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 Table of Contents  
ORIGINAL ARTICLE
Year : 2018  |  Volume : 15  |  Issue : 1  |  Page : 92-97

Management of conjoined twins in Kano, Nigeria: our experience and challenges in a low-resource setting


1 Department of Surgery, Bayero University/Aminu Kano Teaching Hospital, Kano, Nigeria
2 Department of Anaesthesia, Bayero University/Aminu Kano Teaching Hospital, Kano, Nigeria
3 Department of Surgery, Murtala Mohammad Specialist Hospital, Kano, Nigeria
4 Department of Paeditrics, Bayero University/Aminu Kano Teaching Hospital, Kano, Nigeria
5 Department of Radiology, Bayero University/Aminu Kano Teaching Hospital, Kano, Nigeria

Date of Web Publication23-Mar-2018

Correspondence Address:
Dr. Mohammad Aminu Mohammad
Paediatric Surgery Unit, Department of Surgery, Bayero University/Aminu Kano Teaching Hospital, Kano
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/njbcs.njbcs_16_16

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  Abstract 


Introduction: Conjoined twins are abnormalities of twinning in which two individuals are incompletely separated. Conjoined twins can be symmetric or asymmetric and continue to raise significant ethical and legal arguments, resulting in much pressure on the managing teams and hospitals. Patients and Methods: We report 4 cases of conjoined twins, an ischiophagus dipus, pygophagus, thoracoomphalophagus tetrapus, and a parasitic twin tripus. All cases had no prenatal diagnosis and were delivered via spontaneous vaginal delivery. The pygophagus tetrapus was separated and a mortality of 50% was recorded in the immediate post separation period; the surviving twin is 8 years old and doing well. Two were lost due to severe birth trauma, and the last was lost to overwhelming from an infected gluteal congenital defect. Results and Discussion: Conjoined twins are one of the most fascinating congenital malformations not only to the medical practitioners but to communities and general public also. To our knowledge, as of 2012, only 15 cases have been reported from Nigeria. The incidence is 1:50,000–1:100,000 births. Females are affected more frequently than males by a ratio of 3:1. Separation of conjoined twins is a complicated procedure. The importance of a multidisciplinary team approach with several rehearsals of all aspects (surgical, anesthetic intensive care, transfer from the theatre to intensive care unit, and nursing) of the operative procedure cannot be overemphasized. Conclusion: Conjoined twins are one of the most fascinating congenital malformations. Early prenatal diagnosis, antenatal care, choice of mode of delivery, thorough assessment of the extent of shared organs to guide decisions on surgical separation, adequate planning, and rehearsals can reduce morbidity and mortality in these patients. In our series, all pregnancies and deliveries were unsupervised leading to poor outcome.

Keywords: Conjoin, parasitic, pygopagus, separation, twins


How to cite this article:
Mohammad MA, Anyanwu LJC, Abdullahi LB, Liadi S, Aji SA, Atiku M, Ismail H, Ibrahim M, Abdussalam M, Danbatta HA, Yahuza M. Management of conjoined twins in Kano, Nigeria: our experience and challenges in a low-resource setting. Niger J Basic Clin Sci 2018;15:92-7

How to cite this URL:
Mohammad MA, Anyanwu LJC, Abdullahi LB, Liadi S, Aji SA, Atiku M, Ismail H, Ibrahim M, Abdussalam M, Danbatta HA, Yahuza M. Management of conjoined twins in Kano, Nigeria: our experience and challenges in a low-resource setting. Niger J Basic Clin Sci [serial online] 2018 [cited 2021 Jun 16];15:92-7. Available from: https://www.njbcs.net/text.asp?2018/15/1/92/228352




  Introduction Top


Conjoined twins are abnormalities of twinning in which two individuals are incompletely separated.[1],[2],[3] Conjoined twins can be symmetric or asymmetric. They continue to raise significant ethical and legal arguments among physicians, lawyers, judges, politicians, lay people, and of recent, press and media houses.[2],[3] This puts a lot of pressure on the managing teams and hospitals in addition to the complexity of the abnormality and planning of their treatment.[1],[4]

This report is intended to highlight the challenges faced in the management of conjoined twins and propose recommendations to minimize the effect of these challenges in a low-resource setting.


