|Year : 2018 | Volume
| Issue : 3 | Page : 125-129
Omphalocele: 15-years experience from a single center in developing country
Kamal Nain Rattan1, Jasbir Singh2, Rakesh Jakhar2, Poonam Dalal2, Pallavi Sonika2
1 Department of Paediatric Surgery, PGIMS, Rohtak, Haryana, India
2 Department of Paediatrics, PGIMS, Rohtak, Haryana, India
|Date of Web Publication||2-Aug-2018|
Dr. Jasbir Singh
Department of Paediatrics, PGIMS, Rohtak - 124 001, Haryana
Source of Support: None, Conflict of Interest: None
Background: Omphalocele is one of the most common anterior abdominal wall defects and still remains a management challenge in developing countries. A multidisciplinary team approach involving various prenatal and postnatal interventions is required to have a favorable outcome. In this study, we had analyzed the clinical profile and outcome of the neonates with omphalocele in a developing country. Materials and Methods: We had done a retrospective analysis of medical records of neonates who were admitted with omphalocele at our center from 2002 to 2016. Results: A total of 65 neonates were enrolled in the study with a male-to-female ratio of 1.6:1. Mean birth weight was 2550 ± 670 g and mean gestational age was 37.2 ± 3.4 weeks. Polyhydramnios was present in 40% pregnancies and only 30% (20/65) were diagnosed on prenatal ultrasound screening. Nearly 31 neonates (48%) were having omphalocele minor, and 34 (52%) were diagnosed with omphalocele major. The incidence of associated anomalies was 40% (26/65) with cardiac diseases being the most common followed by gut anomalies. Primary closure of defect was done in 78.5% (51/65) neonates, and delayed surgical repair was performed in 21.5% (14/65) neonates. Mean duration of postoperative hospital stay was 15.5 ± 3.5 days. Five patients (7.6%) expired during study period including two with associated congenital anomalies and one with rupture of omphalocele major sac membrane. Conclusion: Despite significant improvements, management of omphalocele still poses a challenge due to variable size of the defect and associated anomalies. Whenever feasible, early primary surgical repair remains the ideal procedure to have a good outcome.
Keywords: Congenital, escharotics, neonates, omphalocele, surgery
|How to cite this article:|
Rattan KN, Singh J, Jakhar R, Dalal P, Sonika P. Omphalocele: 15-years experience from a single center in developing country. J Clin Neonatol 2018;7:125-9
|How to cite this URL:|
Rattan KN, Singh J, Jakhar R, Dalal P, Sonika P. Omphalocele: 15-years experience from a single center in developing country. J Clin Neonatol [serial online] 2018 [cited 2018 Dec 13];7:125-9. Available from: http://www.jcnonweb.com/text.asp?2018/7/3/125/238393
Omphalocele is a congenital anomaly consisting of herniation of viscera into membranous sac through a ventral abdominal wall defect. The reported prevalence of omphalocele is 0.9—3.8 per 10,000 live births. The preferred treatment for omphalocele is the primary closure of abdominal wall defect. However, large-sized defects are difficult to manage owing to size disproportion between the omphalocele content and abdominal cavity. In such cases, staged repair is performed which includes various measures to enhance the size of the abdominal cavity. Since the concept of a ventral hernia was given by Gross in 1948, staged repair techniques have evolved considerably., Many methods using prosthetic material such as Schuster bag, silastic silo, tissue expander, and vicryl mesh had been used with variable success rates., However, still, there is no universally accepted method of managing patients with a giant omphalocele. The purpose of this study was to analyze the clinical profile and short-term outcome in neonates with omphalocele who were treated at our center.
[TAG:2]Materials and Methods[/TAG:2]
It was a retrospective study done at a tertiary care center in North India during the period spanning over 2002—2016. We analyzed the medical records of neonates admitted with omphalocele at our center. Data collected was analyzed for gestational age, gender, birth weight, size of the defect, associated congenital anomalies, surgical intervention performed, and final outcome.
The lesion was classified as omphalocele minor if the size of the defect was <5 cm and omphalocele major if it was >5 cm. In addition, if sac contains major part of the liver, it was labeled as omphalocele major. As a department policy, in omphalocele minor and some cases of omphalocele major where the content of sac can be reduced without any complications, we had performed early primary closure of the defect. In omphalocele major with significant size disproportion, the delayed repair was performed after a variable period of escharotics therapy.
