CURRENT CONCEPTS

Congenital Longitudinal Deficiency

Michael S. Bednar, MD, Michelle A. James, MD, Terry R. Light, MD

Radial and ulnar longitudinal deficiencies are the 2 most common types of congenital
longitudinal deficiencies of the arm, with radial deficiency being 3 to 4 times more common.
They are a spectrum of abnormalities, ranging from mild deficiency of the digits to complete
loss of one-half the forearm, wrist, and fingers. Radial longitudinal deficiency is associated
with a number of medical syndromes that require a comprehensive medical evaluation, while
ulnar longitudinal deficiency (ULD) is associated with other musculoskeletal anomalies.
Both conditions have a high incidence of ipsilateral thumb abnormalities. Wrist and forearm
procedures, such as soft tissue distraction and centralization, are more often required in
radial longitudinal deficiencies than in ULD. Elbow involvement can occur in both
conditions but is more frequent and often more severe in ULD. (J Hand Surg 2009;34A:
1739–1747. © 2009 Published by Elsevier Inc. on behalf of the American Society for
Surgery of the Hand.)
Key words Radial longitudinal deficiency, ulnar longitudinal deficiency, congenital abnormality,
distraction osteogenesis.

ONGENITAL LONGITUDINAL deficiencies of the arm blood vessels. This review focuses on the treatment of

C are divided into radial, central, ulnar, and inter-

segmental forms. Phocomelia, which had been
classified as an intersegmental deficiency, has been
redefined as a type of longitudinal dysplasia. Goldfarb
et al. subdivided phocomelia into 3 groups: group 1 has
a proximal radial longitudinal dysplasia, group 2 has a
proximal ulnar longitudinal dysplasia, and group 3 has
a combined dysplasia, with either absence of the fore-
arm or forearm and arm.1 Central deficiencies are char-
acterized by the absence of the index, middle, and ring
rays and central carpus. Radial and ulnar deficiencies
each demonstrate a spectrum of malformations from
minor digital involvement to complete absence of a
forearm bone, carpal bones, and digits with hypoplasia
of the joints, muscles, tendons, ligaments, nerves, and
From the Department of Orthopaedic Surgery and Rehabilitation, Stritch School of Medicine, Loyola
University,Maywood,IL;andtheShriners’HospitalforChildren,NorthernCalifornia,Sacramento,CA.
Received for publication August 17, 2009; accepted in revised form September 2, 2009.
radial and ulnar dysplasia as they affect the forearm and
wrist. Treatment depends on the severity of the malfor-
mation. A variety of newer techniques have been de-
scribed for the treatment of these conditions. For more
severe radial dysplasia, distraction lengthening has
proven a useful tool but requires attention to detail over
several months.
Radial longitudinal deficiency (RLD) is the most
frequently occurring pattern of congenital longitudinal
deficiency.2 The condition occurs in approximately
1:30,000 live births and is more often a sporadic mu-
tation rather than an inherited condition. Although RLD
is often bilateral, the extent of involvement is often
asymmetric, varying as widely as complete absence of
the radius, radial carpus, and thumb on 1 side and minor
hypoplasia of the thumb on the other (Fig. 1).
Radial longitudinal deficiency is often associated
with other systemic and musculoskeletal conditions.
Current Concepts

No benefits in any form have been received or will be received related directly or indirectly to the
Goldfarb et al.3 recently reported that 67% of RLD
subject of this article. patients had an associated systemic or musculoskeletal
Corresponding author: Michael S. Bednar, MD, 1700 Maguire Center, 2160 S. 1st Avenue, May- abnormality, meaning that only 33% present with an
wood, IL 60153; e-mail: mbednar@lumc.edu. isolated upper limb deformity. Common syndromes
0363-5023/09/34A09-0027$36.00/0 associated with RLD include thrombocytopenia absent
doi:10.1016/j.jhsa.2009.09.002
radius syndrome, Holt-Oram syndrome, VACTERL as-

