Congenital clubfoot: clinical guidelines

List of Abbreviations

ALT – alanine aminotransferase

AST – aspartate aminotransferase

CK – creatine kinase

LDH – lactate dehydrogenase

MMA – multimodal analgesia

MRI – magnetic resonance imaging

RCT – randomized controlled trial

RSCI – Russian Science Citation Index

CT – computed tomography

US – ultrasound examination

Terms and Definitions

Congenital clubfoot is a complex malformation in which the altered external shape of the foot as an equinovarus deformity is a manifestation of bone, joint, neural, and vascular pathology of the lower limb.

Idiopathic congenital clubfoot is a condition in which the cause of the deformity is unknown.

Non-idiopathic congenital clubfoot is a condition in which the cause of the deformity is known. Non-idiopathic forms include neurogenic, teratogenic, and clubfoot associated with genetic syndromes.

Recurrent clubfoot (after conservative and surgical treatment) is the return of the deformity or its components after adequate treatment.

Antibacterial drug, antibiotic – a medicinal product from the group of antibacterials for systemic use (J01).

Beta-lactam antibiotics – a group of antibacterial drugs sharing a common structural element—the beta-lactam ring. They inhibit bacterial cell wall synthesis by binding to penicillin-binding proteins, leading to lysis and death of the microbial cell. According to the Anatomical Therapeutic Chemical classification, beta-lactam antibiotics include the following main groups: penicillins (J01C) and other beta-lactams (J01D), of which first-generation (J01DB), second-generation (J01DC), third-generation (J01DD), and fourth-generation (J01DE) cephalosporins, other cephalosporins and penems (J01DI), carbapenems (J01DH), and monobactams (J01DF) are of greatest clinical significance.

1. Brief information on the disease or condition

1.1 Definition of the disease or condition

Congenital clubfoot is a developmental foot pathology. A normally developing fetal foot becomes clubfooted at approximately the third month of intrauterine development. There is idiopathic congenital clubfoot, a condition where the cause of the deformity is unknown, and non-idiopathic congenital clubfoot, a condition where the cause is unknown. Non-idiopathic forms include neurogenic, teratogenic, and clubfoot associated with genetic syndromes. Clubfoot can be combined with arthrogryposis, spina bifida, diastrophic dysplasia, Möbius syndrome, amniotic band syndrome, congenital metatarsus adductus, and forefoot malformations (adactyly, syndactyly, polydactyly).

To date, among treatment options for congenital clubfoot, the Ponseti method [1, 2] has taken a special place due to its truly worldwide spread. In the 1950s and 1960s, Ignacio Ponseti developed a clubfoot treatment principle based on a detailed study of normal and pathological foot biomechanics [3]. Over the past decade, the majority of orthopedists worldwide have recognized the Ponseti method as the "gold standard" for clubfoot treatment [4, 5, 6]. Treatment consists of three main stages: correction of the deformity through sequential manipulations and plaster casts, percutaneous Achilles tenotomy (closed tenotomy), and maintenance of the achieved result using abduction braces. According to the author and his followers, the efficacy of the method reaches 98 %, which has made it possible to rethink clubfoot as a surgical pathology in principle [7].

1.2 Etiology and pathogenesis of the disease or condition (group of diseases or conditions)

The etiology of clubfoot remains unknown to date. There are many theories, but currently no single unifying theory exists [8]. In the pathogenesis of idiopathic clubfoot, interactions between genetics and environmental factors likely play a role, indicating a multifactorial etiopathogenesis of the disease. Numerous studies using various candidate genes provide information on potential pathways involved in clubfoot pathogenesis, but at this stage, scientists have not been able to find the primary gene causing this condition. Clubfoot has complex inheritance mechanisms, with over 75 % of all cases having no family history [9].

Congenital clubfoot has historically been associated with several risk factors: oligohydramnios, maternal smoking and alcohol consumption, parental age, parental education level, and season of birth. Environmental factors were hypothesized to play a role in pathogenesis. In 2014, a twin study conducted in Denmark (examining 34,485 twins) found evidence for the role of environmental factors. The authors concluded that the role of genetic factors in disease development is insufficient to explain the statistics. They identified compelling evidence for the influence of environmental factors explaining the statistical analysis results [10]. Another study in 2013 [11] was conducted in a rural area of Turkey and showed that parental consanguinity was associated with a higher risk of congenital clubfoot. Although the study sample was small, this result may supplement the etiological hypothesis based on the influence of multiple genetic mechanisms and environmental factors. Although the role of environmental factors has been confirmed by several studies, all proposed factors except smoking were not significantly associated with clubfoot; the effect of the latter is related to oxidative DNA damage caused by tobacco smoke [12, 13, 14, 15].