A total of 65 neonates were included in the study with a male-to-female ratio (M:F) 1.6:1. Mean gestational age was 37.2 ± 3.4 weeks and mean birth weight was 2550 ± 670 g. Although a history of polyhydramnios was present in 40% (26/65) pregnancies, only 30% (20/65) were diagnosed on prenatal ultrasound screening. About 15% of neonates in the study were preterm.
About 31 neonates (48%) were having omphalocele minor and 34 (52%) neonates were with omphalocele major [Table 1]. Associated congenital anomalies were seen in 40% (26/65) neonates [Table 2]. Cardiac anomalies were the most common to be observed in eight patients. The incidence of congenital anomalies was significantly higher in neonates with omphalocele minor (48%) as compared to omphalocele major (32%). We had performed chest and abdomen radiograph, ultrasound abdomen (USG), and computed tomography scan to rule out associated anomalies. Echocardiography was done in all patients, and magnetic resonance imaging scan was done when the clinical examination was indicative (e.g., hetropagus twins). Karyotyping was done in 21 neonates and confirmed Down's syndrome in five patients. In patients with chromosomal anomalies, we had planned for genetic counseling to the parents.
|Table 1: Distribution of patients according to defect size and surgical interventions performed|
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- Primary closure [Table 1], Group A and B1] — After preoperative optimization, primary repair was performed in 51 neonates including 20 with omphalocele major. The timing of primary closure was within first 48 h of life in 44 neonates; however, it got delayed beyond the 1st week in seven patients due to a late referral from the periphery. In associated anomalies, the teratoma was excised without any complications, and the primary repair of exomphalos major was performed. Three cases were having broad base Meckel's diverticulum, so they were not excised. In neonates with ileal atresia and patent vitello-intestinal duct, we had performed resection and anastomosis. The colostomy was performed in a neonate with the anorectal malformation (ARM). The patients with Beckwith—Wiedemann syndrome and exstrophy of the bladder were managed conservatively
All neonates were managed in surgical Neonatal Intensive Care Unit during the postoperative period. Monitoring for hemodynamic stability and metabolic complications such as hypoglycemia and electrolyte imbalance was done. The patients were transfused with blood product as and when needed. Postoperatively, when the baby had passed meconium, supervised feeding was started. Once the baby started tolerating feeds, passing feces and showed weight gain, discharge from hospital was planned.
- Delayed repair [Table 1], Group B2] — In 14 cases with omphalocele major associated with significant size discordance, the delayed repair was performed after escharotics therapy. It consists of daily application of 5% povidone-iodine solution on omphalocele sac followed by an antiseptic dressing. In regular weekly follow-up, we continued to assess the general condition of the baby, weight gain, and feed tolerance. Mean duration for epithelialization was 12 ± 2.5 weeks. Depending on the discrepancy of size between omphalocele contents and abdominal cavity, the definitive repair was performed at an age varying from 3 to 8 months.
Morbidity and mortality
Mean hospital stay was 15.5 ± 3.5 days in primary repair group (Group A and B1) as compared to 22.0 ± 4.5 days in the second group (Group B2). In late referrals where the primary repair was delayed, dense adhesions were developed between sac membrane with underlying gut wall and the liver surface. During separation of membranous adhesions peroperatively, gut perforation had occurred in four cases and significant hemorrhage occurred from the liver surface. Postoperative sepsis was seen in nine cases (four omphalocele minor and five omphalocele major), which were managed conservatively. Among the five deaths observed during the study period, four neonates were preterm and low birth weight (<2200 g). Out of five deaths, two neonates with associated congenital anomalies expired in the postoperative period (one each with congenital heart disease [CHD] and hetropagus parasite twin). In one neonate with omphalocele major, rupture of sac occurred and emergency closure of defect had to be performed. This patient expired later in postoperative period due to sepsis. Overall mortality in primary repair group was 3.9% (2/51) and 14% (2/14) in delayed repair group.
Omphalocele is the anterior abdominal wall defect characterized by herniation of various organs in midline translucent sac. Faulty reduction of physiological herniation of gut at the 10—12th week of intrauterine period is thought to be the underlying cause. The covering of translucent sac is formed by peritoneum, Wharton's jelly, and amnion. Depending on the size of defect (< or >5 cm) and content of sac (part or whole liver), it may be classified as omphalocele minor or major, respectively. Mac Bird et al. had reported that omphaloceles occur more frequently in males than in females. In present series, we had also observed a male preponderance.
