessitating multiple surgeries with consequent hypothermia, loss of body fluid, and septicemia. Therefore, it was not surprising that the mortality rate was 100% in these patients. Recently, preformed spring-loaded silastic bag have been introduced in high-income countries to cover the defect without fascial closure, thereby eliminating the need for anesthesia and thus reduce the risk of compartment syndrome and stress of surgery. This newer device will benefit low-income countries where neonatal and pediatric anesthesia is quite a challenge, especially in patients with multiple congenital anomalies who are brought to referral hospitals in compromised health status. We believe that the provision of neonatal ventilator support (NICU), ideal silastic bags, and total parenteral nutrition may improve survival in these patients.
Initial conservative management of omphalocele major involved the use of escharotic agents such as 70% alcohol, povidone-iodine, silver sulfadiazine, honey, sufratulle, dermazine cream, and 2.5% formal saline.,,,, The choice of escharotic agent was based on the preference of the surgeon and to some extent, on the degree of contamination or infection of the sac. In our series, we preferred to use 70% alcohol because it was readily available and free. The only drawback of 70% alcohol was its toxic side effect that made the babies to sleep for a prolonged period. Subsequent treatment of the ventral hernia when the intra-abdominal volume was considered to be adequate involved simple fascial closure which could be reinforced by mesh or not. Other modern devices that can be used ab initio at birth or infancy include staged reduction with tissue expanders, alloderm graft over the fascial defect, and vacuum-assisted closure device which allow for better outcome of treatment and excellent long-term quality of life in high-income societies. Since these devices are lacking in our sub-region, pediatric surgeons often rely on conservative therapy until these children have developed adequate abdominal volume before natural tissue repair (fascial closure) of ventral hernias are embarked upon, sometimes at a relatively older age.
Our study observed that while all babies in Group C died, those in Group A had the lowest mortality rate. The low mortality rate in Group A was probably due to the small size defect and lack of life-threatening comorbidities. Our experience was similar to that of Osifo et al. The overall mortality rate of 42% in this series is comparable to previous studies from Nigeria and other developing nations but far higher than those from developed countries.,,,, However, Rattan et al. in India recorded a single-digit mortality rate of 8% in their series. We believe that with improvement in prenatal diagnosis, better obstetric services, availability of life-supporting facilities and parenteral nutrition, surgeons practicing in low-income countries may witness improved survival among these patients.
Pediatric echocardiography was not done in most of the children in this study. Hence, a lot of patients with congenital heart defects would have been missed.
| Conclusion|| |
The management of omphalocele remains problematic in our hospital due to the ruptured membrane, sepsis, and probably undiagnosed cardiac defects. The mortality rate remains high in our hospital. We are of the opinion that with good obstetric services, availability of modern supportive equipment and total parenteral nutrition will improve survival in these babies.
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Conflicts of interest
There are no conflicts of interest.
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[Table 1], [Table 2], [Table 3]