1.3 Epidemiology of the Disease or Condition

It is estimated that 0.5–2 out of every 1,000 newborns are affected (150,000–200,000 newborns per year and 7–43 clubfoot cases per year per million population), with a male-to-female ratio of 2:1 [16]. For the period 2020–2024, the incidence rate in Saint Petersburg was 0.9 per 1,000 newborns [17].

1.4 Features of Coding the Disease or Condition

According to ICD-10, clubfoot in children is coded based on the presence and type of foot deformity, as well as the etiology of the deformity (Table 1).

Table 1. Nosological forms of clubfoot in children

1.5 Classification of the disease or condition

To date, numerous classifications of congenital clubfoot based on different principles have been proposed [7].

Classification of congenital clubfoot

By etiology:

- Idiopathic:

▪ Typical

▪ Atypical

- Non-idiopathic:

▪ neurogenic

▪ teratogenic

▪ clubfoot associated with genetic syndromes

By the number of affected limbs:

- Unilateral;

- Bilateral.

To assess the severity of clubfoot, the Pirani score [18] Appendix G1 is most frequently used.

1.6 Clinical presentation of the disease or condition

The initial examination of an infant with congenital clubfoot should include all elements of an orthopedic clinical examination. To do this, the child must be completely undressed and placed on a table or couch. Physical examination reveals the following deformity components:

1. Equinus position of the foot.

2. Varus deviation of the hindfoot.

3. Adduction of the forefoot.

4. Cavus.

5. Reduced calf circumference—the triceps surae (gastrocnemius, soleus, plantaris muscles), tibialis posterior, and toe flexors are shortened and reduced in size.

6. A clubfoot typically has a smaller size compared to the healthy foot.

2. Diagnostics, medical indications and contraindications for diagnostic methods

Criteria for establishing the diagnosis/condition: Intrauterine clubfoot can be diagnosed using fetal ultrasound as early as the 13th week of pregnancy, although the diagnosis is more commonly established between the 18th and 24th weeks of pregnancy. Up to 85 % of diagnoses are made by ultrasound between 12 and 23 weeks of gestation.

There is no consensus regarding whether fetal karyotyping (cytogenetic testing [karyotype]) should be offered when isolated clubfoot is diagnosed via prenatal ultrasound [19]. Ultrasound accuracy depends on many factors, such as the physician's experience, gestational age, equipment quality, and technique used. A diagnosis of congenital clubfoot cannot be made in the first trimester of pregnancy, as transient varus positioning of the foot occurs during normal lower limb development [20]. Ultrasound scanning performed between the 20th and 24th weeks of pregnancy has been established as the most reliable for confirming or ruling out a diagnosis of clubfoot. Prenatal ultrasound diagnosis of clubfoot is less accurate in multiple pregnancies [21, 22]. The false-positive rate of prenatal diagnosis varies widely from 0 to 40 % for isolated clubfoot, with a lower (< 10 %) false-positive rate when prenatal diagnosis is performed in specialized centers.

Postnatally, the diagnosis of congenital clubfoot is primarily established clinically based on the combination of inspection and physical examination findings: equinocavovarus foot deformity is noted, and a clinical examination of the patient is also performed to identify associated pathologies.

2.1 Complaints and Anamnesis

The diagnosis of congenital clubfoot is made at birth during the initial neonatal examination based on a combination of equinus, cavus, and varus components of foot deformity that are not passively correctable to the normal range of motion in the respective joints [7, 18].

2.2 Physical Examination

The clinical diagnostic method is primary in establishing the diagnosis and planning treatment. First, it is necessary to determine whether the child truly has congenital clubfoot. At the same time, one must keep in mind other foot deformities that may look like clubfoot at first glance (e.g., congenital metatarsus adductus). Deformity severity is assessed using the Pirani score (Appendix G1. Pirani score).

2.3 Laboratory Diagnostics

Laboratory diagnostic methods have no diagnostic value for congenital clubfoot and are performed as indicated in the presence of comorbid conditions [26].

2.4 Instrumental Diagnostics

Radiological examination (foot radiography in one/two projections) in typical congenital idiopathic clubfoot in infants under one year of age does not provide diagnostic or prognostic information [30]. The indication for radiography in infants under one year of age with congenital clubfoot is a relapse of deformity following successful primary treatment and adherence to the preventive orthoses regimen.

