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Indian J Pediatr

DOI 10.1007/s12098-015-1860-x

REVIEW ARTICLE

Congenital Clubfoot: Early Recognition and Conservative


Management for Preventing Late Disabilities
Yubin Liu 1 & Dahang Zhao 1,2 & Li Zhao 1 & Hai Li 1 & Xuan Yang 1

Received: 30 April 2015 / Accepted: 22 July 2015


# Dr. K C Chaudhuri Foundation 2015

Abstract Congenital clubfoot is one of the most common Keywords Clubfoot . Early recognition . Ponseti method .
musculoskeletal deformities presenting at birth. Many high risk Disability prevention
factors have been associated with clubfoot such as male gender,
primiparous mothers and maternal smoking. Accurate under-
standing of clubfoot pathoanatomy is supposed to be the basis Introduction
for deformity correction. Prenatal ultrasonography is of refer-
ence value in recognizing clubfoot during pregnancy. Congenital clubfoot, i.e., congenital talipes equinovarus, de-
Neglected clubfoot can eventually cause a noticeable disability scribes a range of foot and ankle deformities usually present at
and severely influence the quality life in adulthood. Early rec- birth, which can be separated into four major components:
ognition and treatment are presumed to be the key for preven- forefoot adduction, hindfoot varus, equinus and cavus
tion of late disabilities. Nowadays, Ponseti method, as one of (Fig. 1). The term Bclubfoot^ refers to the way the foot is
the conservative treatment regimen, has been widely accepted positioned at a sharp angle to the ankle, like the head of a golf
because of the reported good results of long-term follow-up. club. Left untreated, clubfoot can eventually cause discomfort
However, special attention should be paid to the details in clin- and become a noticeable disability. Careful physical examina-
ical practice for achieving even better correction and a lower tion after birth by an experienced doctor is recommended as
rate of relapse. After the complete correction, brace wearing is an economical and effective measure for screening. Early rec-
critical for preventing deformity relapse. Non-compliance or ognition and intervention for clubfoot can help improve treat-
non-adherence with the brace protocol has been considered as ment results and decrease the risk of late disabilities.
the predominant risk factor predisposing to the relapse of club-
foot, which is still a challenging problem. This paper was pre-
pared to give a general introduction about clubfoot, in terms of Definition and Epidemiology
the importance of early recognition and conservative manage-
ment, especially Ponseti method, for preventing late disabilities. Clubfoot could be isolated, which is commonly considered
idiopathic clubfoot [1], or associated with other medical syn-
dromes or neuromuscular disorders such as spina bifida,
myelomeningocele and arthrogryposis, which account for
* Li Zhao
about 20 % of all cases. Clubfoot can occur all around the
orthzl@126.com world with an incidence from 0.39 to 8 per 1000 live births
[2, 3]. The ratio of clubfoot between male and female is about
1
2:1 and up to 50 % of cases are bilateral [4]. Among unilateral
Department of Pediatric Orthopedics, Xin-Hua Hospital, Shanghai
Jiao Tong University School of Medicine, No. 1665 Kongjiang
cases, the affected feet are more common in right side [5].
Road, Yangpu District, Shanghai 200092, China Although numerous hypotheses, such as neuromuscular,
2
The Third Department of Orthopedics, The First Affiliated Hospital
bone, connective tissue and vascular factors, have been report-
of Harbin Medical University, No. 23 Youzheng Street, ed [6], the precise etiology and pathogenesis of this deformity
Harbin 150001, Heilongjiang Province, China remain unclear. However, clubfoot clusters in families suggest
Indian J Pediatr