©  Published by Elsevier, Inc. on behalf of the ASSH. 䉬 1739

 1740 CONGENITAL LONGITUDINAL DEFICIENCY

sociation, and Fanconi anemia. Thrombocytopenia ab- (15% of RLD) (Fig. 4). Types 0 and 1 are most often
sent radius syndrome is an autosomal-recessive disor- associated with proximal radioulnar synostosis and con-
der characterized by an absent radius with a relatively genital radial head dislocation. These conditions are
normal thumb. Holt-Oram syndrome is an autosomal- seen in 44% of patients with type 1 RLD and 11% with
dominant disorder in which RLD is accompanied by type 0.
either an atrial or ventricular septal defect. Fanconi Treatment of RLD is based on the functional impair-
anemia is an autosomal-recessive pancytopenia, once ment and type of anomaly present. Functional impair-
invariably fatal, that is now ment reflects thumb hyp-
routinely treated with bone EDUCATIONAL OBJECTIVES oplasia or absence, wrist
marrow transplantation. ● Describe the types of radial deficiency instability with radial de-
VACTERL or VATER ● Discuss the syndromes associated with radial deficiency and their respec- viation of the hand on the
association is a sporadic tive evaluation forearm, and a shortened
cluster of congenital anoma- ● Describe the technique of centralization and associated complications forearm. Type N isolated
lies, several of which may ● State the predominant upper extremity findings with ulnar deficiency thumb anomalies are sub-
occur in the same child. The ● classified by the modified
Discuss the various techniques to treat ulnar deficiency
abnormalities that often oc- Blauth classification and
cur in these children are ver- Earn up to 2 hours of CME credit per JHS issue when you read the related are treated with recon-
tebral defects, anal atresia, articles and take an online test. To pay the $20 fee and take this month’s test, struction or pollicization,
cardiac malformation, tra- visit http://www.assh.org/professionals/jhs. depending on the presence
cheoesophageal fistula, esoph- or absence of a stable
ageal atresia, renal anomalies, and limb anomalies. thumb carpometacarpal joint. Type 0 RLD limbs
The most frequent limb abnormality is RLD. demonstrate a normal length radius with anomalies
Goldfarb et al.3 reported that although only one-third of the thumb and carpus and a variable degree of
of their RLD patients have a syndrome, 102 of 138 radial deviation deformity of the wrist. Mo and
patients with RLD have an associated systemic or mus- Manske6 intraoperatively identified the anatomic
culoskeletal abnormality. The most common associated factors producing the radial tether as the radial
musculoskeletal abnormality was scoliosis. Other asso- wrist extensors and the tight radial capsule. In their
ciated upper limb anomalies include humeral hypopla- series, the wrist was centralized by release of the
sia, proximal radioulnar synostosis, congenital radial radial wrist capsule, release of the radial wrist
head dislocation, and digital stiffness. An association extensors, sectioning of the extensor carpi ulnaris
was noted between increasing severity of RLD and the (ECU) tendon proximal to its insertion, transfer of
percentage of patients with an associated condition. the ECU to the dorsal wrist capsule, transfer of the
All children with RLD should undergo a thorough released radial wrist extensors to the distal stump
musculoskeletal and systemic examination. One should of the ECU, pinning of the carpus in neutral, and
consider further screening with spinal radiographs, car- cast immobilization for 6 to 8 weeks. In this series,
diac echocardiogram evaluation, renal ultrasound, and a
complete blood count. Because children with Fanconi
anemia often do not manifest aplastic anemia on routine
blood testing until after age 3, Kozin4 recommended a
screening chromosomal challenge test when the chil-
dren are first seen. A health care provider who is qual-
ified to provide genetic counseling should perform the
Current Concepts

work-up for syndromes and associated conditions.

James et al.5 radiographically classified radial longi-
tudinal deficiency into 6 types (Table 1). Type N is an
isolated thumb hypoplasia or absence. Type 0 is type N
plus an anomaly of the radial carpus (Fig. 2). Types N
and 0 accounted for 52% of all RLD in James et al.’s
series. Types 1 to 4 demonstrate increasing extent of FIGURE 1: Bilateral hand anteroposterior x-ray of a 5-year-old
deficiency of the radius. Of these, type 4, complete girl with type 4 RLD on the left and type N on the right. She
underwent centralization on the left and pollicization on the
absence of the radius, is the most common (27% of
right.
RLD) (Fig. 3) and type 1 is the second most common

