Conjoined Twins

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Total Cases Seen
Cases of Conjoined Twins in Saudi Arabia
The Multidisciplinary Team Approach

TOTAL CASES SEEN: 48 SETS

Nationality of Cases:

Country	Number of Cases
Saudi Arabia	27
Sudan	6
Yemen	2
Egypt	2
Malaysia	1
Philippines	2
Polish	1
Morocco	2
Iraq	2
Cameroon	1
Oman	1
Syria	1

Conjoined Type:

Type	Number of Cases
Thoracopagus	17
Omphalopagus	5
Thoraco-ompalo-ischio-pagus	17
Ischiopagus	3
Parasiticus	4
Pyopagus	1
Craniopagus	1

Management of Conjoined Twins

•	Twins successfully separated:	13
•	Twins evaluated but not separated:	20
•	Twins attempt at separation:	1

OPERATED CASES: 19

	Type	Shared Organs	Outcome
1	Omphalopagus	Liver, skin, muscles	Both survived
2	Xipho-omphalo-ischio-pagus tripus	Liver, small and large bowel, one urinary bladder, one anus and rectum, one pelvis	Both survived
3	Xipho-omphalo-ischio- pagus tripus	Liver, small and large bowel, one urinary bladder, one atretic anal canal, one pelvis	Both survived
4	Ischiopagus tripus	Liver, small and large bowel, one rectum and anus, one pelvis, two communicating bladders, congenital heart disease in one twin	Both survived(One twin with major heart problems died 6 months later)
5	Omphalopagus	Liver, pericardium, small bowel	Both survived
6	Thoraco-omphalo-ischio-pagus tripus	Liver, small and large bowel, one anus, one pelvis	Both survived
7	Omphalopagus	Liver, diaphragm	Both survived
8	Abdominal heteropagus		survived
9	Caudal duplication		survived
10	Omphalo-ischio-pagus tetrapus	Urogenital, large bowel, pelvis	Both survived
11	Pyopagus tetrapus	One rectum, lower spine and sacrum, lower spinal column and crossing nerves	Both survived
12	Omphalopagus tetrapus	Common pericardium, fused liver, CBD and pancreatic duct, pancreas, duodenum, jejunum, omphalocele, CHD	Both survived
13	Ischiopagus tetrapus	One anus and rectum, genitourinary system, one bladder, spinal column	Both survived
14	Thoraco-omphalo-pagus tripus	Liver, small and large bowel, GU, pelvis and anus	Both survived
15	Thoraco-omphaloischio-pagus tripus with exstrophy of cloaca	Liver, bowel, GU, one abnormal phallus, one anus, pelvis	Both survived
16	Caudal duplication	Lower limbs and pelvis	Survived
17	Thoraco-omphalo-pagus tripus	Liver, small and large bowel genito-urinary system	Both survived
18	Craniopagus	Skull, meninges, crossing veins	Both survived
19	Thoraco-omphalopagus tetrapus	Shared liver and bowel omphalocele	Both survived
20	Thoraco-omphalopagus tetrapus	Shared liver, bowel and pericardium	Awaiting separation

Non Operated Cases: 28

Rationale for non-operative approach:

Single heart
Major communicating hearts
Major anomalies

	Type	Shared Organs	Outcome
1	Thoracopagus tetrapus	Single heart, VSD, ASD, pulmonary hypoplasia, shared liver	Died at 10 days
2	Thoracopagus tetrapus	Communicating hearts at ventricles with major anomalies and shared liver	Died at 5 days
3	Brachothoraco-omphalopagus bipus	Single heart with large VSD and TGA, shared liver and bowel	Died at 12 days
4	Thoraco-omphalopagus tetrapus	Single deformed heart, shared liver, omphalocele	Died at 1 hour
5	Thoracopagus tetrapus	Communicating hearts, one heart TOF, the other TGA	Died at 16 days
6	Brachothoraco-omphalopagus bipus	Single heart with VSD, hypoplastic LV, pulmonary hypoplasia, shared bowel, liver and GU	Died at 5 days
7	Thoracopagus tetrapus	Single deformed heart, liver and bowel	Stillborn
8	Brachothoraco-omphalopagus bipus	Communicating hearts at ventricular level, three chambered heart in one and four chambered heart in the other with LV hypoplasia, VSD, ASD, shared liver and bowel	Died at 16 days
9	Thoracopagus tetrapus	Communicating hearts at ventricular level, VSD in one, TGA the other, respiratory distress	Died at 5 days
10	Thoraco- omphaloischiopagus tripus	Communicating abnormal hearts, liver, large bowel,pelvis and genitourinary system	Died at 8 days
11	Thoraco-omphalopagus tetrapus	Single deformed heart, liver	Died at 22 days
12	Brachothoraco-omphalopagus bipus	Single heart, VSD, LV hypoplasia, liver, bowel, GU	Died at 5 days
13	Thoracopagus tetrapus	Communicating hearts at atrial level, one heart VSD, TOF and the other TGA, liver	Died at 25 days
14	Thoraco-omphalopagus tetrapus	Single heart three chambered, the other RV hypoplasia VSD, ASD, severe respiratory distress, liver	Died at 3 days
15	Bracho-omphalopagus bipus	Single heart, LV hypoplasia, VSD, liver, bowel, GU	Died at 15 days
16	Cephalothoraco-omphalopagus tetrapus	Many major anomalies	Stillborn
17	Thoraco-omphaloischio- pagus tripus	One heart VSD, ASD and the other RV hypoplasia VSD, severe pulmonary hypoplasia, liver, bowel, GU and pelvis	Died at 1 hour
18	Thoracopagus tetrapus	Communicating hearts at atrial level, one heart three chambered with hypolastic ventricle and the other TOF	Died at 17 days
19	Thoraco-omphalopagus tetrapus	Liver, small and large bowel, major CHD	Died at 10 days
20	Thoraco-omphalopagus tetrapus	Communicating hearts at the ventricles, major anomalies in the heart, shared liver and bowel	Died at 6 days
21	Thoracopagus	Communicating abnormal hearts, fused liver	Died 1st day
22	Thoraco-omphaloischio pagus bipus	Abnormal hearts, fused liver, small and large bowel	Died 2nd day
23	Thoraco-omphalopagus	Fused chest, abdomen, single heart, shared liver and bowel	Aborted
24	Cephalothoraco-omphaloischiopagus	Single heart, fused face, chest, abdomen and pelvis, fused liver and bowel	Stillborn
25	Thoraco-omphalo ischiopagus tetrapus	Fused chest, abdomen, pelvis, shared liver and bowel, single abnormal heart	Aborted
26	Thoracopagus tetrapus	Communicating abnormal hearts, shared liver and other anomalies	Aborted
27	Thoraco-omphalopagus tetrapus	Single abnormal heart, shared liver and bowel, omphalocele	Aborted
28	Thoraco-omphaloischio pagus tripus	Communicating abnormal hearts, multiple anomalies	Aborted

Attempted Cases: 1

Craniopagus parasiticus:
Findings -

Complex case
Major vascular communications
Involvement of meninges and brain

Case aborted

Antenatal diagnosis in 28
Delivery – Cesarean Section in 29
One team experience

THE MULTIDISCIPLINARY TEAM APPROACH

Prior to Surgery:

Extensive medical work-up on patients
Multiple meetings and discussions
Involvement of parents
Psychosocial counseling of parents
Rehearsal of the planned surgical procedure
Media contact

Goals of the Operative Team:

Assemble a surgical team for each stage of the operation
Create a detailed written operative plan
Minimize the use of tissue expanders
Avoid prosthetic material (meshes) when possible
Keep blood loss low
Control traffic and media in the operating room

Post-Operative Procedures:

Continue team management
Begin separation-trauma management
Physiotherapy for the separated twins
Rehabilitation of the separated twins