Fig. 1 Four components of


clubfoot deformity: a Forefoot
adduction; b Hindfoot varus;
c Equinus; d Cavus

that genetic heredity may be an important causation for the cuneiforms and plantar flexion of the first metatarsal greater
deformity. The increased risk was also observed in first-degree than the fifth one (Fig. 2) [15].
relative of an affected person than in the general population The ligaments of the posterior and medial aspects of the
[2]. Unaffected parents with a clubfoot child have a chance of foot are thickened and shortened, severely restricting the foot
1 in 40 that another son has clubfoot [3]. The evidence of in the position of equinus and adduction. The severity of club-
environmental factors for clubfoot etiology is scant and foot deformity has a positive correlation with leg muscle sizes.
often derived from poorly designed investigations. The The muscles of tibialis posterior, gastrosoleus and toe flexors
issue of environmental factor was firstly proposed in are smaller in size and shorter. Excessive collagen synthesis is
1970s based on the work by Dunn [7]. Nowadays, many observed in ligaments and tendons, and this progress may
high risk factors have been reported to be associated with the persist until the child is 3 or 4-y-old [16]. The inherent prop-
occurrence of clubfoot such as male gender, primiparous erties of connective tissue, cartilage and bone can be remolded
mothers, maternal smoking, high-intensity of alcohol by the changes of mechanical stimuli regularly, resulting in a
and coffee intake [8–11]. Other risk factors, such as normal looking foot [17]. This may be the basis of manual
marital status, maternal diabetes, maternal age and level of correction of the deformity.
education, are still controversial and need further researches
[4, 9, 10, 12–14].
Prenatal Examination

Pathoanatomy Birth defect is one of the leading causes of infant mortality and
disability. Early detection could lead to early treatment and
Clubfoot is a three dimensional deformity of the foot and eventually improve the life quality of the patients. With the
ankle with the major pathologies at the tarsal bones. The de- widespread use of ultrasonography and improved techniques,
formity has four components: equinus, varus of hindfoot, an increased detection of clubfoot has been made prenatally.
cavus and forefoot adductus. The talus is in severe plantar Because it is difficult to detect clubfoot deformity by means of
flexion and its neck is medial and plantar deflection. The an- ultrasonography before the 16th wk of gestation [18], prenatal
terior portion of calcaneus lies directly beneath the head of the detection of musculoskeletal disorders by means of obstetric
talus, which is responsible for varus and equinus of the heel. ultrasonography is performed usually around 20 wk of gesta-
The navicular turns inward and articulates with the medial tion. However, prenatal ultrasonography diagnosis of clubfoot
surface of the talar head. The medial displacements of the had a false positive (FP) rate between 0 and 29 % [19].
navicular, cuboid, cuneiforms and together with the metatar- Glotzbecker et al. designed a novel sonographic scoring sys-
sals contribute to the adduction deformity. The cavus defor- tem to improve detection of a true clubfoot and reduced the FP
mity is produced by the slight downward displacement of the rate to 7 % eventually as reported [20].
Indian J Pediatr

Fig. 2 Skeletal model showing


the pathologies in tarsal bones:
a Head (TH) and neck of talus are
plantarflexed and medially
deviated. Navicular (N) is
medially displaced on the talus;
b Cavus resulted from a greater
flexion of the first metatarsal bone

