Cleft hand

At Children’s Hospital Boston, our orthopedic experts know that it’s distressing to learn that your baby has a cleft hand. We’ve pioneered innovative surgical treatments for children with all types and variations of cleft hand.

What is cleft hand?

Cleft hand is a rare congenital (meaning your baby was born with it) birth defect in which the hand didn’t develop properly during fetal development. This causes the affected hand to have missing fingers, a V-shaped cleft and other deformities.

What causes cleft hand?

There’s a clear genetic basis for typical cleft hands. Inheritance of cleft hand is autosomal dominant: This means that if a parent has the condition, the child has a 50 percent chance of having it, too.

Cleft hand can occur in isolation or as part of a genetic syndrome, such as cleft lip and palate or ectrodactyly (split hand-split foot malformation).

How common is cleft hand?
Cleft hand is very rare. It affects between one in 10,000 and one in 90,000 babies. An isolated cleft hand (in which there’s no associated clinical syndrome or systemic illness) accounts for fewer than 5 percent of all congenital hand conditions.

How will having a cleft hand affect my child?
It really depends on the severity of the problem, and the severity of the problem is usually tied to how much or little function your child’s hand has. Some children can grasp, pinch and release with just a mild degree of cleft hand, while others experience an extreme lack of hand function.

Does cleft hand cause my baby pain?
No. Typically, a child doesn’t experience pain as a result of cleft hand.

What are the signs and symptoms of cleft hand?

In a cleft hand, there are always clefts in the central (middle fingers) part of the hand, and they’re usually V-shaped. However, clefts can also occur on the thumb (radial) side of the hand, or, less commonly, on the little (ulnar) finger side. They can also occur in various combinations.

How is a cleft hand diagnosed?

Cleft hand develops between the 28^th and 56^th day of fetal development, when the bones of the hand are forming. The condition can sometimes be detected on a routine prenatal ultrasound. After the baby is born, the deformity is visible.

Details of the diagnosis are usually confirmed through a physical exam and x-rays. Your child’s doctor will closely evaluate every anatomic structure of the hand, determining what is present or absent, normal or abnormal. The doctor will take particular care to assess the soft tissue specifics of the hand, such as muscles, ligaments, blood vessels and nerves.

In diagnosing the condition, your child’s doctor will likely classify it as one of five types—with Type 1 the mildest and Type 5 the most severe.

For details about these classifications, see Tests.

Can cleft hand be associated with other conditions?
In the majority of children who have it, a cleft hand may be an isolated occurrence, affecting only her hand. But your doctor will check for other associated deformities or syndromes, including:

• cleft lip and palate
• foot abnormalities
• encephalocele (protrusion of brain membrane)
• conditions affecting the heart and digestive systems
• (rarely) deafness

How is cleft hand treated, and at what age?

In all but mild cases of cleft hand, the condition is treated surgically, usually when a child is 1 to 2 years old. There are several surgical approaches that surgeons can take, but all surgeries aim to:

• increase hand function by reconstructing the thumb and first web space (the area between the thumb and index finger)
• decrease the deformity by closing the cleft

For details, see Treatment & Care.

Will my child be OK? What’s her long-term outlook?

Your child’s future outlook depends on the severity of her condition, as well as that of any associated conditions she may have.

Causes

There’s a clear genetic basis for typical cleft hands. Inheritance of cleft hand is autosomal dominant: If a parent has the condition, the child has a 70 percent chance of having it, too. The condition can occur by itself (in isolation), or as part of a genetic syndrome, such as ectrodactyly (split hand-split foot malformation) or cleft lip and palate.

Signs and symptoms

Signs of cleft hand are visible at birth, and increasingly, on prenatal ultrasound.

• The baby’s hand has missing fingers, a V-shaped cleft and other deformities.
• There is always a central (middle fingers) cleft.
• But there can also be radial (thumb side) cleft, an ulnar (little finger side) cleft, or various cleft combinations.

