Brachial Plexus Palsy-There Is Hope and There Is Help

January 15, 2001
By KATHLEEN CHARTER

What do five of every 1,000 live births in the United States have in common? An injury to the brachial plexus - a group of nerves in the neck and shoulder area that control movement of the shoulders, arms and hands.

The C5, 6, 7, 8 and T1 nerves that make up the brachial plexus are part of the spinal cord. As each of these nerves branches off, gradually growing finer and finer, an injury at any point along this network could spell mobility problems.

There are currently less than 10 hospitals and major clinics in the United States that are able to provide care for brachial plexus injuries, each with a different approach to solving this dilemma. The Texas Medical Center is privileged to have two of them. Baylor College of Medicine supports programs at both The Institute for Rehabilitation and Research and Texas Children's Hospital where families from all over the world come seeking treatment for their children. TIRR is on an even shorter list. Of the few hospitals and clinics that offer Brachial Plexus Palsy programs, it is one of the few that is primarily a rehabilitation hospital.

A sampling of some of the United States cities from which patients have come seeking treatment include, Anderson, S.C., St. Louis, Mo., Swansboro, N.C., Tulsa, and Waco, and some of the foreign countries that patients have come from include Saudi Arabia, Poland and Mexico.

Damian Nowakowski, an 18-month-old boy from Radom, Poland was recently a brachial plexus patient at TIRR. Damian weighed 10 pounds at birth, and incurred a brachial plexus injury. His family found out about Baylor's program from another Polish family who had the same procedure performed at TIRR. They had a good experience and went back to their homeland to share the good news. In the case of Polish citizens coming to the Texas Medical Center seeking treatment, a Polish radio station in Houston announces needs that these families have, and the Houston Polish community rallies to provide housing, an interpreter, and whatever else they may need during their stay.

Dr. Saleh Shenaq, chief of reconstructive plastic surgery at Baylor College of Medicine and chief of surgery at TIRR, said there are three types of brachial plexus injuries, caused by birth trauma. First is the stretch, also known as a Stinger or a Burner. It can vary in intensity, and occurs when the shoulder gets caught on the pelvic bone triggering the plexus nerves to swell and bruise. Ninety percent of these injuries will spontaneously recover on their own with no need for surgical intervention. But that does not mean that these patients won't have any minor deficits, or need surgery. Sometimes a neuroma, scar tissue around the nerve that interferes with the nerve's ability to conduct, may need to be scraped away in a procedure called a neurolysis.

Occupational therapist Nancy Conte, OT assistant manager at TIRR, said, "I've worked in newborn nurseries in acute care settings where we would get OT referrals, in which all cases have to be seen within 24 hours. By the time that I would see the patient, the problem was resolved. They were that minor."

The second type of injury is a rupture, where nerves are torn in one or several places along the nerve network. The third injury type is an avulsion, which is the most serious. Nerves are pulled from the spinal cord, causing the extremities to be flaccid. These last two injury types require surgical intervention.

Conte said patients with brachial plexus injuries will usually present themselves with the arm down against the trunk of the body, the shoulder rotated inward, the elbow straight, and the wrist flexed, commonly referred to as the "waiter's tip".

There are two surgeries to repair a brachial plexus injury. The primary surgery is most commonly performed on children 12-months-old or younger, with the ideal age for this procedure at six-to-eight months. This first surgery is done to repair the nerve. A surgical team performs an average of three of these surgeries per day, each lasting six-to-eight hours. The Baylor team at TIRR consists of Drs. Shenaq and Rahul Nath, plastic surgeons, Dr. John Laurent, a neurosurgeon, and Dr. Maureen Nelson, a physiatrist. They explore the plexus to see the exact location of the injury, the level and severity of the injury, test nerve conduction to see muscle response, and consult with the parents on a plan of action, and then act on it.

Sometimes during this microneuro repair, a nerve graft is needed. Dr. Shenaq said tissue is taken from the sural nerve, a long nerve that gives sensation on the lateral side of the foot.

"The nerve graft is needed to reach muscles and skin and grow across the injury site. The graft acts as a conduit, similar to a fiber-optic telephone cord."

With a brachial plexus injury, early diagnosis is crucial. Dr. Shenaq said that ideally, the best time to repair nerves is within a week of the injury. But here is where it gets tricky. Since the best way to repair the body is by letting nature take its course, surgeons want to see if a brachial plexus injury will be one of the 90 percent that repairs itself. But waiting too long may result in muscle damage.

"With the primary surgery, if you wait too long, you run the risk of muscle atrophy, where the muscles get tight and short and lose their ability to contract and relax," Conte said. "If the surgery is not done by the 12 month benchmark, the muscles won't be as functional."

In addition to that, since there are so few health care facilities that recognize this injury, a child may not get the proper care needed in an expedient fashion. Conte said there is quite an emotional element on the part of the family, and that many parents get the wrong information about appropriate and available treatment. They are told that there is nothing that can be done, the child will grow out of it, or that nothing can be done until the child is older.