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 CONGENITAL LONGITUDINAL DEFICIENCY
TABLE 1. Modified Classification of RLD
Type Thumb Carpus
N Hypoplastic or absent Normal
0 Hypoplastic or absent Absence, hypoplasia, or coalition
1 Hypoplastic or absent Absence, hypoplasia, or coalition
2 Hypoplastic or absent Absence, hypoplasia, or coalition
3 Hypoplastic or absent Absence, hypoplasia, or coalition
4 Hypoplastic or absent Absence, hypoplasia, or coalition
FIGURE 2: Anteroposterior x-ray of a 2-year-old boy with type
N/0 RLD. The carpus has not yet ossified to determine whether
the deficiency is present only in the thumb or in the thumb and
carpus.
all patients preoperatively had a minimum of 35°
of radial deviation. An average preoperative radial
deviation of 58° was improved to 12° postopera-
tively. Type 1 RLD with considerable radial devi-
ation could theoretically be treated in a similar
fashion, although this has not been described in the
literature. Type 2 RLD is rare; no treatment has
been described in the literature, and there is no
consensus on whether it should be treated like
types 0 and 1, or like types 3 and 4.
The most challenging types of RLD to treat are types
3 and 4 with substantial wrist radial deviation and
palmar subluxation. Treatment consists of centraliza-
tion of the carpus onto the end of the distal ulna and
transfer of the aberrant radial wrist extensors. The pro-
cedure should ideally be done at 1 year of age. This is
followed by pollicization if the thumb lacks a stable
carpometacarpal joint or is absent (Fig. 5). The goals of
centralization are to place the hand at the end of the
JHS 䉬 Vol A, November 
1741
Distal Radius Proximal Radius
Normal Normal
Normal Normal, radioulnar synostosis, congenital
radial head dislocation
⬎2 mm shorter Normal, radioulnar synostosis, congenital
than ulna radial head dislocation
Hypoplasia Hypoplasia
Physis absent Variable hypoplasia
Absent Absent
forearm to increase limb length and to stabilize the wrist
in a straighter position.
Traditionally, the tight radial-sided soft tissue is
stretched preoperatively with splints, serial casts, and
hand therapy from the day of birth until the day of
surgery. Parents are instructed to apply gentle hand
pressure to move the hand toward the ulna for 5 to 10
minutes, 4 to 5 times per day. Splints placed radially or
ulnarly are removed intermittently during the day for
unconstrained use of the extremity. Persistent tightness
of the radial tissues at the time of surgery, especially the
median nerve, may limit the ability to place the hand on
the end of the ulna. When the intact carpus cannot be
reduced onto the ulna, skeletal shortening is achieved
by resection of a segment of ulnar diaphysis, shaving
the distal ulna or carpectomy, and thereby creating a
notch in the carpus for the ulna.
When the wrist cannot be passively brought to neu-
tral, preoperative distraction lengthening of the soft
tissues is increasingly employed7 (Fig. 6). At the time
of application of the fixator, a longitudinal incision
over the palmar, radial aspect of the wrist provides
exposure to release the flexor carpi radialis tendon
as well as the tight flexor side fascia.8 Distraction is
continued 2 to 4 times each day until the carpus is
drawn distal to the distal ulnar epiphysis. The dis-
traction device is retained for an additional 4 to 5
weeks to allow the distracted soft tissues to heal. The
distractor is removed at the time of the centralization.
The classic centralization procedure aligns the mid-
dle finger metacarpal on the axis of the ulna. Buck-
Current Concepts
Gramcko popularized the radialization procedure, in
which the axis of the ulna is aligned with the index
metacarpal, thereby ulnarly deviating the wrist.9 Skin
incisions are planned to bring redundant skin from the
ulnar side of the wrist to the radial side. Van Heest re-
viewed the skin incisions proposed by 5 different au-
thors.10 Flap designs by Buck-Gramko, Manske,
Watson, and Tonkin all require excision of excess
 1742 CONGENITAL LONGITUDINAL DEFICIENCY
FIGURE 3: A, B Clinical photograph and AP x-ray of type 4 RLD with considerable tightness of radial soft tissues.
FIGURE 4: Anteroposterior x-ray of a 2-year-old boy with
type 1 RLD. Proximal radioulnar synostosis is present.
ulnar skin with stretching or release of the tight radial
skin. Evans’ bi-lobed flap moves ulnar tissue radi-
ally, but reported problems include an ulnar-sided
dog ear and necrosis of the distal tip of the flap. Van
Heest recommends a dorsal rotation flap, from the
ulnar midlateral border of the wrist, transversely
across the dorsum of the wrist in Langer’s lines, and
to the radial midlateral border of the wrist. When the
hand is centralized with ulnar rotation, the flap is
rotated radially. This approach provides good visu-
Current Concepts
alization of the tight radial-sided structures for re-
lease as well as the ulnar structures for capsular
augmentation and tendon transfer. No primary
wound problems were seen in 21 extremities. A total
of 15 of the wounds were photographed. All were
rated good concerning color, contour, distortion, and
overall appearance. One had dimpling of the exces-
sive ulnar skin.
Soft tissue balancing is required to obtain and main-
tain the carpus centralized over the ulna. The ECU and
JHS 䉬 Vol A, November 
remnant radial wrist extensors are detached from their
insertions. The wrist capsule is opened distal to the
ulnar physis and released until the carpus can be
brought over the distal ulna. The middle finger meta-
carpal is then pinned to the ulna to maintain the posi-
tion. The pin is left in for at least 6 months, but pref-
erably longer. If the ulna is bowed more than 30°, a
closing wedge osteotomy is performed and the longi-
tudinal pin is also employed to secure the osteotomy.
The wrist capsule is imbricated and the extensor reti-
naculum reinforces the wrist capsule. The ECU tendon
is advanced to increase tension on the ulnar side of the
wrist, and the radial wrist extensors, if present, are sewn
to the ECU stump.
Lamb et al. reported a group of patients at a mean of
27 years after centralization.11 Upper limb function
assessed by grip strength, Moberg and Jebsen tests, and
questionnaires about activities of daily living demon-
strated satisfactory limb function. Premature fusion of
the distal ulnar epiphysis was not found. Long-term
maintenance of the correction was often associated with
spontaneous ulnocarpal fusion.
The radial deficient limb is always shorter than a
normal limb. Heikel12 demonstrated that an unoperated
RLD ulna is approximately 60% to 65% of the normal
ulna. Sestro et al.13 reported that after centralization,
RLD limbs treated nonsurgically attained 64% of nor-
mal ulnar length. RLD centralized without notching the
carpus attained 58% of normal ulnar length, whereas
RLD centralized with notching of the carpus attained
48% of normal growth. However, recurrence of radial
deformity for non-notched centralization was higher
than for notched centralization (35.4° vs 14.7°). Al-
though centralization increases the apparent length of
the limb by placing the hand on the end of the ulna, it
may also decrease the length of the ulna at skeletal
maturity.
 CONGENITAL LONGITUDINAL DEFICIENCY 1743