  Patients and Methods Top


From February 2006 to March 2008, 4 sets of conjoined twins were managed at our hospital. Their hospitals records have been retrospectively reviewed.


  Results Top


[Table 1] shows the details of the patient's presentations, management, and outcomes. The separated cases are discussed in details to demonstrate the logistic difficulties in planning separation in our setting. The first set of babies were pygopagus tetrapus conjoined twins [Figure 1], delivered to a peasant farmer by a 28-year-old Para 4 + 2; 5 alive, full-time house wife at 36 weeks of gestation. She neither had antenatal care nor prenatal ultrasonography. Delivery was via spontaneous vaginal delivery at home supervised by a traditional birth attendant. The presentations of the first and second babies were cephalic and breach, respectively. The babies were referred to the State Specialist Hospital and later to our center 96 hours after delivery. The babies had a combined weight of 3.6 kg [Figure 2],[Figure 3],[Figure 4].
Table 1: Patient's presentation, management and outcomes summary

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Figure 1: Ischiophagus dipus conjoined twin

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Figure 2: Pygopagus conjoin twins at admission

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Figure 3: Perineum pygopagus conjoin twins showing common vestibule

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Figure 4: Pygopagus conjoin twins at the time of seperation

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They were generally in a stable condition and tolerated breast feeding and supplementation elemental diet. They passed meconeum 6 hours after birth and continued to have normal defecation. Morphological examination revealed two female babies joined together at the sacrum with each having a separate head, trunk, and two lower limbs. Perineal examination showed a vestibule with 2 vaginal introitus and a single anal opening below the vestibule.

Plain X-ray showed a U-shaped spine joined at the sacrum. Abdominal ultrasound scan showed two normal kidneys and a urinary bladder in each baby. Cardiac echo revealed no abnormality. Intravenous urogram showed functional kidneys with delayed excretion in the second twin. The lower parts of the ureters could not be adequately defined. Barium enema showed single rectum and anal canal with separate sigmoid colon and other parts of the intestine. All hematological investigations were essentially normal.

The babies were admitted and nutritionally optimized for 6 weeks during which their combined weight increased steadily from 3.6 kg to 11.4 kg. The 6-week period allowed the babies to physiologically stabilize and provided an opportunity for the surgical stakeholders to prepare for the surgical separation of the babies [Figure 4].

Two joint meetings between peditric surgeons and the anesthesiologists/intensivists, urologists, theatre, intensive care, and pediatric surgical ward nursing staff were held during which the procedure and the role of each team was defined. The surgical separation was started at 08.00 am. With a divided sigmoid colostomy, other major findings were common rectum with both sigmoid colons forming a Y shape [Figure 5].
Figure 5: Showing the shared common rectum and anal canal

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The rectum was allocated to the first twin in whom the anal canal was present in the perineum. Appropriate anal and perineal reconstruction was carried out. The right ureter of the second twin emptied in to the bladder of the first twin which was re-implanted in to the corresponding urinary bladder. the spine was joined at the terminal part of the sacrum which was cartilaginous. The median sacral vessels of the two babies were wider and were the major circulatory communication between them. Following the completion of the separation procedure, pull-through and anal and perineal reconstruction was done. Both babies were transpired to intensive care unit. The first twin had an uneventful postoperative recovery, except for a superficial surgical site infection. The postoperative period of the second twin was characterized by low urine output, bradycardia, trachypnoea, and steadily falling oxygen saturation despite oxygen therapy. She was sedated and ventilated. She had two cardiac arrests at 30-minute intervals, and resuscitation having been unsuccessful after the second arrest she was certified dead 8 hours after separation of the twins. The first twin is now 8 years old in class 3 and doing well [Figure 6] and [Figure 7]. Case 2-4.9 [Figure 8],[Figure 9],[Figure 10],[Figure 11],[Figure 12] were summarized in [Table 1].
Figure 6: Survivin twin at the age of 6 month

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Figure 7: Surviving twin at the age of 8 years

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Figure 8: Thoraco-omphalopagus conjoin twins

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Figure 9: Autopsy specimen of the thoraco-omphalopagus conjoin twins showing the shared organs.