Parents may be extremely stressful about the immediate Conservative Treatment Methods
diagnosis after baby birth, especially when there is lack of
detailed information of clubfoot and explanations of progno- Owing to the fact that the etiology of clubfoot remains
sis. With a prenatal diagnosis of clubfoot, parents may be unknown, we can hardly treat this condition at the
more active to counsel for knowledge of clubfoot and prepare levels of ligament, tendon and muscle pathology, which
the families for the arrival of their babies. Prenatal detection may be the main causation [23]. The treatment goal of
avoids a surprise of clubfoot, and most mothers seem to ap- clubfoot is acquisition of a pain-free, functional and planti-
preciate having a prenatal diagnosis [21]. grade foot without the need to wear modified shoes.
Neglected clubfoot deformity can deter the development of a
normal gait, lead to a lifetime disability for the patient, limit
Diagnosis and Classification employment opportunities and socialization, and often result
in the problems at the family level. Besides, ostracism or
Although prenatal obstetric ultrasonography plays an impor-
tant part in clubfoot screening, a definite diagnosis of the
Table 1 Clubfoot incidence in association with various syndromes
clubfoot depends on typical clinical examination after birth,
which can be easily made by pediatrician, pediatric orthope- Syndromes Abnormalities except for clubfoot deformity
dist and even physical therapist. Classification of clubfoot can
be divided into four types based on the cause and response to Arthrogryposis Limited range of motion in the involved joints;
A firm, inelastic end point;
treatment as follows: idiopathic, postural, neurogenic and Lack of normal skin creases
syndromic clubfoot [22]. The idiopathic clubfoot, accounting Stickler syndrome Eye problems;
for most cases, occurs in an otherwise normal child. This Hearing difficulties;
deformity does not resolve spontaneously and the treatment Overly flexible joints;
should be started soon after the birth. The long-term follow-up Scoliosis and Osteoarthritis in adolescence
outcome with the Ponseti method is usually good or excellent. BTARP^ syndrome Atrial septal defect;
Robin sequence;
Postural clubfoot is a deformity secondary to intrauterine Persistence of the left superior vena cava
crowding. This type of foot deformity is flexible and usually Ampola syndrome Mental retardation;
becomes normal along with the time. Follow-up is mostly Facial anomalies;
recommended for the clinical observation. However, this type Short stature;
of deformity can occur at an early stage of a true clubfoot in Seizures and finger and toe abnormalities
preterm babies with the gestational age of 24 to 30 wk. Bruck syndrome Osteoporosis and bone fragility;
Progressive joint contractures
Neurogenic clubfoot is often associated with other neurolog-
De Grouchy syndrome A short stature;
ical disorders such as spina bifida and meningomyelocele, for
Hypotonia (Lack of muscle tone);
which correction is usually more difficult. Syndromic clubfoot Hearing impairment;
is one of the manifestations in the patient with a syndrome Distal hypoplastic tapering of lower legs;
such as multiarthrogryposis, Stickler syndrome and so on Vertical talus
(Table 1). Conservative treatment remains the first choice, Potter syndrome Limb contractures;
Sacral agenesis;
but may be more difficult. The prognosis of this type
Pulmonary hypoplasia;
may depend much more on underlying conditions than the Cranial anomalies
clubfoot.
Indian J Pediatr