FIGURE 5: A 1-year-old girl with type 4 RLD and a pouce flottant. A Wrist rests in radial deviation. B, C Wrist can be passively
brought to neutral position, indicating supple soft tissues on the radial side of the wrist. D Preoperative AP x-ray of the wrist. E
Clinical result 1 year after centralization with a stable wrist in neutral position. F AP x-ray 1 year postcentralization. Pin is
maintained in the carpus and ulna. G Clinical photograph immediately after pollicization.

In some RLD patients, the distal ulnar physis con-
tributes little longitudinal growth, either because of
distal ulnar has been damaged after centralization or
because of limited intrinsic growth potential (Fig 7). A
method. Eight children with unilateral RLD under-
went 9 lengthening surgeries, and 1 child with
bilateral RLD underwent 4 lengthening proce-
dures. The average age at the time of surgery was
Current Concepts

unilateral severely shortened limb is an aesthetic con-
cern, whereas bilateral short limbs may pose a func-
tional issue with perineal care.
A few studies of distraction lengthening have
reported the use of bone lengthening to increase
forearm length in RLD children. The most recent
paper by Peterson et al.14 reported on 9 children
who had 13 ulnar lengthenings using the Ilizarov
12 years. The overall increase in ulnar length was
4.4 cm (range, 1.8 – 8.0 cm). The average time of
lengthening was 14.4 weeks (range, 3–23 weeks)
and the average consolidation time was 23 weeks
(range, 12– 45 weeks). The mean lengthening in-
dex (total time in fixator per length gained) was 8.6
weeks/cm (range, 3.6 –24 weeks/cm). Complica-
tions were frequent. All patients developed pin