When to see a specialist

If a fetal ultrasound reveals that your baby has a cleft hand, you’ll be referred to a hand specialist, who will help you plan for your child’s care after she’s born. If you haven’t learned during your pregnancy that your child has a hand problem, the cleft hand will be visible when your baby is born, and you will be referred to a hand specialist.

Questions to ask your doctor

Lots of parents find it helpful to write down questions as they occur to them before their appointment—that way, when you talk to your child’s doctors, you can be sure that all of your concerns are addressed.

Some questions you could ask are:

• Why did my child develop a cleft hand? (You would ask this if neither your partner nor you have a cleft hand.)
• What will x-rays reveal beyond what is visible to the eye?
• What are the associated conditions, if any, with a cleft hand?
• Is surgery necessary, and if so, what does it entail? Are there alternative therapies?
• Will my child be OK after surgery? Could there be complications?
• Will my child recover full function of her hand? Will it look OK?
• Will there be restrictions on her activities or capabilities?
• Will my child need physical therapy?
• What will be the long-term effects?
• What can we do at home?

Complications

As the child grows, some gaps and deformities that were reconstructed by original surgery can recur. In these cases, additional surgery or surgeries may be needed.

Long-term outlook

Your child’s outlook depends on how severe her cleft hand is, as well as the extent of any associated conditions.

The quality of the reconstruction of your child’s thumb and digits depends on the severity of her original malformation. You can expect that she will have active, functional grasp, pinch and release, and an improvement in the aesthetics of her hand. Alignment of her fingers should improve.

For parents

If your baby was born with a cleft hand—or if you’re expecting a child who will have a cleft hand—you’re probably disappointed that your child and your family are facing a complicated path so early in her life. If you feel frustrated or depressed, speak to your doctor or counselor to get help. Professionals in Children’s Center for Families can provide you with important resources and referrals.

Cleft hand glossary

• The Center for Families at Children’s: dedicated to helping families find the information, services and resources they need to understand their child’s medical condition and take part in their care.
   
• autosomal dominant: genetically predisposed to have the same trait as a parent; in the case of cleft hand, if a parent has the condition the child has a 70 percent chance of having it, as well.
   
• cleft hand: hand is missing fingers (cleft) and has highly variable deformities. Clefts are always central (middle fingers) and are usually V-shaped; they can also be radial (thumb side), ulnar (little finger side), or in combinations of these.
   
• congenital: present at birth
   
• embryonic development: development of the fetus in the womb
   
• (first) web space: the space between digits; the first web space is the space between the thumb and index finger
   
• in isolation: a condition that occurs “by itself,” rather than as part of a larger syndrome
   
• in utero: in the womb (uterus)
   
• occupational and physical therapy: services offered by trained professionals to help restore function or (re)teach basic life skills, like dressing oneself or grasping objects
   
• orthopedics: the medical specialty concerned with diagnosing, treating, rehabilitating and preventing disorders and injuries to the spine, skeletal system and associated muscles, joints and ligaments
   
• orthopedic surgeon, orthopedist: a physician specializing in surgical and non-surgical treatment of the spine, skeletal system and associated muscles, joins and ligaments
   
• post-operative (post-op): occurring after surgery
   
• prenatal (fetal) ultrasound: ultrasound performed at several stages of pregnancy; can detect radial club hand in the fetus
   
• pre-operative (pre-op): occurring before surgery
   
• radial club hand: a deformity in which the forearm doesn’t develop properly, causing the hand to be bent inward toward the thumb with limited movement
   
• radius: forearm bone on the inner (thumb) side
   
• range of motion (ROM) exercises:physical therapy exercises designed to improve or restore flexion and extension of joints
   
• reconstructive surgery: surgery performed to repair and/or restore a body part to normal or as near normal as possible
   
• sporadic: occurring without apparent cause
   
• syndactyly:digits partially or completely united
   
• ulna: forearm bone on the outer (little finger) side
   
• x-rays: a diagnostic test that uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film

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