Comments: in children over one year of age, radiographs are performed when a relapse is suspected or present, taken in a weight-bearing (standing) position to assess foot bone alignment, and functional radiographs with maximum dorsiflexion are performed when necessary. If a teratogenic nature of the deformity is suspected (in the presence of associated anomalies), foot radiographs in two projections with maximum passive dorsiflexion are performed. When performing anteroposterior radiographs, the beam is directed at a 15° angle toward the heel to prevent overlapping of anatomical structures of the foot by the lower leg bones. When performing the lateral projection, one should orient to the position of the ankle joint rather than the foot to obtain a correct image of the talus [33, 34]. On the lateral projection in clubfoot, the angle between the long axis of the talus and the long axis of the calcaneus (talar-calcaneal angle) is less than 25°. The long axis of the calcaneus is drawn along the plantar surface. The normal range is 25-45°. On the anteroposterior projection in clubfoot, the talar-calcaneal angle is less than 15° [35].

Comments: studies regarding the use of CT to evaluate the foot in clubfoot are scarce. Disadvantages of CT include the risk of ionizing radiation, unossified tarsal bones, sensitivity of images to motion artifacts, and the need for expensive equipment and software applications for multi-planar reconstruction. On the other hand, complex three-dimensional foot deformity in clubfoot can be better assessed using 3D reconstructions than 2D radiographs. CT can be used to identify deformities and displacements that are obscured by overlapping on conventional radiographs. Furthermore, relationships between hindfoot bones and lower leg bones can be evaluated more precisely. The use of MRI is limited due to its numerous drawbacks, including equipment requirements and patient anesthesia. Conversely, the advantages of MRI over conventional radiography and CT lie in the ability to visualize cartilaginous templates and surrounding soft tissues.

2.5 Other Procedures

3. Treatment, including drug and non-drug therapies, diet therapy, and pain management

3.1 Conservative Treatment

Comments: the technique of manipulation is based on an understanding of foot biomechanics: to correct the position of the hindfoot, it is necessary to manipulate the forefoot and midfoot. The initiating movement in correcting the entire deformity is abduction of the forefoot.

Technique of manipulations

1. Stabilize the talus using the thumb placed over its head. This point becomes the center of rotation around which foot abduction occurs.

2. Manipulate the foot in the direction of supination followed by abduction using the second hand. At the start of treatment, it is necessary to supinate the foot more; however, as correction and abduction increase, supination automatically decreases.

3. Hold the correction with gentle pressure, then release the foot and repeat the maneuver.

   The plaster cast is applied after manipulations and immobilizes the foot in order to stretch shortened ligaments, joint capsules, and tendons.

Comments: long leg casts (up to the groin crease) must always be used to prevent foot rotation at the ankle joint. In infants under one year of age, the knee joint is positioned in up to 90° of flexion within the cast. Cast changes are performed every 7 days. The final cast, applied after tenotomy, is left in place for 3 weeks in younger infants and 4 weeks in infants older than 6–8 months.

Comments: the plaster cast is applied over a thin cotton stockinette/padding. Particular care must be taken in molding the cast around the heel region, malleoli, and plantar surface of the foot.

The following rules should be observed during casting:

• Never hold the heel.

• Never cast with the foot in pronation.

• Avoid external rotation of the tibia/lower leg.

The first plaster cast provides correction of cavus, midfoot inversion, and heel varus, achieving alignment of the forefoot and midfoot in the frontal plane through supination and moderate abduction.

The sequence of manipulations performed by the physician's hands should be as follows:

1. Stabilize the talus by placing the physician's thumb over the lateral aspect of the talar head.

2. Elevate the first ray of the foot and achieve alignment of the forefoot and midfoot in the frontal plane. Then gently abduct the foot.

3. Hold the foot in the achieved correction position while the assistant applies padding and the cast. Adjust the position of the physician's fingers to avoid indentations in the cast and carefully mold the heel region, malleoli, and plantar surface of the foot.

In younger children, correction of cavus usually occurs after the first cast. In severe cavus, this may require 2–3 casts. Subsequent casts provide correction of midfoot inversion and heel varus, and continued correction of cavus if necessary. With each subsequent cast change, foot supination is reduced by increasing abduction. In severe clubfoot with rigid cavus, its correction remains the priority. An indicator of adequate correction is achieving a neutral heel position and foot abduction up to 60–70° in infants under one year of age; in children over one year, the indicator of adequate correction is achieving a neutral heel position and foot abduction up to 40–50°.

Removal of the cast should be performed in the clinic immediately before applying the next cast. Correction may be lost if the foot remains unfixated for more than an hour.

Percutaneous Achilles tenotomy

Comments: performing a tenotomy is necessary in the majority of children, including older ones. Attempts to correct equinus by gradual stretching of the Achilles tendon with casts can lead to compression of the talus and flattening of its trochlea. In some mild cases with slight limitation of dorsiflexion, it is possible to avoid achillotomy. If, after correction of the remaining deformity components, dorsiflexion is greater than 15°, then tenotomy is not indicated.

