Giving a hand

A disabling orthopedic condition called Erb’s palsy caused – rarely – during childbirth is becoming even rarer due to better medical teaching, methods and awareness.

November 3, 2013 00:57
Pregnant women

Pregnant women [illustrative]_311. (photo credit: Reuters)

Thanks to major medical discoveries, technological innovation and improved doctor training, many serious and even devastating conditions are now much less common than they were only a decade or so ago. For example, permanent damage from strokes has been minimized; the birth and even conception of severely defective fetuses have been prevented; and clogged coronary arteries are cleaned up to reduce damage and deaths from heart attacks.

Another condition, which is little known to the public and even many physicians, used to cause temporary or even permanent disability among a significant number of newborns around the world, including in Israel.

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Erb’s syndrome – which can occur as a result of brachial plexus injuries – fortunately is much less common than it used to be. But the handful of affected newborns today must get the rehabilitation they need to reduce their disabilities and even ensure normal functioning.

“Brachial” refers to the arm, while “plexus” (from the the Latin for “braid”) is a branching network of nerves or blood vessels. The nerves are typically peripheral ones outside the central nervous system. Erb’s (or Erb- Duchenne) palsy is a paralysis of the arm caused by injury to the upper group of the arm’s major nerves, specifically the severing of the upper-trunk C5 and C6 nerves.

In 1861, Guillaume Benjamin Amand Duchenne gave a name to the condition “obstetric palsy of the brachial plexus” after examining four babies suffering from paralysis of identical muscles in the arm and shoulder.

The syndrome was also named for Dr. Wilhelm Heinrich Erb, a German neurologist who lived from 1840 to 1921 and used electricity to diagnose and treat nervous disorders. He studied adult brachial plexus injuries.

Older children and adults can suffer from this condition as a result of road accidents and other trauma to the head and shoulder, when the nerves of the plexus are violently stretched.

Yet brachial plexus injuries arise most commonly from shoulder dystocia during difficult birth. Shoulder dystocia may occur when a baby’s head is delivered, but the shoulder that is in the frontal position can’t pass through because the baby’s chin presses against the walls of the perineum.

If a baby is stuck, obviously it’s an obstetrical emergency, and in extremely rare cases, the infant can die due to lack of oxygen because of pressure on the umbilical cord. If the baby survives, there may be temporary paralysis that disappears on its own or with physical and occupational therapy. But in a small minority of cases, physical disability remains and even surgery is required.

An Israeli expert on brachial plexus, Prof. Shlomo Weintroub is a pediatric orthopedist at the Dana Hospital of Tel Aviv Sourasky Medical Center.

“The general public don’t know about brachial plexus or Erb’s palsy at all,” he says. “It occurs [today] much less frequently than before, and often if it’s a big baby, if the mother suffers from regular or gestational diabetes or has a narrow pelvis and the fetus can’t easily get out vaginally.”

“But better technology in delivery rooms, more knowledge and awareness among midwives, obstetricians and ambulance medics and other improvements have significantly reduced the number of cases we see and the number of children with permanent damage.

This has created a revolution in recent years, and there are only a few such children nationwide each year.”

Nevertheless, parents around the world whose children were not saved from devastating injury pour out their hearts in blogs where they seek advice and comfort.

ONE ISRAELI woman, A.D., writes that she has not one, but two children with Erb’s palsy. “FR is not my first child with it. My oldest son, now nine years old, was also born in a traumatic birth and his arm was affected.

Only then, no one told me the truth about what happened at his birth, and they still won’t. His folder in the hospital doesn’t even say ‘shoulder dystocia.’ “But on the second, while putting him down in his cot, I saw his arm was limp. I freaked out thinking maybe I put him down too roughly.”

She recalled that she rushed to the hospital nurses’ station, but “they brushed it off, saying it was ‘probably from the birth, nothing to worry about, definitely not my fault,’ and sent me to speak to the pediatrician before we were discharged.

“He informed me that it was nerve damage from being born, nothing major really, and that it would resolve within a few weeks. And to go see a physical therapist. I really didn’t worry about it.”

In her second birth, 18 months later, her son suffered from shoulder dystocia but it resolved itself very quickly with no lasting damage.

“At that point, I was told a C-section might be preferred but I thought – what’s the big deal?” she wrote.

“Erb’s Palsy, I was told, is a condition that is temporary and resolves itself. When my 3rd child was born and I told the hospital I was not having the C-section, they were worried but she was born just fine. With the knowledge I had at the time, I saw no reason to even worry about something happening at birth, since all was fine, wasn’t it?”

She continued: “Along came FR three years ago. He was a really big baby. My doctor pulled him out, and it was Erb’s palsy again. It was pretty traumatic; my son was totally white and floppy when born... I asked the physiotherapist how long it would take until he would be normal. I’ll never forget the sinking realization, as time went by, that it was going to take forever – a lifetime affected by brachial plexus injury, a child’s life forever changed because of the lack of information among doctors and midwives out there.”

Another woman – a licensed physical therapy assistant abroad – said she has trouble keeping a regular job because she has to be available to take her daughter to her various treatments. “It breaks my heart to see the crooked arm, with an abnormal size, that will never be better.”

