1. Success of Surgeries Results in Medical History "Firsts"

As Clarence and Carl rest and evidence rapid recoveries from the historic surgery at The Children's Hospital at Montefiore that resulted in their separation on August 4, doctors say both boys continue to show every sign of being "neurologically intact". 

"I would have to say that what we are seeing in terms of their rapid recovery and outcomes is a medical miracle," said James T. Goodrich, MD, the boys' pediatric neurosurgeon.  Dr. Goodrich worked for more than a year with his partner and the boys' lead pediatric plastic surgeon, David Staffenberg, MD, to map out and execute the successful separation strategy at CHAM. "Within days after the separation, both boys were awake, eyes alert, breathing on their own, watching TV and sitting up in separate beds," said Dr. Goodrich. 

"All other recorded craniopagus separation surgeries in history have required immediate post-op visits to the operating room to repair serious brain vessel leaks, infections or hydrocephalous," said Dr. Staffenberg.  "Carl and Clarence came out of the OR after this last surgery and have not looked back. Our decision to stage the separation surgeries over several months, and give the boys the time they needed to heal and grow stronger, has made all the difference."   

"From the beginning, our goal has been not only to successfully separate Carl and Clarence but to give them the best chance to live viable, independent lives," said Spencer Foreman, MD, president, Montefiore Medical Center.  "We have now accomplished a huge step toward that goal."

While the short-term recovery of the boys is unprecedented, the larger goal of having both boys live to be healthy, normal adults also has never been accomplished in the 500 years of recorded craniopagus twin separations.  While the Montefiore surgeons are cautious about attaining this goal, they are hopeful and committed to the challenge.  

The results from the series of four operations at CHAM also has made surgical history. 

A Short History of Craniopagus Twins

• 1495 – oldest known published case of craniopagus twins occurs in Worms in Palatinate, Germany 
• 1505 – first recorded surgical separation of craniopagus twins in a German publication; one died pre-operatively, the other lived for three days.
• 1928 – first case of a separation in the United States (Cameron). Both twins died.
• 1953 – first successful operation where one lived (Grossman-Sugar)
• 1957 – first double survival (Voris) with considerable medical complications.
• 2004 – first double separation without need for immediate additional surgery (Goodrich & Staffenberg) and first ever with no immediate signs of neurological impairment or deficit (Goodrich & Staffenberg).

Incidence of Craniopagus Twins

• 1:200,000 births result in conjoined twins (twins can be joined at the chest, abdomen, sacrum, pelvis, face, head or back).
• 1:10,000,000 births result in craniopagus (head-to-head) twins  

Survival

• 78% of craniopagus twins die by the time they reach one year of age.
• 90% of craniopagus twins die by the time they reach ten years of age.
• Survival is low because if one twin dies, the other dies within minutes because of unrestricted blood flow into the deceased twin from the live one.  

Terminology

• “Craniopagus” is derived from the Greek: kranio = head; pagus = together.  The term was first used in 1865 by Forster

Key Support for "Team Aguirre" at Montefiore

The highly specialized and skilled teams of surgeons, anesthesiologists, other physician specialists, nurses and therapists at Montefiore Medical Center and Blythedale have been the fundamental reason for, and the brains and hearts behind, the success of the separation. Dr. Goodrich and Dr. Staffenberg point to the following factors as uniquely contributing to the boys’ successful surgery.   

• Staged Operations – the boys had four operations over a ten-month period, allowing CHAM surgeons to gradually assess the boys' health after each procedure and to proceed to the next stage only if the boys were fit. The staged operations resulted in:
  • Enabling one twin, Carl, to slowly and steadily "plump up" his existing network of veins needed to drain blood from his brain. Both boys initially shared a group of small veins as well as the main vein that drains the brain, called the sagittal sinus. During the final separation surgery, when surgeons assigned the sagittal sinus to Clarence, Carl had in place increased capacity in his own network of veins to take up the role of a sagittal sinus.
  • Relatively small amount of blood loss.  Approximately 1½ “volumes of blood”, a measure used by surgeons, was lost in total by both twins during the four surgeries.  Recent marathon surgeries for other twins’ separations have seen as much as 6 body volumes of blood loss in a single procedure.
  • Reduced time in the operating room.  The boys overall time in the operating room, despite having four procedures, was less than the time in the OR for all recent marathon, single-procedure separation surgeries.      
• Rotation of the Boys Heads – the boys’ heads were attached with only a 15% rotation off center.  They were almost mirror images of each other.  This is called an O’Connell Type 1.  Most craniopagus twins’ heads are attached at a 30% rotation or more.  Traditionally, the less the rotation, the better the outcome. 
•  Technological Advances – an unprecedented combination of advanced anatomical models, holographic imaging and blood volume technology (some of this technology was used for the first time ever in this procedure) provided an exquisitely detailed roadmap for surgeons so they could assess the twins’ blood vessels and skull surgery  progress before, after and in between each of the four operations. 
  •  Anatomical Models – Before and after each operation, translucent, plastic replica models of the boys’ conjoined heads, showing details of the blood vessels inside and surrounding the brains, were created using CT and MRI imaging.  As more of the network of veins was cut, these tactile models allowed for “hands-on” surgical planning and even special surgical instrument development for the next procedure. These were created by Medical Modeling Inc. Clearview Anatomical Models developed the stereolithography process to translate CT and MRI images into useable, tactile models. 
  • Holographic Imaging – prior to and after each surgery, holographic images provided surgeons with the most accurate  3-D representation of the boys’ blood vessels and skulls.  The holograms, viewed on a monitor-like screen, were produced using CT and MRI two dimensional scans and are products of Voxel, Inc.  
  • Blood Volume Supply – during the ten months of surgery, surgeons received blood volume analysis updates using an advanced blood volume analyzer that measures the input and output of blood supply, gas, electrolytes and chemistry to the boys’ brains, providing essential data for their decisions to proceed with surgery. The technology is produced by Nova Biomedical.  

© 2012 Montefiore Medical Center