Medical Miracle in Indianola, Mississippi: The Untold Story of Dr. Clinton Battle and the Indianola Conjoined / Matthews Siamese Twins

Conjoined twins are very rare. The overall survival rate has been estimated to be approximately 25% to 50%. We report a case of conjoined twins delivered in Indianola, Mississippi in 1955 who made medical history. The Matthews’ conjoined twins were the fi rst in history to be separated and both survive. They were delivered in rural Mississippi at home by vaginal delivery by Dr. Clinton Battle, a 29-year-old Meharry School of Medicine graduate. The Matthews twins are now 61 years old (retired school teachers) and their 92-year-old mother, Missouri Matthews, are alive and well living in Mississippi. Case Report


Introduction
Conjoined twins, a rare phenomenon in which identical twins are joined in utero, are estimated to occur in the range of 1 in 50,000 births to 1 in 200,000 births, with a somewhat higher incidence in Southwest Asia and Africa [1]. Approximately half are stillborn, whereas a smaller percentage of couplets are born alive but have abnormalities incompatible with life [2,3]. The overall survival rate for conjoined twins has been estimated to be approximately 25% to 50% [4]. The condition has a higher female preponderance with a ratio of 3:1 [2,3].
The earliest known documented case of conjoined twins, also referred to as Siamese twins, dates back to the year 942 when a pair of conjoined twin brothers from Armenia were brought to Constantinople for medical evaluation [5]. The most famous pair of conjoined twins was Chang and Eng Bunker (1811-1874), Thai brothers born in Siam, now Thailand. They travelled with P.T. Barnum's circus for many years and were referred to as the Siamese Twins. Thus, the term "Siamese twins" came to be used synonymously for conjoined twins, due to the brothers' fame and the rarity of the condition during that time [5].
Although Siamese twins share a single common placenta, chorion and amniotic sac, these characteristics are not limited to conjoined twins. In actuality, there are some monozygotic but non-conjoined twins that also share these structures in utero [3]. Two contradicting theories have been traditionally put forth to explain the origins of conjoined twins. The older supposition is fi ssion theory, which asserts that the fertilized egg splits partially. The second and more commonly accepted viewpoint is the fusion theory, in which a fertilized egg completely separates. However, stem cells appear to fuse with similar cells on the other twin and connect the fetuses together.
In a recent study detailing the twenty-year history at a reference center in Brazil, the authors shared the experience of one hospital regarding the surgical aspects, anatomic investigation and outcome of the management of 21 conjoined twin pairs over the past 20 years. All cases of conjoined twins who were treated during this period were reviewed. There were eight sets of ischiopagus twins, seven sets of thoracopagus twins, three sets of omphalopagus twins, two sets of thoracoomphalo-ischiopagus twins and one set of craniopagus twins. Nine pairs (43%) of these conjoined twins could not be separated secondary to the complexity of the organs (mainly the liver and heart) that were shared by both twins; these pairs included one set of ischiopagus twins, six sets of thoracopagus twins and one set of thoraco-omphalo-ischiopagus twins.
Twelve sets (57%) were separated, including seven sets of ischiopagus twins, three sets of omphalopagus twins, one set of thoracopagus twins and one set of craniopagus conjoined twins. The surgical survival rate was 66.7% [6]. In short, each set of conjoined twins is unique. An imaging strategy to accurately defi ne anatomic fusion, vascular anomalies, and other associated abnormalities, is important for surgical planning and prognostic information [7]. Timing of operation and separation plan should be given according to the circumstances and the nature of the organ shared in each individual set of twins [8]. Based on its organsharing classifi cation system, there are 7 major types of conjoined twins, with thoracopagus (thorax) being the most common type [7]. We present the case of one of the earliest known successful separation of conjoined twin as well as report the unheralded story of Dr. Clinton Battle, the physician who delivered them on that fateful day in September 1955 [9].

Discussion
Conjoined twins, also referred to as Siamese twins, are very rare congenital malformations [8]. They occur in approximately one in 50,000 or so live births [1]. The majority do not survive long-term [20]. Timing of operation and separation plan should be given according to the circumstances and the nature of the organ shared in each individual set of twins [8]. Therefore, a careful imaging evaluation must be performed to detail the abdominal anatomy (particularly the liver), inferior vena cava, spleen and pancreas, to identify the number of organs and to evaluate the degree of organ sharing. The importance of a multidisciplinary approach with extensive investigations required pre-operatively in order to defi ne areas of organ and bony conjunction, other congenital anomalies of each twin and surgical teamwork cannot be over-emphasized [12]. The timing of separation has been ideally set at between 5 and 9 months with 6 to 8 weeks of prior tissue expansion but earlier operation has frequently been required because of cardiorespiratory problems or organ failure in one of the twins. In most cases, the goal of obtaining separate, independent and intact individuals is achievable [20,21]. as conjunction is usually fatal, due to their association with major congenital defects. The greater the extent of thoracic cage fusion, the greater the chance of associated severe anomaly.
Ischiopagus and pygopagus conjoined twins, on the other hand, manifest an interesting array of spinal abnormalities, which present challenges, not only at the time of separation, but also in their long-term management [22]. Pygopagus conjoined twins are joined at the rump and have union of the gastrointestinal and genitourinary systems in addition to the occasional involvement of neural elements [22].
In a study of 22 conjoined twin managed from 1974 to 2006 in the Philippines, it was elucidated that emergency separations yielded dismal results due to poor patient conditions; delay in separation allowed progressive deterioration and resultant poor outcome. However, elective separation had more favorable results due to well-planned strategies, team preparedness, and better patient conditions. A multi-disciplinary approach, with parental participation, was also found to be integral in the holistic management of conjoined twins, with both radiologist and pediatric surgeons playing key roles [23].
Magnetic resonance imaging and computed tomography provide excellent anatomic and bone detail, demonstrating organ position, shared viscera, and limited vascular anatomy.
Contrast material radiography allows evaluation of the gastrointestinal and urogenital tracts, and a shared liver requires assessment of anatomy, vascularization, and biliary drainage. Angiography helps defi ne specifi c vascular supply, which is useful in determining the distribution of shared structures between the twins at surgery [7].

Conclusion
Improved recent survival is probably the result of the availability of more accurate imaging studies and better anesthetic and operative techniques with greater emphasis on performing immediate reconstruction whenever possible.
Use of skin expanders and prosthetic mesh has facilitated wound closure. In the future, ex vivo cardiac reconstruction and autotransplantation may permit separation of twins with complicated conjoined hearts.