Due to her injury, her daughter has no reflexes in her right arm. “This means that as she was learning how to ride her bike, she once lost control and fell flat on her face. Because she did not have a face-mask helmet, she landed directly on her mouth. That was terrifying for her and for us, and we quickly bought her a full-face helmet.”

After her first surgery, as feeling started to return to her right hand, the girl began biting her right index finger. “She was still too young to talk, but the doctor explained that it would be the same feeling of your foot ‘falling asleep.’ We all know how painful and annoying that is, but she didn’t know how to respond so she began biting that finger. To the point that she had a chunk of flesh gone from it. I had to start putting a tube sock over her right arm, then using masking tape to wrap it on there so she couldn’t remove it and couldn’t keep biting her finger,” the mother recalled.

A woman from California described her 12-year-old daughter who was born with the injury but is today an “accomplished intermediate synchronized swimmer.

She was just chosen for the emerging athletes program for swimming in the US Paralympics and will be training for 2020. She is able to cope with her Erb’s palsy even though she is unable to do what most human beings take for granted on a daily basis.”

Yet another woman writing in the blog said that her second child had the disability for a week after delivery, but “nothing was explained to me except that it ‘will get better.’ By his two-week checkup it was. But I wasn’t informed and did no research. Now my forth baby girl has it. It could have been prevented as all warning signs were there; gestational diabetes, my major weight gain during pregnancy and a history of large babies.

“Natalie’s arm woke up at three months, but now at six months, her arm is still not 100 percent. She has movement but not like a healthy baby. Now when I see a pregnant woman, I talk to her and tell her my story and explain how it can be prevented.”

WEINTROUB NOTED that midwives, obstetricians and MDA ambulance personnel are much better trained and informed on prevention of brachial plexus injury today.

Cesarean sections – which in normal cases are not preferable to vaginal births – are the delivery of choice if it seems likely that delivering the baby will be difficult.

“And most babies that are born with it need conservative treatment – physiotherapy and occupational therapy – rather than surgery. With better physical exams and imaging, it’s much better for children who do not recover on their own.”

A number of Israeli medical centers have expert microsurgeons who work on peripheral nerves that were damaged during birth, he continued. It used to be thought that severed or otherwise damaged peripheral nerves cannot be repaired. But they can grow back and be fixed; the central nervous system, however, still cannot be repaired.

“I don’t think brachial plexus injuries can be completely eliminated. It will always be sporadic, but with increased awareness during the last trimester of pregnancy and the proper type of delivery, the cases will really be reduced,” said Weintroub. “There are biotech companies working on techniques to encourage peripheral nerves to grow, but if it works at all, this will take many years to succeed.”

DR. SHIRLEY MEYER, a pediatric orthopedic surgeon by training and a former director-general of Jerusalem’s Alyn Pediatric Rehabilitation Center where she continues to practice medicine, says that it receives an average of four new cases of brachial plexus injury children each year. This is in addition to older youngsters who come from around the country to get rehabilitation.

“The number of cesareans has shot up in the past decade. It’s not good to do a cesarean as an elective procedure, but if a woman has trouble delivering vaginally or if there are clear problems in her case, a surgical delivery is preferable. Doctors and midwives are more liberal on this than they used to be. Instead of using a forceps or vacuum delivery, they will do a cesarean.”

Asked whether there are more cases at specific hospitals – which would indicate a problem – Meyer said it is sporadic. When asked whether there are more when babies are delivered in an ambulance or elsewhere when the woman has not yet reached a hospital, she said no.

“When they don’t reach the hospital in time, it usually is because it is sudden and the baby just slips out.”

Alyn does not perform any surgery but sends young patients to nearby Hadassah University Medical Centers or Shaare Zedek Medical Center to do this. “If there is evidence of a tear in the nerves of the plexus, they can be repaired by surgery around six weeks. Early surgery encourages regeneration,” said Meyer.

“When it’s an older child who suffers from residual injury at the ages of three, four, five or six, we send them for surgery to transfer muscles and even for cutting the bone and rotating the limb to its proper position.

But about half of these are left with some kind and degree of permanent injury.”

DR. DANNY WEIGL, director of the orthopedics rehabilitation clinic at the Schneider Children’s Medical Center in Petah Tikva, trained in Miami and specializes in surgery for the condition.

“Neurosurgery on the nerves never completely restores functioning, so they need follow-up in orthopedics.

To improve the results of physiotherapy, we often give Botox injections to reduce the tonus of internal rotator muscles. This relaxes them for a few months, providing a window of opportunity to repair some muscles. We do it with orthopedics and physiotherapy, it really helps.

“Beyond the age of three or four years, if the limb remains weak, we often add orthopedic surgery and transfer ligaments and muscles.”

His hospital currently has about 25 children under treatment, with about five or six new cases a year.

“About 85 percent of our children fully recover, higher than in Alyn because they may get the most serious patients,” Weigl said.

He urges parents not to be afraid of surgery as early as possible.

“When babies improve under physiotherapy, some parents try to delay surgery, thinking that will make it all better. But this often means the surgery will come too late, and without it, there will be deformities.”

Another problem has nothing to do with medical treatments but is social and educational. “These children often suffer from bullying and ridicule from classmates, because they raise their arms awkwardly from the shoulder and find it hard to play ball or put on their coats. They try to avoid participating in gym classes. Healthy children should become used to being with disabled children so they accept them,” he concluded.

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