Clubfeet and Constriction Band Syndrome - Case Report

Background: Clubfeet and constriction band syndrome is very rare nonidiopathic condition. Treatment is often dicult and the recurrence deformity rate is high. The purpose of this study was to assess the effectiveness of Ponseti method in treatment of congenital constriction band syndrome accompanied clubfoot deformity and lymphedema. Case presentation: We are presenting an interesting case of bilateral clubfeet and congenital circumferential constriction band syndrome in lower limb. Ponseti method of correcting the congenital clubfoot deformity was applied. Constriction band release is accomplished by two stage completely excising the ﬁ brous band and multiple two stage Z-plasties on the right calf. Conclusion: The results of this study indicate that the Ponseti method of gentle, systematic manipulation and weekly cast changes is effective treatment of nonidiopathic clubfoot distal to congenital amniotic constriction band.


Introduction
The constriction amniotic band syndrome (CABS) is a collection of fetal malformations associated with brous bands that appear to entangle various fetal parts in utero, leading to deformation malformation, or disruption (1,2). The amniotic band syndrome occurs sporadically and the incidence is one case per around 15,000 live births.
It is characterized with compression in the soft tissue usually involving the deep fascia surrounding the leg at the time of birth (3,4). The fact that there are many theories that attempt to explain the etiology of amniotic band syndrome suggest that etiology of this condition remains unknown (1).
An associated clubfoot deformity has been reported to occur in between 12% and 56% of patients with CABS (4,5). The involved foot may occur below an ipsilateral band, or appear in a limb without a proximal band. Some authors have found the clubfeet that are associated with this syndrome to be rigid and resistant to nonoperative management, and the majority of patients in a number of early series were treated by extensive surgical release (5)(6)(7) Clubfoot combined with circumferential amniotic band syndrome is distinguished from other types of congenital deformity because of risk of multiply relapses (6,8,9). According to Ponseti under ultrasound examination a clubfoot is rarely detected before the 16th week of gestation (10). When a clubfoot deformity is suspected during prenatal ultrasound screening this should lead to more thorough search for co-morbidity (11).
This case is reported because of its extreme rarity of bilateral congenital constriction band syndrome accompanied clubfoot deformity and lymphedema. The purpose of the present study was to evaluate the results of the Ponseti method for the treatment of clubfoot associated with constriction band syndrome.

Case Presentation
A male child of Caucasian origin, was brought to our hospital at the age of 5 days with circumferential congenital constriction rings just above of the ankle joint, and severe clubfeet. The pediatric examination revealed no other abnormality in upper extremities or other organs. Pregnancy was uneventful, but during a routine ultrasound examination at 18 weeks of gestation, the gynecologist noticed bilateral clubfoot deformities and informed the parents (Fig. 1). The child was born by a normal vaginal delivery at full term with cephalic presentation. There was no family history of congenital anomalies On inspection, both feet had inversion at the subtalar joint, equinus and varus in the ankle joint, adduction of the forefoot, pronation of the forefoot in relation to the ankle joint, cavus (excavatum), internal rotation of the crural region. (Fig. 2) The right foot : a constriction circumferential ring, type II in Patterson classi cation, was located about 4.0 cm above ankle joint without neurologic de cit, but with dorsal lymphedema. The toes of the right foot were hypoplastic. There was also a severe clubfoot deformity grade 4 according to Dimeglio classi cation with marked medio-tarsal crease.
The left foot: a circumferential constriction band, type II in Patterson classi cation, was located above 3.8 cm the ankle but without lymphedema. The clubfoot deformity was grade 4 according to Dimeglio.
There wasn't noticed any limb-length discrepancy.
Clubfeet were corrected with Ponseti method as a safe and effective procedure. During treatment of clubfoot 3 phase were essential: reduction of deformation (2 months), consolidation of obtained results (4 months) and manage the risk for relaps(3 months).
A long-leg-cast was applied with knee exed 90 . The cast was changed every week with gradual correction of the deformity according to Ponseti protocol. Cavus, adductus and varus was fully corrected but dorsi exion was limited for 20 degrees bilaterally so the tenotomy of Achilles tendon was indicated. Because of lymphedema from amniotic band syndrome on the right foot, we decided to cut the constriction bands in order to releasing the tourniquet-like effect. Percutaneous Achilles tenotomy was performed bilaterally and three longitudinal incision were made through the constriction band, on the right foot. Of course, later reconstruction procedure was necessary. There was no bleeding complications following percutaneous tendo achilles tenotomy (Fig. 3B). The post-tenotomy casting remained for 3 weeks with changing the cast casts every 10 days.
After Ponseti procedure the foot abduction brace (FAB) protocol was applied to maintain the correction: the brace protocol included 23 hours a day at 70 0 of external rotation for three months, then reduced to 18 hours a day and then removed gradually, one hour a day until a use of 12 hours a day. After walking age, the brace was worn at night (Fig. 3C).
The patient was followed until 2 years old, and there was no relapse of the deformity.

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A two-stage circumference excising of the congenital constriction band was done by plastic surgeons, with a three months interval between stages (Fig. 5). The rst stage of surgery was done at age of 13 months and 3 weeks, and the second stage three months later. There was no wound complication. The lymphedema was withdrawn.

Discussion
In our case clubfeet associated with constriction bands were successfully treated with Ponseti method and multiple Z plasties. Tada et al reviewed a series of 83 patients with congenital annular constricting bands and found 19 children with clubfeet (4). Askins and Ger reported a 49% association of congenital band with clubfeet (12). Most of the clubfeet associated with CABS that have been reported in the literature to date have been managed by extensive soft tissue release. Auington et al assessed the outcome of treatment in 18 patients with clubfeet distal to a congenital band, the majority of which were treated using extensive surgical release (13). Syndromic clubfeet have been reported to be resistant to nonoperative management, and complete releases are routinely required. In patients with constriction band syndrome, Hennigan and Kuo (6) noted 62% good results. The results of surgery have often been inconsistent.
Recently, because of the goog outcomes with idiopathic clubfeet, the Ponseti method has been used for non-idiopathic cases, also. Advantage of the Ponseti method are: High quality reduction of clubfoot with the restoration of a sub-normal anatomy, low cost and small displeasing worry for the parents. But, Jackson et al concluded that clubfoot associated with constriction band required more casts to achieve an acceptable correction and had an increased risk of deformity recurrence compared with subjects with isolated clubfoot (15).
Constriction circumferential band on the right calf in our case was operated by plastic surgeons in two stages, during the rst stage anterior part of constriction band was release and in the second stage posterior part.