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Figure 10: Parasitic twin

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Figure 11: Septic defect at the sacral area

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Figure 12: Plain X-ray of the parasitic twin

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The average mother's age was 21.5 years, an average gestational age of 35.5 weeks, and presented at an average of 41.75 hours. The three mortalities occurred with an average of 31.3 hours after admission, while the second twin in the separated pair died within 8 hours after the surgery.


  Discussion Top


Conjoined twins are one of the most fascinating congenital malformations not only to the medical practitioners but to communities and general public also.[5] Up to 2012, only 15 cases from Nigeria could be identified in the English literature.[6] This does not reflect the true incidence in Nigeria as most of the cases are not reported on account of being stillborn and the fear of associated stigma attached to the delivery of deformed babies. Cases of conjoined twins seen in our hospitals are rare and pose a management challenge when they present because of paucity of large scale experience in managing them.[6] This may be the largest case series of conjoined twins reported from Nigeria. Literature abounds with confusing terminologies and classifications of conjoined twins; thoracopagus: joined at the thoracic level; the most common type accounting for 40% of the cases; omphalopagus: joined at the abdomen often including the lower thorax; accounting for 32% of the cases; others are pyopagus-sacral fusion; ischiopagus and craniopagus: Joined at the head.[7],[8],[9] In this case series, we had three symmetrical (cases 1, 2, and 3) and one asymmetrical (case 4) with all the different complexities associated with the malformation.

We also had an opportunity to go through the process of planning and separating one of them. Surgical separation of conjoined twins is one of the most stressful procedures in terms of planning and actual performance of the procedure. The anxiety and pressure from the hospital management and the staff, the parents, the press, the public, as well as the medicolegal implications of every step taken in the planning, surgical operation, and the care of these patients is great. All these in addition to the ethical considerations in terms of obtaining ascents, consent and prognostication of the outcome of surgical separation are enormous. Having a set of conjoined twins in a unit is enough to disrupt the day to day schedules of the unit in a low-resource setting like ours in terms of manpower and resources and other patients are bound to suffer some delay.

Separation of conjoined twins is a complicated procedure. The importance of a multidisciplinary team approach with several rehearsal of all aspects (surgical, anesthetic intensive care, transfer from the theatre to intensive care unit, and nursing) of the operative procedure cannot be overemphasized.[6],[8],[9],[10],[11],[12] Although the outcome is influenced by careful planning and organization from all participants, the prognosis is often predetermined by the underlying anatomy which may preclude successful separation.[4] The decision for surgical separation of conjoin twins is determined based on the complex nature of the shared organs and general wellbeing one or both of the twins. In general, they are categorized as follows.[5]

  • Group 1, Nonoperative treatment, owing to cardiac fusion or gross anatomical union, case number 3 belongs to this category
  • Group 2, Those requiring emergency separation when one of the twins died or is threatening the survival of the other sibling. As in our cases number 1 and 3 both twins were severely ill at presentation that there was not enough time for adequate resuscitation before their demise
  • Group 3, Those who are stable and can be planned for elective separation, allowing time for detailed preoperative investigation and planning. Nutritional rehabilitation and treatment of infection if any. Our case number 2 belongs to this category. They were optimized and planned for elective separation.