With the availability of better prenatal screening methods such as ultrasound (USG) and alpha-fetoprotein analysis, the omphalocele can be detected in intrauterine life. However, since the reduction of physiological herniated gut occurs at the 6—10th week of gestation, most of these cases are detected late in the first trimester. Liang et al. had reported improvement in first-trimester prenatal detection rate over from 18% to 43% in their series during 1994—2011. The prenatal detection rate in our series was 30%, which is considerably lower than the Western countries. In addition, the mean age of prenatal diagnosis in our series was 25 weeks, which can be attributed to lack of access to advanced diagnostic techniques. As most of the patients belong to remote peripheral areas with inadequately equipped health centers, many complications arise at the time of high-risk delivery. Moreover, these neonates are exposed to hypothermia and dehydration during transport to referral centers. Hence, prenatal screening quality needs to be improved in our settings so that these cases can be referred in utero to the well-equipped hospital for better management. Adequate nutrition supplementation during pregnancy is also helpful as it had been reported that with the use of periconceptional multivitamin, the risk of nonsyndromic omphalocele gets reduced by 60%.
Omphalocele had been described to be associated with other congenital anomalies in 40%—80% patients., In our series, the incidence of anomalies was 40% with CHD being most common. Askarpour et al. had reported the incidence of associated anomalies as 27.8% in their review of 42 neonates with omphalocele. As reported by Kumar et al., size of omphalocele had an impact on the type of associated anomaly. Smaller lesions are associated with gut malformations, and larger lesions are likely to be associated with cardiac disease. Our study also supports these findings as all of the gastrointestinal malformations were observed in neonates with omphalocele minor. Among these, three patients were found to have ileal atresia. Although the exact mechanism for association of gut atresia is not clear, vascular catastrophe is thought to be an underlying cause. In view of the presence of associated anomalies, the clinician should remain alert to detect them early for planning appropriate treatment and to minimize the complications.
Elective surgical interventions are the mainstay of the treatment in the neonate with omphalocele. Early primary repair of defect often yields good results as obtained in our study. However, in late referral, the sac membrane became infected due to lack of dressing materials and adequate care of the newborn at periphery. The thickened and infected membrane develops dense adhesion with gut wall and viscera leading to fatal complications during surgery. Hence, peripheral health workers should be taught about the basic care of sac and early referral. The decision to perform primary repair may be undertaken in neonates with good general condition, small size defect, and hemodynamic stability. This procedure is of short duration and has advantages of early recovery and shorter hospital stay. In omphalocele major, due to the disproportion between size of the sac content and abdominal cavity, reduction of the herniated viscera into the abdomen can lead to complications such as abdominal compartment syndrome. In these cases, staged repair is planned which consist of various measures to enhance abdominal cavity volume. A conservative method employed for the management of omphalocele major is “escharotic therapy” which results in gradual epithelialization of the omphalocele sac by application of antiseptic solution and corrective surgery can be performed later on. Various reagents such as povidone-iodine and merbromin can be used to promote escharification of the omphalocele sac membrane. Due to increased risk of complications with iodine-based escharotics such as hypothyroidism, their use is an issue of debate. In a review of 173 neonates with a giant omphalocele, Kouame et al. had reported favorable outcome with application of dissodic 2% aqueous eosin. Pandey et al. had documented good outcome using a combination of povidone-iodine and topical antibiotics as it results in rapid escharification and minimizes the risk of hypothyroidism. In resource-poor country similar to our settings, Nicoara et al. had used cost-effective Manuka honey as escharotic agent and described no adverse effect.
With five deaths, the overall survival rate in present cohort was 92% (60/65). Osifo et al. had documented good survival of 97% (32/33) in neonates with small size defect (<4.5 cm) and intact omphalocele membrane. Marshall et al. had reported a survival rate of 71.3% in their population-based study and recognized chromosomal anomalies and very low birth weight as consistent determinants of 1-year survival. The most significant factors associated with poor outcome in our series were suboptimal prenatal screening, prematurity, low birth weight, associated congenital anomalies, late referral, and rupture of sac membrane. Thus, managing omphalocele may prove difficult and a multidisciplinary approach is essential is to have a favorable outcome.
Omphalocele still remains a challenging issue for pediatric surgeon and neonatologist. Simple interventions in low resource settings like aseptic dressing and planned transport may decrease the complications. Early primary surgical repair of the defect has good outcome and may be preferred treatment option whenever feasible. The conservative treatment of giant omphalocele with the application of the povidone-iodine can be utilized effectively in resource-poor settings.
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