multidisciplinary care – Inside Pediatrics

An endoscopic approach for skull base conditions

Dr. Jessica Grayson (left) and Dr. James Johnston perform an endoscopic procedure on a patient at Children’s of Alabama.

A growing number of children with complex skull base conditions can now be treated with minimally invasive surgery at Children’s of Alabama thanks to a collaboration between pediatric neurosurgeon James M. Johnston, M.D. and otolaryngologist Jessica Grayson, M.D. Together, they lead an integrated pediatric skull base surgery program that offers endoscopic procedures for conditions such as skull base tumors, traumatic injuries, complex pituitary lesions, and congenital abnormalities such as encephaloceles—in which brain tissue protrudes through an opening in the skull.

“Endoscopic approaches have been part of pediatric neurosurgery here for years for things like hydrocephalus or intraventricular tumors,” said Johnston, director of the Division of Pediatric Neurosurgery at Children’s and the University of Alabama at Birmingham (UAB). “What’s new and exciting is how we’ve expanded endonasal skull base surgery through this collaboration.”

The procedure involves threading a tiny camera and instruments through the patient’s nasal passages to reach the brain. “That means smaller incisions, less blood loss and a much shorter recovery time,” said Grayson—one of the few clinicians in the country who is fellowship trained in rhinology and skull base surgery for both adults and children, with extensive expertise in endoscopic endonasal surgery.

After tumor removal, Grayson works to patch any small holes created between the brain and the nose. This is one of the most critical aspects after the removal—if the small holes aren’t properly sealed, cerebral spinal fluid could leak out into the nose, leading to a high risk of infection. Grayson typically uses a nasoseptal flap to close any openings. She peels a small piece of the mucosa covering the nasal septum while maintaining its blood supply, then flips it over to cover any holes created during surgery.

The program is multidisciplinary, involving ENT, neurosurgery and occasionally plastic surgery. The team-based model also allows for comprehensive case review and planning. “We often consult with our adult colleagues at UAB when a case is really complex,” Johnston said. “It’s like having a built-in tumor board.”

Offering this type of approach for children is another way the program is unique—this method typically has been reserved for adults. And “the technical aspects are different from adult cases,” given their smaller anatomy and less-developed sinuses, Johnston noted. “But with collaboration, it’s absolutely feasible. We’ve even done this in infants as young as a few months old.”

The first collaboration—a case of congenital encephalocele in which the protruding tissue was initially mistaken for adenoid tissue—highlighted the potential of combining expertise. “That was the moment we realized we could safely and effectively treat these cases together using a minimally invasive endoscopic approach,” Grayson said.

Nationally, this type of program is rare. “In many places, kids are sent to adult hospitals for these procedures,” Grayson said. “Here, they can stay in a pediatric environment with pediatric anesthesiologists, nurses and postoperative care, which is crucial for safety and comfort.”

Last year, the team did about 40 cases, and the number of referrals is growing as more clinicians become aware of what’s possible. “We’re seeing more cases from outside hospitals,” Johnston said. “And we’re better at recognizing which patients are good candidates.”

July 30, 2025 by childrensal

Endocrinology

A Multidisciplinary Approach to Metabolic Bone Disease

Drs. Margaret Marks (left) and Ambika Ashraf lead the metabolic bone disease clinic at Children’s of Alabama.

Treating metabolic bone disease in children involves a team of specialists including a pediatric endocrinologist, pediatric orthopedic surgeon, geneticist, physical medicine rehabilitation specialist and nutritionist. Where once patients and their families had to navigate this web of specialists, now the metabolic bone disease clinic at Children’s of Alabama assembles the entire care team, whom patients often see in one visit, thanks to a multidisciplinary approach.

“When we initially started out, we weren’t sure how many patients we’d have,” clinic director Ambika Ashraf, M.D., said. “Subsequently, we realized most of these patients were going out of state.” Today, the clinic follows more than 300 patients. Most are from Alabama, but patients also travel from Tennessee, Mississippi and Georgia.

Since most of the conditions the clinic sees are complex and require multidisciplinary care, “getting to see the different specialists on the same day is a huge benefit,” Ashraf said. Otherwise, it could take six to eight months to get an appointment with individual specialists.

Patients have a varied spectrum of conditions including osteogenesis imperfecta; fibrous dysplasia; complex disorders of calcium, phosphorous and vitamin D metabolism; fragility fractures due to low bone density and osteoporosis; hypophosphatasia; and skeletal dysplasias.

Pediatric metabolic bone disease spans a spectrum from mild disease with a relatively low risk of fractures to disease so severe that just a small bump could result in a broken bone. Despite treatment, patients tend to be small in stature for their age, with multiple deformities resulting from fractures and poor healing, Ashraf said. They are also prone to problems in other areas, including cardiovascular and pulmonary complications. “We make sure they see those specialists, too,” she said.

The most common condition the clinic treats is osteogenesis imperfecta (OI), or brittle bone disease, a genetic defect that affects the body’s ability to make collagen, which is required for strong bones. These children may have dozens or even hundreds of fractures before they reach adolescence.Most patients with OI receive bisphosphonate infusions in the Children’s infusion center to strengthen their bones.

Physical medicine, or physiatry, plays an important part in managing these children, Ashraf said, because many have some type of abnormality related to muscle tone or movement, joint laxity, joint contractures or muscle weakness. They may also need help with a wheelchair, braces/splints or other mobility devices.

“This is a fascinating time for metabolic bone disease,” Ashraf said. Just a decade ago, there were few treatments beyond the supportive and palliative. For instance, until a few years ago, the only treatments for X-linked hypophosphatemic rickets were oral phosphate and calcitriol. They helped, but not enough, and patients still required frequent surgeries. With the availability of burosumab, a monoclonal antibody that binds to and inhibits the activity of fibroblast growth factor 23—which blocks phosphate absorption—children with the condition now need fewer surgeries and experience fewer limb deformities.

Bisphosphonate infusions help reduce the number and severity of fractures in OI patients, and physical therapy can help with deformities. For hypophosphatasia, enzyme replacement helps manage the condition. Caring for these children “is a joy,” Ashraf said. “Especially when we can make a difference in their quality of life.”

January 16, 2023 by childrensal