derision derived from the deformed appearance of the foot and efficacy has been extensively demonstrated around the world
ankle can also be a source of psychological harm. So, wide- [30]. Great advantages of the Ponseti method have been found
spread concern should be aroused to initiate a positive action in that it is easy to learn for spreading and affordable for
for clubfoot treatment at an early stage. ordinary families with the initial correction rate even as high
Treatment protocol has been shifted from a standard surgical as 100 % and excellent long-term follow-up outcomes [25, 31,
management to the conservative treatment over the past 2 de- 32]. Despite of the great popularity of the Ponseti method, the
cades [24] because the extensive soft-tissue release was report- regimen should be intensive and special attention should be
ed to result in a scarred, stiff, weak and painful foot, and to paid to the details for a higher success rate [33].
impair the quality of life significantly in the long-term follow-
up [25]. However, children with stunted, rigid feet or those with Practical Issues Relating to Ponseti Method
a very severe deformity may still require a surgical procedure of
soft tissue release. Nowadays, most orthopedists have agreed The Ponseti method, developed by Ignacio Ponseti in the late
that the initial treatment of clubfoot should be non-operative, 1940s, is based on adequate understanding of the functional
meanwhile the prior choice is that including serial manipula- anatomy of the foot and ankle. The outlines are as follows: 1)
tions and casts applied soon after the birth [26, 27]. The success all the deformities are corrected simultaneously; 2) cavus is
rate of clubfoot management mainly depends on the severity corrected at the first maneuver of manipulation to elevate the
and stiffness of the deformity. However, it is still difficult to first ray of foot; 3) to abduct the forefoot while applying
foresee how a clubfoot will respond to the treatment. For ex- counter-pressure against the lateral aspect of the head of the
ample, some severe clubfeet may be easily corrected, while talus; 4) heel varus is corrected when the entire foot is fully
some others may be corrected with great difficulties by means abducted, but the heel is never touched; 5) abduction of the
of the same procedure of manipulation and casting. forefoot is increased progressively; 6) in the last cast, the foot
Three conservative approaches for correction of clubfoot should be markedly abducted to 60° to 70° without pronation;
named Kite, French and Ponseti methods were introduced as 7) residual equinus is usually corrected by means of percuta-
follows: neous Achilles tenotomy (PAT) with the indication when 15°
of ankle dorsiflexion is not obtained; 8) post PAT long-leg cast
Kite Method is applied and left in place for 3 wk to allow for healing of the
Achilles tendon; 9) for maintaining the correction obtained by
According to the Kite method, the thumb in manipulating manipulation with or without PAT, a well-molded plaster cast
should be located on the lateral side of the calcaneocuboid is applied in 2 sections with knee flexed at 90° or more and the
joint, which may block the abduction of the calcaneus. As it cast is changed weekly; 10) when the full correction is
is reported, the incomplete or defective correction rate is up to achieved, brace is used to maintain the foot at 60° to 70°
95 % [28]. Besides, the complications of Kite method include external rotation on the affected side and 30° to 40° on the
the rocker-bottom deformity, fracture of metaphysis and stiff- normal side.
ness of the joint. The initial correction rate of this method can be achieved
reportedly in more than 95 % [25] and satisfactory clinical and
French Method functional results have been demonstrated by long-term fol-
low-up of the patients [16, 23]. However, some practical is-
The French method, also known as Bfunctional method^, was sues of the Ponseti method should be raised in clinical aspects.
developed by Dr. Masse in 1970s [26]. It consists of daily According to the Ponseti method, treatment should be started
manipulation, muscles stimulation and correction mainte- soon (7 to 10 d) after birth. However, Iltar et al. reported that
nance of the foot using non-elastic straps and splints. The casting with the Ponseti method in infants older than 1 mo of
encouraging result was reported; almost 86.6 % of the cases age or with an affected foot ≥8 cm in length had a better final
were excellent and good using this method [29]. However, Dimeglio score [34]. In authors’ institute, they start the treat-
this method is very time consuming and difficult to master, ment at 4 to 5 d after birth. The deformity could be corrected in
with a success rate depending on the skills of individual phys- most cases with no more than five long-leg Ponseti casts (av-
ical therapists [26, 29]. erage 4.1 casts). The initial correction rate in the case of un-
treated idiopathic clubfoot is 100 % (Data not yet published).
Ponseti Method To demonstrate the association between clubfoot and DDH
(developmental dysplasia of the hip) in the neonates so that
Nowadays, Ponseti method has become the most popular ap- both conditions can be treated simultaneously at the possible
proach for the clubfoot management and its safety and early stage, hip sonography is recommended in authors’
Indian J Pediatr

institute. According to Graf classification, the incidence of


DDH is greater (2.7 %) in the neonates with idiopathic club-
feet [35]. These findings in authors’ institute help the early
recognition of DDH, which also requires the early interven-
tion for the better outcome (Fig. 3).
Clubfoot can be diagnosed immediately by first looking at
the foot. The question is raised as to how to accurately assess
the severity of the deformity at birth. Several assessment sys-
tems have been established to monitor and document the se-
verity of the deformity. Currently, Pirani and Dimeglio Score
systems have been widely used and a good correlation has
been demonstrated between these two systems [36]. Pirani
score is more commonly used to recognize the severity of each
component of the deformity, scored as 0 (normal), 0.5 (mild)
and 1 (severe). Besides, Pirani score can also help decide
when to perform a PAT procedure.
Successful management needs an adequate understanding of
pathoanatomy of clubfoot. Kite method was developed to cor-
rect foot equinus, varus and adductus deformities respectively
in a gradual fashion. As requested in this technique, the thumb
should be located on the lateral side of the calcaneocuboid joint,
which eventually leads to the failure of clubfoot correction. The
first element in the manipulation of Ponseti method is to correct
the cavus deformity by elevating the first ray of the forefoot
(Fig. 4). Elevation of the first metatarsal and supination of the
forefoot seems to be Bcounterintuitive^ for increasing the ap-
Fig. 3 A boy, 6-wk-old, with clubfoot and DDH: a & b An Otto Bock pearance of foot inversion. However, it is the most critical step
splint is worn after the fourth Ponseti cast; c An Otto Bock splint is worn
during the phase of foot abduction orthosis (reproduced with permission
to position the forefoot in proper alignment with the hindfoot.
from Zhao D, 2013 [35]) The cavus deformity is often corrected with the first plaster
cast. Then, the foot is abducted to correct adductus and varus