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 1744 CONGENITAL LONGITUDINAL DEFICIENCY

FIGURE 6: A 1-year-old boy with thrombocytopenia absent radius syndrome. A Wrist is held in radial deviation and cannot be brought
to neutral with passive ulnar deviation. B External fixator placed to distract taught radial soft tissues. Palmar radial incision for release of
flexor carpi radialis tendon and radial wrist capsule. C Clinical photograph 3 months after removal of the external fixator.

recommended for mature older children or teenagers

FIGURE 7: Patient after centralization of RLD type 4 with
substantial shortening of the ulna.
tract infections that required oral antibiotics. Three
Current Concepts
with good family support who have realistic expecta-
tions and emotional stamina.
Vilkki17 reported a different approach to balancing
the radial deficient wrist. He carried out soft tissue
distraction for at least 9 weeks, followed by a vascular-
ized second toe metatarsophalangeal joint transfer to the
radial side of the ulnocarpal joint. The procedure im-
proves wrist motion and stability while preserving lon-
gitudinal growth. Distally the phalangeal portion of the
graft is secured to the index metacarpal and proximally
the metatarsal is secured to the distal ulna proximal to
the physis. The radial soft tissue tether is released, but
no tendon transfer is performed. The fixator is left in
place for 2 months while the vascularized graft is in-
corporated. At 6-year follow-up, the mean ulnar length
was 67% of the contralateral side. The 3 cases compli-
cated by microvascular failure or fracture of the graft
had less favorable results. In uncomplicated cases, the
results for hand–forearm angle and ulnar bowing were
classified as good to very good. It is recommended that
the procedure be done between 1 and 2 years of age.
Although promising, the technique is technically de-
manding and should be undertaken only by surgeons

patients required intravenous antibiotics and pin
removal and were further complicated by failure of
the osteotomy to consolidate, thereby requiring
internal fixation and bone grafting. Six lengthening
procedures were associated with elbow or finger
stiffness, which resolved in 5 of the 6 cases. Of
9 patients, 8 stated they were satisfied with the
additional forearm length, and 1 was lost to follow-up.
These results are similar to those published by other
investigators.15,16 Osseous distraction lengthening is
skilled in microvascular free toe transfer.
ULNAR LONGITUDINAL DEFICIENCY (ULD)
Ulnar longitudinal deficiency occurs in 1 in 100,000
live births and is 4 to 10 times less common than RLD.2
ULD is a sporadic, non-inherited condition. Unlike
RDL, it is not associated with systemic conditions, but
is associated with other musculoskeletal conditions
such as proximal femoral focal deficiency, fibular defi-
ciency, phocomelia, and scoliosis.

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 CONGENITAL LONGITUDINAL DEFICIENCY 1745

TABLE 2. Modified Classification of ULD

Type Ulna Radius Elbow Wrist/Hand

0 Normal Normal Stable Absence/hypoplasia of digits

Carpal bone absence or coalition
1 Hypoplasia Mild bowing Stable Mild ulnar wrist deviation
Distal and proximal Absence/hypoplasia of digits
ephiphyses present common
2 Partial aplasia Bowing Stability variable Mild ulnar wrist deviation
Proximal ulna present Radial head may dislocate posterolaterally
Anlage tethers radius
3 Total absence Straight Unstable Less ulnar wrist deviation
No anlage Posterolateral dislocation of radial head Carpal/digital deficiences severe
Severe elbow flexion deformity may coexist
4 Usually completely Severe radial Radiohumeral synostosis Ulnar wrist deviation
absent bowing Humeral internal rotation
Analage present Forearm pronation
5 Total absence Straight Radiohumeral synostosis Less ulnar wrist deviation
Humeral bifurcation or large medial condyle Carpal/digital deficiences severe

