Ed. note: This is an update of a post that originally appeared in 2014.
The neural tube is supposed to close during the first month of prenatal development, forming the spinal cord and the brain. In children with spina bifida, it doesn’t close completely, leaving the nerves of the spinal cord exposed and subject to damage. The most common and serious form of spina bifida, myelomeningocele, sets a child up for lifelong disability, causing complications such as hydrocephalus, leg paralysis, and loss of bladder and bowel control.
A growing body of research from Boston Children’s Hospital, though still in animal models, suggests that spina bifida could be repaired at least partially early in pregnancy, through intrauterine injections of a baby’s own cells. …
Tissue expanders — small balloons that can be filled with saline solution or other fluids to grow skin — have long been used in plastic surgery, most commonly breast reconstruction. They’re based on the simple idea that the surrounding skin will stretch as the device expands over time. That extra skin can then help repair injuries or congenital anomalies or accommodate implants.
Midaortic syndrome occurs when the middle section of the aorta is narrowed and typically affects children and young adults. It can cause severe hypertension and can be life-threatening if left untreated. The surgical approach to this condition would be to replace the damaged portion of the aorta with nearby healthy blood vessels. However, this usually isn’t possible because these vessels tend to be too short to adequately fill in. …
By the time he arrived at Boston Children’s Hospital, the 6-month-old boy was near death from midaortic syndrome — a rare but life-threatening condition marked by narrowing of the middle section of the aorta, the largest artery in the body. It had left him with severe hypertension, acute kidney injury and heart failure. As cardiologists worked to stabilize him, the surgical team weighed the options.
With diminished blood flow to the chest, abdomen and lower limbs, a significant number of people with untreated midaortic syndrome die from complications by age 40. The condition can be treated surgically, traditionally with a prosthetic graft made from synthetic material to perform an aortic bypass. But synthetic grafts can pose a number of challenges in children.
“Synthetic grafts don’t grow with the patient, which means that multiple surgeries may be necessary through the years to ensure appropriate graft size,” explains nephrologist Michael Ferguson, MD, who was a member of this patient’s care team. “Artificial grafts also carry a higher risk of thrombosis and infection.” …
Placed near the heart, the device can potentially predict life-threatening cardiac arrest in critically ill heart patients, according to tests in animal models. The technology was developed through a collaboration between Boston Children’s Hospital and device maker Pendar Technologies (Cambridge, Mass.).
“With current technologies, we cannot predict when a patient’s heart will stop,” says John Kheir, MD, of Boston Children’s Heart Center, who co-led the study. “We can examine heart function on the echocardiogram and measure blood pressure, but until the last second, the heart can compensate quite well for low oxygen conditions. Once cardiac arrest occurs, its consequences can be life-long, even when patients recover.”
In critically ill patients with compromised circulation or breathing, oxygen delivery is often impaired. The new device measures, in real time, whether enough oxygen is reaching the mitochondria, the organelles that provide cells with energy. …
A 4-year-old has a progressively enlarging head and loss of developmental milestones: a clear case of hydrocephalus. He undergoes a minimally invasive endoscopic third ventriculostomy (ETV) to drain off the trapped cerebrospinal fluid.
This requires puncturing the floor of the brain’s third ventricle (fluid-filled cavity) with an endoscope — while avoiding a lethal tear in the basilar artery, which lies perilously close.
There are no good neurosurgical training models for this rare and scary operation.
“We semi-blindly poke a hole through the ventricle floor,” says Benjamin Warf, MD, director of Neonatal and Congenital Anomaly Neurosurgery at Boston Children’s Hospital. “To make the technique safer and to be able to train more people, it would be very helpful to make that hole in a way that’s less anxiety-provoking.” …
For a tissue graft to survive in the body — whether it’s a surgical graft or bioengineered tissue — it needs to be nourished by blood vessels, and these vessels must connect with the recipient’s circulation. While scientists know how to generate blood vessels for engineered tissue, efforts to get them to connect with the recipient’s vessels have mostly failed.
“Surgeons will tell you that when putting tissue in a new location in the body, the small blood vessels don’t connect at the new site,” says Juan Melero-Martin, PhD, a researcher in Cardiac Surgery in Boston Children’s Hospital. “If you want to engineer a tissue replacement, you’d better understand how the vessels get connected, because if the vessels go, the graft goes.”