Fig. 4 Manipulative procedure:


a Supinate forefoot to correct
cavus; b Lateral head of talus is
the fulcrum; c Abduct forefoot to
correct adductus and varus;
d Before each cast is applied, the
foot is manipulated
Indian J Pediatr

Fig. 5 Well-molded plaster cast:


a a long leg cast from toes to
groin; b the cast with knee at
flexion of 90° from lateral view

deformity simultaneously with counterpressure applied on the prevent the relapse of deformity. The brace consists of a pair
lateral aspect of the head of the talus. Long leg plaster casts of straight high-top shoes and a connected bar. The shoes
(Fig. 5) from toes to groin are applied immediately to maintain should be placed at the position of abduction with 60 to 70°
the position achieved by manipulation. This progress is repeat- for the affected foot and 30 to 40° for the normal foot. The
ed weekly until the full correction is completely obtained. The connected bar should be at shoulder length and bent at 5 to 10°
total number of cast changes may be five or six. Cautions of with the convexity away from the baby. The brace should be
cast care should be informed by the practicing doctor, especial- worn at least 23 h per day for the first 3 mo, then a total of 14
ly of poor circulation of toes end and cast slipping off. to 16 h per day until the patient is 3 to 4-y-old [37]. In authors’
When the talar head is covered, heel is in valgus, forefoot is institute, they have slightly modified the brace protocol as:
abducted 60° to 70° and the dorsiflexion is less than 15°, the full-time use for the first 3 mo, then 16 to 18 h until the age
PAT procedure is required to correct the residual equinus un- of 2-y-old, and then 14 to 16 h until the end of 4-y-old. Long
der local anaesthesia or sedation (Fig. 6). As reported in the term follow-up of this brace protocol has shown excellent
published studies, up to 70 to 75 % of patients underwent a results of foot morphology and function (Data not yet pub-
procedure of percutaneous Achilles tenotomy [37]. The suc- lished). Skin irritation caused by brace wearing is often a big
cessful indication is that an additional 15 to 20° of dorsiflexion problem in infants. So the feet should be examined several
at the ankle is gained after tenotomy. Then, a long leg cast times a day for the first week, and comfortable shoes with
should be applied for 3 wk with the foot dorsiflexion at application of soft liners are very necessary. The expected
15~20° and abducted at 70°. After removal of the final cast, a outcome for the patients treated with the Ponseti method is
foot abduction orthosis (Fig. 7) is prescribed immediately to that the affected foot should be strong, flexible and pain free,

Fig. 6 Percutaneous Achilles


tenotomy (PAT): a When the talar
head is covered, heel is in valgus,
forefoot is abducted 60° to 70°
and the dorsiflexion is less than
15°, PAT is indicated; b The blade
is inserted at the level 1 to 1.5 cm
proximal to the heel tuberosity
where the Achilles tendon is
attached; c The dorsiflexion of
ankle reaches to 20° to 25° after
PAT with moderate compression
for 1 to 2 min on the cutting site to
control bleeding; d Dressing the
incision
Indian J Pediatr

Fig. 7 Brace and diagram for


FAO brace design: a The α angle
is 60° to 70° external rotation on
the affected side and 30° to 40° on
the normal side, The length
between two shoes (L) is equal to
the shoulder length; b The β
angle is 5° to 10° to hold the feet
at the valgus position; c The γ
angle is more than 15° to keep the
foot at the dorsiflexion;
d Mitchell-Ponseti Brace

but slightly shorter and narrower than the normal foot. Ponseti method soon after birth. Difficulties of the Ponseti
Figure 8 shows the appearance at the pre-treatment and method involve perineum hygiene, skin irritations and anxiety
follow-up in a case, who underwent the management using from Achilles tenotomy.