"It is hard to diagnose this injury to this nerve complex that you can't really pinpoint it easily," Conte said. "So many families come to us frustrated-so extremely tearful and frustrated. They have been misinformed, often by their pediatrician or other specialists in their hometowns, and if they are not familiar with these types of injuries they may not know how to help. And even if they are familiar, they just don't know what help is available out there."

Conte said that when families find out about the programs at TIRR and Texas Children's, they feel excitement and enthusiasm, knowing that their prayers have been answered, and their child will get the help they have been longing for.

The secondary surgery is primarily a plastic surgery. It is performed on the muscles of patients that are 13 months and older, many that have had muscle atrophy, and muscle and tendon transfer or transplantation may be in order. As with the primary surgery, the younger the patient, the more successful the outcome. However, many of the younger children who have the primary surgery have had such a successful outcome, that there is no need for this secondary surgery.

The recovery rates for the patients who have a brachial plexus surgery vary with the degree of the initial injury. Regardless of the outcome, there will always be some degree of functional benefit.

"Recovery is not all or none. It could be partial," said Dr. Shenaq.

The most common type of brachial plexus injury is classified as Erb's Palsy, occurring when there is a condition called Shoulder Dystocia. The birth is typically a head delivery, and the shoulder gets stuck.

"Approximately 60 to 65 percent of all brachial plexus injuries are classified as Erb's Palsy," said Conte. "It becomes a medical emergency. They have to be pretty aggressive about getting the baby out. Erb's Palsy affects just C5 and 6, and children who have it often have use of their hands, however, there isn't a lot that they can do with their hand if their arm is stuck at their side."

The post-surgical therapy that the children experience is just as important as the surgeries themselves. Conte said that it is important to counteract the body posture caused by a brachial plexus injury. After the primary surgery, the arm is immobilized with a soft foam rubber splint, day and night, for four weeks. It is removed once per day for inspection and care of the incision, and bathing, maintaining the splinted position when the immobilizer is off. Home therapy is performed for four weeks, and includes scar massage and range of motion exercises of the elbow, forearm, wrist and fingers, but not the shoulder.

After the secondary surgery, the arm is typically splinted at a 150-degree angle, with the inside of the elbow pointed to the ear. This ensures that the child's arm is properly externally rotated. The splint is worn day and night for six weeks, removing once per day for inspection and care of the incision, and bathing, maintaining the splinted position when the immobilizer is off. Home therapy is performed for six weeks, and includes scar massage and range of motion exercises of the elbow, forearm, wrist and fingers. Shoulder range of motion exercises typically begin in week seven, and children practice movements that take the arm away from the trunk, and externally rotating the shoulder. Conte recommends things like taking hats on and off and putting hula-hoops over the head.

"The two most common types of actions that I recommend are the jumping jack motion and putting the hand out to the side of the body with the elbow bent at a 90-degree angle, similar to the way a policeman would stop traffic," said Conte. "I tell parents to involve their kids in activities like spritzing a window and having the child help wipe it down, or drawing with shaving cream or bath foam on the bathtub wall, or putting stickers as high as they can on a wall."

Conte said that post-surgical follow up is very important, as well.

"Since most families are from far away, we request that they send us a video three months after the procedure. We ask them to show us the range and strength of the shoulder with both front and back views."

The self-satisfaction that Conte receives from her job is great. She gets all kinds of cards, letters and pictures from former patients. A 10-year-old girl who had surgery several years ago sent Conte a roll of film showing things that she is now able to do on her own.

"It is so exciting to see. I get teary all the time. It's nice when we hear from the older kids who tell us, `I can play basketball better,' or `I can wash my own hair.' It's not always a perfect outcome, but you can see the glow on their faces."

Parental networks for those who have had the experience of a child with Brachial Plexus Palsy are essential. Much of it is Internet-based, and there are many chat sites for parents seeking information about these injuries.

"The Internet enhances parents' abilities to communicate with each other and connect," says Conte. "If they have a good experience, the whole world knows about it. If something went wrong, again, the whole world knows."

Based on the need for networking, a weekend summer family camp was put together by some of the parents who have had children with Brachial Plexus Palsy. They are scheduled to happen every other year, and two have taken place, so far. The camps have ready-made cabins, restrooms with toilets and showers, and kitchen facilities, as well as traditional camp activities like arts and crafts and swimming. Conte said although the two camps that have been held were both in Texas, families came from as far away as Washington, New York, and Florida.

"The Baylor team was invited to participate in panel question and answer sessions. Other invited participants have been aquatherapists who have done demonstrations, psychologists, and basically any professionals with information to share. It is great to see these families interacting with one another, and watching the children interact with their parents and siblings as they discover that there are other families out there like their own," Conte said.

Source: Texas Medical Center

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