There are many gray areas in the management of conjoined twins such as who has the consent authority, what is the acceptable operative risks, the postoperative quality of life, and the question as to whether we are dealing with one or two human beings. In the opinion of most authorities, conjoined twins with one head and parasitic twins are considered to be one person. Furthermore, conjoined twins with one heart may also fall into this category. It is also believed that the operative mortality should not exceed 50–60% and that parental consent is essential. In the only set we separated the second twin died, this is a mortality of 50%, which is mainly due to the possibility that the second twin may have immature organs and the other was doing most of the physiological functions. Therefore, after separation, it became independent and incapable of maintaining homeostasis as evidenced by the inadequate urine output poor oxygen saturation, tachypnea, and possibly respiratory distress syndrome.[10]

Early prenatal diagnosis may also change the concept of the management of conjoined twins. There are several reports showing that prenatal diagnosis of conjoined twins can be achieved as early as 10–12 weeks of gestation.[5],[10] The legality of early abortion for conjoined twins varies from one country to another. Experts opinion believed that certain prerequisites are essential in the approval for termination of conjoined twins pregnancy, such as confirmed diagnosis by two independent teams; the presence of major anomalies (which may endanger the life of the twins such as in group 1); and lastly, the safety of the mother.[10]

Anesthesia is another serious challenge in the management of conjoined twins, which can either be given for separation surgery or for magnetic resonance imaging or other evaluation procedures. It is an enormous challenge to the pediatric anesthesiologist.[11] The anesthesiologists need to care for 2 patients at the same time and on the same table instead of one. Anesthetic consideration for conjoined twin's separation surgery is centered on the following: (1) Organ maturity and development, occasionally one twin may be more physiologically active than the other hence problems may start from the intraoperative period. (2) Physiological concerns such as crossed circulation, distribution of blood volume, and organ sharing with their anesthetic implications. (3) Long duration surgery with massive fluid shifts and loss of blood and blood components and their rapid replenishment. (4) Meticulous planning for organized management of long hours of anesthetic administration in two pediatric cases simultaneously with multiple surgical specialties' involvement and their unique requirements.[11],[12] In our case, the space constraint was also a challenge; their were two anesthetic teams and their gadgets, two surgical teams, and all other operating room staff all centered around the infants.

Antenatal care is essential and should be encouraged, especially in the low-resource countries, through public enlightenment, girl child education, and prenatal ultrasound scan. All pregnant women, especially in suspected multiple pregnancies, should undergo sonography to enable early diagnosis of gross fetal malformation and to enable the obstetrician to counsel the family and plan the mode of delivery. In our cases, only one of the mothers had some form of antenatal care and none had prenatal ultrasound scanning and severe birth trauma from spontaneous vaginal delivery may have contributed to the immediate high mortality in two of our cases.

Recommendations

Antenatal care and prenatal ultrasound scanning should be done for all pregnant women, especially in suspected twin pregnancies. All pregnant women whose fetuses are found to be abnormal, especially conjoined twins, should be referred to tertiary health facility were skilled obstetric, neonatal, and pediatric surgical services are readily available to decide the mode of delivery and prompt care of the delivered conjoined twins. Accurate planning, preoperative investigations, multidisciplinary approach, and meticulous operative and postoperative management are essential for the successful management of conjoined twins.


  Conclusion Top


Conjoined twins or double monsters have always been a subject of curiosity for lawyers, judges, lay people, politicians, and of recent, the press and media houses. These cases emphasize the need for antenatal care with prenatal ultrasound monitoring of high-risk pregnancies to determine the nature of the perinatal management required. When serious malformations that are incompatible with postnatal life are diagnosed early enough, the family may opt for terminating the pregnancy. Decision to undertake the rigors of surgical separation may be determined by the extent of vital organs shared. Success of surgical separation depends on dedication, multidisciplinary approach, and careful selection of patients after thorough evaluation and long-term experience in the management, which is generally rare.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Acknowledgments

The authors appreciate the contribution of the resident doctors and staff of the Radiology, Pediatric surgery and Anesthesia department, special baby care unit of Aminu Kano Teaching Hospital, in the management of these patients.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

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Hirayama Y, Kubota M, Kakita A, Kawasaki T, Hasegawa G, Tanaka S, et al. Sacral parasite with histopathological features of an unequally conjoined twin. Pediatr Surg Int 2007;23:715-20.  Back to cited text no. 3
    
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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12]
 
 
    Tables

  [Table 1]


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