Archives of International Surgery

CASE REPORT
Year
: 2019  |  Volume : 9  |  Issue : 4  |  Page : 116--119

The role of radiology in the management of omphalopagus: Our experience at university of Abuja teaching hospital


Joshua O Aiyekomogbon1, Ukamaka D Itanyi1, Samson Olori2, John Y Chinda2, Philip Mshelbwala2, Daphnie M A Leslie1, BA Ekele3,  
1 Department of Radiology, University of Abuja Teaching Hospital, Abuja, Nigeria
2 Department of Surgery, Paediatric Surgery Unit, University of Abuja Teaching Hospital, Abuja, Nigeria
3 Department of Obstetrics and Gynaecology, University of Abuja Teaching Hospital, Abuja, Nigeria

Correspondence Address:
Dr. Joshua O Aiyekomogbon
Department of Radiology, University of Abuja, Abuja
Nigeria

Abstract

Conjoined twins result from incomplete division of embryonic cell mass in monozygotic, monochorionic, and monoamniotic twins occurring at very early stage of development. We present a set of male conjoined twins referred from a peripheral hospital to our health facility 18 h after cesarean birth on account of abnormal body fusion at the abdominal regions. Both babies passed meconium within 6 h of birth and were clinically stable. Radiological evaluation of the babies using ultrasound, echocardiography, and computed tomography showed hepatic fusion but their respective bowels and remaining viscera were separate and distinct for each of the twins. They were managed at the special baby care unit for 4 months to enable them achieve appreciable weight and clinical stability before having a successful surgical separation. They were discharged home following a good clinical outcome on the 23rd day of surgery as two separate babies.



How to cite this article:
Aiyekomogbon JO, Itanyi UD, Olori S, Chinda JY, Mshelbwala P, Leslie DM, Ekele B A. The role of radiology in the management of omphalopagus: Our experience at university of Abuja teaching hospital.Arch Int Surg 2019;9:116-119


How to cite this URL:
Aiyekomogbon JO, Itanyi UD, Olori S, Chinda JY, Mshelbwala P, Leslie DM, Ekele B A. The role of radiology in the management of omphalopagus: Our experience at university of Abuja teaching hospital. Arch Int Surg [serial online] 2019 [cited 2020 Nov 27 ];9:116-119
Available from: https://www.archintsurg.org/text.asp?2019/9/4/116/300555


Full Text



 Introduction



Conjoined twins results from incomplete division of embryonic cell mass in monozygotic, monochorionic, and monoamniotic twins occurring at 13–16 days gestational age (GA).[1],[2] It is seen in 1:200,000 pregnancies with female preponderance.[2],[3] The classification of conjoined twins is based on the site of anatomical fusion, and the order of occurrence is thoracopagus being the commonest, followed by omphalopagus, pygopagus, ischiopagus, craniopagus, parapagus, cephalopagus, and rachipagus.[4] Accurate preoperative radiological assessment is required for successful surgical separation, and the choice of radiological modality depends on the site of anatomical fusion.[5],[6]

Each set of conjoined twins is unique and meticulous radiological evaluation is required for surgical planning with the ultimate goal of good prognostic outcome for both or at least one of them. Imaging ascertains visceral involvement, associated anomaly particularly the heart.[6] The degree of cardiac fusion and severity of associated cardiac anomalies determine post natal viability.[6],[7],[8],[9],[10] This brings to the fore the usefulness of fetal echocardiography in all cases of conjoined twins. A case of conjoined twins with imaging diagnosis of hepatic fusion is presented, buttressing the pivotal role of radiologist in the diagnosis and management of these patients.

 Case Report



B babies were male twin neonates referred from a peripheral hospital to our health facility 18 h after birth through cesarean section on the account of abnormal body fusion at the abdominal regions. Both passed meconium within 6 h of birth and were not in any form of distress. They were fused from the xiphisternum to the region of the umbilicus with an omphalocele at the inferior aspect of the fusion. Bowel sounds and rectal examination were essentially normal for both twins, and the remaining systems were unremarkable. They had a combined weight of 5.15 kg.