Radial-sided abnormalities consisted of 4 patients with

TABLE 3. Thumb Abnormalities in ULD
Type Characteristics
A Normal first web space
B Mild first web and thumb deficiencies
C Moderate to severe first web and
thumb deficiencies
Potential loss of thumb opposition
Malrotation of the thumb into the
plane of other digits
Thumb–index syndactyly
Absent extrinsic tendon function
D Absent thumb
Upper limb involvement is more commonly unilat-
eral.2 The entire involved limb is hypoplastic. The el-
bow is abnormal or fused in most cases. Although the
ulna may be partially or completely absent, a cartilag-
inous analage is often present. The hand and carpus
are almost always affected. Up to 90% of hands
have missing digits and 30% have syndactyly. As
much as 70% of patients with ULD have thumb
abnormalities.
Bayne18 classified ULD primarily on the basis of
hypoplasis or aplasia of digits. One patient had an
Hands index–middle syndactyly. At the carpus, the most com-
27%
mon abnormality was intercarpal fusions, seen in 5
patients.
18%
Treatment of the ULD limb begins with assessment
36%
of the thumb and fingers. Cole et al.21 classified thumb
abnormalities into 4 types (Table 3). In their series, 75%
of type C and 80% of type D hands were treated by
hand reconstruction procedures, whereas only 7% of
type A and 30 % of type B underwent surgery. In the
type C thumbs, the most common procedures were
18%
first-web space deepening, thumb metacarpal rotational
osteotomy, and thumb syndactyly release. The most
common surgery on the other digits was syndactyly
release.
Treatment of ULD wrist and forearm deformity is
based on the degree of deformity. Excision of the ulnar
anlage has been shown to improve both function and
the appearance of the hand (Fig. 8). Although ulnar
anlage excision is usually indicated in patients with
documented progression of ulnar deviation, it may be
valuable in any patient with fixed ulnar deviation of the
wrist.
Current Concepts

forearm and elbow anomalies. Havenhill et al.19 added
type 0 to include patients with normal elbow and fore-
arms and abnormalities of the hand and carpus, whereas
Goldfarb et al.20 added type V (Table 2). Of the 13
patient studied with type 0 ULD, 3 had complete ab-
sence of the small finger ray, 6 had absence of both the
small and ring rays, and 4 had hypoplasia of the small
finger, 3 with a synostosis of the ring–small metacarpal.
El Hassan et al.22 reviewed 17 limbs in 14
patients with type IV ULD, characterized by radio-
humeral synostosis (Fig. 9). The elbows were
fused at an average of 63° of flexion (range, 10° to
90°). None of the patients demonstrated an internal
rotation contracture. The only wrist and forearm
procedures in this group were forearm rotational
osteotomy in 1 patient and bilateral ulna anlage

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 1746 CONGENITAL LONGITUDINAL DEFICIENCY

FIGURE 8: A Anteroposterior x-ray of a 1-year-old girl with a ULD type 4 and ulnar deviation of her wrist. B AP x-ray 3 years
after excision of the ulnar analage. There has been radial growth past the distal end of the ulna.

daily living. A total of 5 of 11 participated in

sports and considered themselves capable of doing
most activities compared with their peers. All 3
bilateral patients were satisfied with their upper-
extremity function. In this study, most patients
with radio-humeral synostosis functioned well
without wrist or forearm surgery.
When the elbow of a type IV limb is positioned
in hyperextension, the hand rests on the hip. A
flexion osteotomy will bring the hand forward.
Shortening may be required to relieve soft tissue
tension and neurovascular traction. In some cases,
gradual reorientation of the limb may be achieved
by ring fixator distraction.
Radial and ulnar longitudinal deficiencies may
range from mild abnormalities of the digits to
complete absence of half of the forerarm, wrist,
and hand. Both have a high incidence of thumb
anomalies, many of which may be surgically
treated to improve hand function. Shortening of
Current Concepts

the limb appears to be tolerated in both conditions

FIGURE 9: Anteroposterior x-ray of ULD type 4 with
radiohumeral synostosis and absence of the ring and small rays
and underlying ulnar carpus.
excision in a second patient. Six hand surgery
procedures were performed. Of 11 unilateral pa-
tients, 9 reported no limitations in activities of
when unilateral.
Radial longitudinal deficiency is commonly associ-
ated with syndromes that require a comprehensive med-
ical evaluation, whereas ULD is commonly associated
with additional musculoskeletal abnormalities. Wrist
and forearm procedures, such as soft tissue distraction
and centralization, are required more often in RLD than
in ULD. Excessively shortened limbs are more com-
monly treated with distraction lengthening in RLD than

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 CONGENITAL LONGITUDINAL DEFICIENCY
ULD. Elbow involvement can occur in both conditions,
but is more frequent and often more severe in ULD.
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Current Concepts