Fig. 8 A boy with bilateral


clubfeet before and after the
management using Ponseti
method: a & b the prominent
deformities were observed in
bilateral feet before treatment;
c–f good morphology and
function of bilateral feet after
treatment, c dorsal appearance,
d lateral view of ankle at dorsal
flexion, e and f posterior view of
the heels in neutral position with
white arrows indicating the tiny
incision scar from PAT
Indian J Pediatr

compliance is the discomfort in brace wearing. Several novel


types of newly designed foot abduction orthosis (FAO), such
as dynamic foot abduction orthoses [41, 42], unilateral foot
abduction orthoses [43] and comfortable shoe braces, have
been reported with their expectation to improve compliance
or adherence. Furthermore, education of the Ponseti method
and the efforts in encouraging calls and regular patient’s visit
are also very helpful for brace compliance. It is still uncertain
whether the intensified education on bracing compliance
or adherence is required in a certain group of patients
with potential risks for relapsing. Early relapse of defor-
Fig. 9 Clinical tips for recognizing brace compliance: White arrows mity, characterized by the loss of foot abduction and
indicate the slightly narrowing appearance above the ankles. This was dorsiflexion, is still a challenging problem for orthope-
one of our patients, who had worn the brace by 23 hours per day dists to manage. Repeated treatment of serial manipula-
adherently for about 2 mo
tion and casting is the prior choice to correct the relapsed
deformity. Transfer of anterior tibialis is indicated in some
Bracing Compliance of the cases presenting with dynamic supination during
the swing phase of gait, which is usually evaluated after
The bracing phase of the method, which is the only effective walking.
way for relapse prevention, requires the efforts from not only
providers but also patients and parents. General education on
brace compliance is therefore, necessary. The patient educa- Conclusions
tion is very important to ensure the bracing compliance and/or
adherence to the primary requisite of the Ponseti method, Early recognition of congenital clubfoot is critical for
because brace phase is critical in maintaining correction family education and appropriate referral to the pediatric
and preventing relapse despite of a number of variables orthopedic specialist. Early and proper treatment can
reported to be associated with the occurrence of relapse. lead to a good long-term result. Ponseti method of club-
Nowadays, it is suggested in a number of published foot management, one of the conservative, suitable and
studies that non-compliance with the brace regime is currently worldwide-accepted regimens, has a good out-
closely associated with the relapse of deformity [25, come with radical reduction of extensive surgery [24] in
31, 32]. The importance of brace-wearing should be stressed most cases of clubfoot according to the published stud-
to the parents at the beginning of treatment and an internet- ies for the treatment as early as 4 or 5 d after birth and
based platform created by the physicians to introduce the is of significant value in preventing late disabilities in
Ponseti method is of great necessity for parents' education. terms of safety, efficiency, effectiveness and economical
The parents or caregivers should be encouraged to call or visit healthcare resource. Ponseti method requires not only metic-
the clinic if they experience any problems during the ulous attention to the technical details, but also dedicated
brace phase. At each visit to the clinic, the physician care to the post-correction phase of brace wearing,
should ask how many average hours the child keeps whose value in preventing relapse of deformity seems
wearing the brace each day and discuss with the parents to be not often stressed enough. The education of brace
the difficulties of brace wearing. Encouraging calls and importance in patients and parents is the elementary part
shorter interval for follow-up are helpful for brace com- in implementing this method.
pliance to address any problems that the parents may
encounter. A useful clinical tip for recognizing brace
compliance is the slightly narrowing appearance above Contributions YL was responsible for searching the literature and
the ankles (Fig. 9). The most important factor for brace drafting the manuscript, DZ did the data collection and drafted the part
of the manuscript, LZ planned this review and formatted the manuscript,
wearing is to confirm proper seating of the heel in the HL did literature reading and analysis, and XY did data collection and
brace. interpretated the clinical relevance. YL and DZ contributed equally to this
There are many reasons for brace non-compliance such as article.
physician’s poor training in the Ponseti method, poor commu-
nication between physician and parents, low educational level Conflict of Interest None.
of caregivers, annual family income, physical distance and
transport [31, 38–40]. The most common reason for non- Source of Funding None.
Indian J Pediatr

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