Melero-Martin and colleagues have uncovered several strategies to help these connections form, as they describe online today in Nature Biomedical Engineering. The strategies could help improve the success of such procedures as heart patching, bone grafting, fat transplants and islet transplantation. …
At five months’ gestation, Bentley Yoder was given little chance to live. A routine 20-week “gender reveal” ultrasound showed that a large portion of his brain was growing outside of his skull, a malformation known as an encephalocele. But he was moving and kicking and had a strong heartbeat, so his parents, Sierra and Dustin, carried on with the pregnancy.
Born through a normal vaginal delivery (the doctors felt that a C-section would interfere with Sierra’s grieving process), Bentley surprised everyone by thriving and meeting most of his baby milestones.
But the large protuberance on his head was holding him back. It steadily got larger, filling with cerebrospinal fluid. Bentley couldn’t hold his head up for more than a few seconds. …
Nearly 100 years ago, William Ladd, MD, of Boston Children’s Hospital, helped establish pediatric surgery as a medical subspecialty. The recognition that children require unique surgical management hasn’t changed, but the instruments and procedures we use to operate on children have evolved dramatically. Here’s a glimpse of the surgical state of the art then and now.
The 1920s marked the earliest use of scrub attire. White gowns, white masks and white linens emphasized the importance of cleanliness — and perhaps compensated for the dim lighting. Chloroform and ether, dating back before the Civil War, were the anesthetics of the day. Though penicillin was discovered in 1928, antibiotics were still two decades away from actual use. Imaging was limited to X-rays. It was in this setting that pediatric surgery began to evolve.
Today’s operations are increasingly more precise and less invasive. Surgeons can practice on custom 3-D models of patients’ anatomy, take an MRI scan mid-operation to ensure accuracy and (at least in animals) repair a still-beating heart with a patch delivered through a vein. “GPS” systems are guiding surgeons to deep lesions through the smallest possible incisions, lasers are replacing scalpels and robots are handling complex moves. Above, surgeons operate on a child with spasticity, opening a small window in his spine and carefully stimulating each nerve before deciding which to cut.
Four children with life-threatening malformations of blood vessels in the brain appear to be the first to benefit from 3D printing of their anatomy before undergoing high-risk corrective procedures.
The children, ranging from 2 months to 16 years old, all posed particular treatment challenges: cerebrovascular disease often entails complex tangles of vessels in sensitive brain areas.
“These children had unique anatomy with deep vessels that were very tricky to operate on,” says Boston Children’s neurosurgeon Edward Smith, MD, senior author of the paper and co-director of the hospital’s Cerebrovascular Surgery and Interventions Center. “The 3D-printed models allowed us to rehearse the cases beforehand and reduce operative risk as much as we could. You can physically hold the 3D models, view them from different angles, practice the operation with real instruments and get tactile feedback.” …
Although global health has come a long way over the past 25 years, access to surgical care remains very uneven across the world. Five billion people lack access to basic surgical care; this translates into unnecessary death and disability. More than one-third of all global deaths are from conditions requiring surgical care—more than the number of deaths from HIV/AIDs, tuberculosis and malaria combined. In addition, one-quarter of the world’s disability has been attributed to surgically treatable conditions.
In January 2014, an international team of 25 surgeons and public health experts launched The Lancet Commission on Global Surgery to address the widespread need for surgical care around the world. After 14 months of global consultation and four international meetings, the commission published a 32,000- word report today in TheLancet that provides a strategy for governments, policy makers, non-profits, funding agencies, academic institutions, professional associations, health care providers and local communities to engage in concrete action in low- and middle-income countries.
On May 6, the commission hosts its North American launch in Boston to present its key findings and priority action items. John G. Meara, MD, DMD, MBA, Plastic Surgeon-in Chief at Boston Children’s Hospital and the Kletjian Professor of Global Surgery at Harvard Medical School, is one of three chairs of the commission. We sat down with Meara to learn more about the commission’s work, which he describes as one of the “most impactful things he has done in his career to date.” …