Gray scale ultrasound scan with GE LOGIQ F8 Expert machine (2016), using high-frequency linear and low frequency curvilinear transducers showed fusion of the livers, but the biliary drainage, gall bladder, and porta hepatis were separate. Only some branches of the portal veins were seen linking the two livers [Figure 1]a and [Figure 1]b. A computed tomography (CT) scanogram following ingestion of gastrographin, and the 3-D reconstructed images using TOSHIBA ACTIVION 16 SLICE CT SCANNER (2014) confirmed fusion of the livers at the midline but the stomach, small and large intestines of the twins were demarcated by a radiolucent cleft [Figure 2] and [Figure 3]. The axial slices of the CT study [Figure 4] and [Figure 5] confirmed the above findings and showed better anatomical definition of the viscera. About 80% of the cranio-caudal dimensions of the livers were fused from the level of the falciform ligaments. The kidneys, spleen, pancreas, and great vessels of the babies' abdomen were well delineated and specific for each of the twins. Upper and lower gastrointestinal (GI) series were not done as the oral contrast administered during the CT study sufficed in this circumstance. The pericardium, hearts, lungs, and hemidiaphragms of the babies were also separate and specific for each of the twins [Figure 6].{Figure 1}{Figure 2}{Figure 3}{Figure 4}{Figure 5}{Figure 6}

The packed cell volume for babies A and B was 48% and 49%, respectively. They were managed at the special baby care unit (SCBU) for 4 months to achieve appreciable weight and clinical stability before surgical separation.

Intraoperative findings at 4 months of life revealed conjoint of the umbilicus and fusion of the liver at the level of falciform ligament. The xiphoid processes of the babies were also fused but they had separate biliary systems, intestines, great vessels of the abdomen, and remaining viscera. They were successfully separated and discharged on the 23rd day of surgery.

 Discussion



Conjoined twins are monozygotic, monoamniotic, and monochorionic, resulting from the failure of complete separation of embryonic plate between 13 and 17 days of gestation.[7] Ultrasonography is a valuable imaging modality employed for prenatal diagnosis of conjoined twins at 12 weeks gestation, a more specific evaluation of visceral fusion is possible from 20 weeks and should include fetal echocardiography as congenital heart disease is a common association with conjoined twins of any type.[6],[7],[8],[11],[12],[13] In the index case, antenatal diagnosis of omphalopagus was missed as the ultrasound scan was done at a peripheral hospital. The sonographic depiction of hepatic fusion of the omphalopagus twins was, however made post delivery at our health facility. Postnatal viability of the conjoined twins is greatly dependent on the degree of cardiac fusion and severity of associated cardiac anomalies.[6],[13] The index case of omphalopagus was not diagnosed antenatally and fetal echocardiography was therefore not done. This was largely due to the fact that their mother did not book early enough, and the only scan she has had was at 36 weeks of gestation when amniotic fluid volume is normally reduced and the fetuses were already big, making anatomical delineation relatively difficult. The diagnosis was missed in view of this, coupled with the fact that specialist radiologist did not attend to her all through the pregnancy.[7],[8],[9],[11] The diagnosis was made at cesarean delivery occasioned by prolonged labor and abnormal presentation. The twins were delivered and immediately referred to our facility for specialist care.

Ultrasound, echocardiography, CT, magnetic resonance imaging, angiography, upper and lower GI series, and intravenous urography are the required radiological investigations used in the evaluation of conjoined twins. The choice of the modality (ies) is/are largely dependent on the area of anatomical fusion. The index case benefitted from CT, echocardiography, ultrasound scan (USS), and Doppler examination as the fusion was at the umbilical and upper abdominal regions. Upper and lower GI series were not considered because the babies benefitted from CT scan which was done with oral contrast (gastrographin) administration. Also, CT scanogram sufficed for chest and plain abdominal X-rays. This consideration reduced logistics of unnecessary patients' handling, cost, and radiation dose.

Hepatic fusion is seen in over 80% of all cases of omphalopagus as noted in the index case.[12],[13] Also, the stomach and proximal intestines of omphalopagus twins are usually separate but terminal ileum and proximal colon may be fused in 30%[12],[13] but in the index case, the entire gastrointestinal tracts of the twins were distinct and specific for each of the twins. There were also no major vascular connections established between the twins, and the biliary systems of the twins were separate. The kidneys, ureters, pancreas, and remaining abdominal viscera were clearly demonstrated on CT and ultrasound evaluation, and found to be specific for each twin. This anatomic distinction between the abdominal viscera could also be made with gadolinium-enhanced MRI, angiography, percutaneous cholangiography and endoscopic retrograde cholangiopancreatography, but were not found necessary in the index case in view of the fact that CT and ultrasound which are more readily available were found satisfactory. Radiological diagnosis of omphalopagus with hepatic fusion was made and this was confirmed at surgery. The xiphoid processes were additionally found to be fused at surgery. This was not appreciated radiologically due to abnormal position of the babies which faced each other, making ideal positioning for both CT and USS procedures difficult. They were successfully separated and both are doing well as two different beings.

Radiological evaluation of omphalopagus twins which was missed prenatally has been presented, and this calls for first and second trimesters' anomaly USS of all pregnancies by specialists to avert the diagnostic pitfalls observed in this case.[11] Also, the needs to consider and maximize the usefulness of radiological modalities that are sensitive, cheap, and relatively safe in neonatal management are also re echoed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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