Leah’s Mystery

By Greg Raver-Lampman 

A CT scan revealed a 4-centimeter mass in Leah's ear canal.
A CT scan revealed a 4-centimeter mass in Leah's ear canal.

Two weeks after her birth in September 2007, Leah Hannon came down with a condition so perplexing it stymied her doctors, including specialists at MCV. Eventually, one doctor told the baby’s desperate mom, “You need to go to CHKD.”

The Hannons, who live just south of Richmond, first noticed something wrong with their baby girl two weeks after she was born: A small white skin tag poking out of her ear. Two days later, an ear-nose-and-throat specialist clipped it out. It grew back the next day.

The doctor sent Leah to another ENT, who took a biopsy that wasn’t conclusive. A CT scan, however, revealed a 4-centimeter mass in Leah’s ear canal. It had destroyed the part of the skull beneath Leah’s ear and grew into her brain, pushing it to the side. The Hannons were referred to an oncologist at MCV, who ruled out cancer.

“They told me it wasn’t cancer,” said Tara, Leah’s mother. “But I was worried because it kept growing.” MCV ENT residents took another piece of the growth and sent it to the pathologist, but those results also proved inconclusive.

On the advice of their Richmond doctors, the Hannons decided to take their daughter to CHKD.

At CHKD, Tara and her husband, Mark, met a phalanx of specialists, including neurosurgeon Joe Dilustro and otolaryngologist Stephanie Moody, whose specialty focuses on diseases that afflict the ears, temporal bone and skull base.

“The doctors at CHKD were the first ones who really stepped up and had a game plan,” Tara said. “They said ‘this is what we need to do.’”

To get a precise diagnosis, they needed a biopsy of the tissue from deep inside the growth. Because previous pathology reports were inconclusive, surgeons sent samples directly from CHKD’s operating room to a waiting pathologist to make sure they had sufficient tissue.

What they discovered was an extremely rare condition called “giant cell reparative granuloma.” Doctors describe it as a wildly out-of-proportion immune response.

“You might find 10 or 12 documented cases where an adult or a child has a similar lesion in this location, but never has it been reported in a three-month-old baby,” Dr. Moody said.

Granulomas are masses formed by globs of sticky white blood cells, often occurring in places where there has been some sort of trauma. Dubbed multinucleated giant cells, this type of white blood cell carries an acid that dissolves bone. Nobody knows exactly what causes granulomas, which usually grow on the jaw.

In Leah’s case, the growth destroyed her mastoid bone, the layer of skull beneath and behind her ear. The size and rate of growth suggested to Dr. Moody that Leah’s mass started growing before she was born.

Richmond family travels to CHKD for answers and a cure.
Richmond family travels to CHKD for answers and a cure.

Because it was unprecedented in newborns, Dr. Moody conducted extensive research to find the best possible treatments. When she didn’t get answers from journal articles, she tracked down experts around the nation.

“I must have called 10 different physicians to ask whether they’ve seen anything like this,” Dr. Moody said. Included was one of the foremost pediatric ENTs in the country, who practices in Pittsburgh.

“He’s about 80 years old and has been practicing otolaryngology for 50 years. He said he’s never seen anything like this before,” Dr. Moody said. “In fact, none of the doctors had seen a case like Leah’s.”

After discussing all their findings, CHKD doctors and surgeons opted to begin treatment with steroids, both intravenous and injected, partly because of the danger of brain surgery on such a small child.

“Dr. Dilustro explained that the dura – the membrane that covers the brain – is the consistency of wet paper at Leah’s age,” Tara said. With each passing week, he told her, the brain’s sheathing would strengthen.

Over several weeks, the steroid injections shrunk Leah’s mass slightly, but decreased her ability to fight infections. An abscess formed in the spaces left by the shrinking granuloma. It had to be drained repeatedly. Moreover, Leah started to feel worse.

Finally, on January 4, doctors decided to operate.

Drs. Dilustro and Moody took turns removing the tissue, meticulously scraping it away from the dura, a process Dr. Moody compared to “scraping mush off a pancake.”

The mass had grown deep into her ear canal. The facial nerve was exposed because its protective bone covering had been dissolved by the granuloma. If the nerve was nicked by a scalpel – or even stretched – it could paralyze half of Leah’s face.

Electrodes placed around Leah’s eyes and mouth detected micro-movements of her face muscles, sounding alarms when the surgeons got close to the nerve.

The growth also descended into Leah’s upper neck. Over eight hours, Drs. Moody and Dilustro were able to remove 80 percent. They hoped that amount might be enough.

“When you remove a large part of a granuloma, it can suffer a kind of shock, and the body can absorb it,” said Dr. Moody.

In the months that followed, Leah had multiple MRIs. The results thrilled Tara. Leah was a different baby, happy and always smiling. The residual granuloma withered away and the eardrum that doctors feared had been destroyed reappeared. Segments of the destroyed skull have started to grow back.

For the Hannons, Leah’s case demonstrated the tenacity of CHKD doctors and their ability to absorb massive amounts of research to develop the best possible treatment plan. Leah’s treatment team included otolaryngologists, neurosurgeons, oncologists, anesthesiologists and radiologists.

Doctors are now composing a manuscript about the case for medical journals. While such publications omit names of patients, surgeons around the world will learn treatment techniques from the unique case of Leah Hannon at CHKD.

Dr. Dilustro is a neurosurgeon with Children’s Neurosurgery, a practice of CHKD Health System. Dr. Moody practices with EVMS at CHKD.

This story was featured in the fourth quarter 2008 issue of KidStuff, a publication of Children's Hospital of The King's Daughters.