After performing the tenotomy, dorsiflexion should increase by 10° or more.

Indications for tenotomy:

1. Foot abduction of 60–70°.

2. The heel is in a valgus or neutral position. Tenotomy must not be performed if the heel is in a varus position, as this indicates inadequate correction.

3. Pirani score indicators (see Appendix G1):

   • Lateral part of the talar head — 0 points

   • Midfoot contracture score — less than 1 point

   • Hindfoot contracture score — more than 1 point

Comment: tenotomy is an intervention that does not require an operating room and can be performed in a treatment room. It should be performed by an orthopedic traumatologist and one assistant. The patient's position on the table is supine, with the lower limb in external rotation. Achillotomy can also be performed with the child lying prone. The assistant holds the limb in a position of knee extension and foot dorsiflexion for maximum tension of the Achilles tendon. The scalpel blade is inserted 1 cm proximal to the calcaneal tuberosity from the medial edge of the tendon and parallel to it, with the cutting edge directed proximally. Then the blade is carefully rotated and moved laterally until the tendon is completely transected. A click is felt, and the foot immediately yields in the direction of dorsiflexion. The wound is covered with a sterile wipe, and observation is carried out for 5 minutes to detect any bleeding. In some situations related to the clinical features of the patient, the categorical desire of the parents, and organizational factors, it is possible to perform achillotomy in a hospital setting, including with the use of sedation and general anesthesia.

Comments: the position of the foot in this case should correspond to 60–70° of abduction and 15–30° of dorsiflexion in younger children, and 30–60° of abduction and 10–20° of dorsiflexion in children older than one year. Usually, only one casting stage is needed after achillotomy, but in complex cases (e.g., severe equinus), an additional cast may be required to achieve dorsiflexion or even a neutral foot position. In this case, the cast change is performed 4–7 days after tenotomy, and the final cast is applied for 3 weeks in younger children and for 4 weeks in older children.

The causes of congenital clubfoot recurrences are not yet sufficiently clear [44]. The most obvious causes are non-compliance with the brace-wearing protocol (orthopedic footwear) as well as muscle imbalance [45, 46]. Among other causes, congenital foot anomalies, neuromuscular diseases, and congenital anomalies of the spine and spinal cord are of significance.

The basis of clubfoot recurrence treatment is repeated plaster casting correction according to all the Ponseti method principles described above.

3.2 Surgical Treatment

Indications for surgical treatment in idiopathic congenital clubfoot are quite controversial. Primary recurrences, by general consensus, are subject to retreatment using the Ponseti method, which also includes manipulations, serial plaster corrections (Application of a plaster cast for bone fractures, Application of a circular plaster cast), achillotomy (Achilles tendon repair, Muscle and tendon repair, Closed/open repair of Achilles tendon) (including repeat ones) in case of dorsiflexion deficit, as well as wearing braces (orthopedic footwear) after cast removal.

Comments: The procedure is performed through two approaches on the dorsum of the foot. The medial approach is used to detach the tibialis anterior muscle from its insertion site. The tendon is sutured using a bioresorbable thread. After that, the tendon is passed subcutaneously into the lateral incision and then transosseously through the center of the third cuneiform bone to the plantar surface of the foot. The ligature used to suture the tendon is fixed on the plantar surface of the foot using a Bunnell pull-out technique (with a sterile button or other blocking material). An aseptic dressing and a long-leg circular plaster cast are applied up to the upper third of the thigh with the knee flexed at 120° and the foot in maximum dorsiflexion.

Tibialis anterior tendon transfer for clubfoot does not correct foot deformity; it merely restores the balance between pronators and supinators. All components of the deformity must be corrected prior to the transfer. This is optimally achieved through serial casting, the number and total duration of which are determined by the rigidity of the deformity. In cases where residual foot deformity persists after serial casting, it must be surgically corrected to the necessary extent, which may include joint releases, tenoligamentocapsulotomy, foot bone osteotomies, repositional arthrodeses, as well as correction using external fixation devices (Bone reconstruction. Bone osteotomy using internal fixators and external fixation devices, application of external fixation devices using a compression-distraction external fixation apparatus).

3.2.1 Surgical antibiotic prophylaxis

• In the surgical treatment of patients with clubfoot, it is recommended to perform surgical antimicrobial prophylaxis (SAP) for surgical site infections by administering a single preoperative dose of an antibacterial drug to reduce the risk of infectious complications [67, 68].

Leveled CGraded 5Level of recommendation evidence - C (level of data reliability – 5)

Comments: in accordance with international clinical guidelines for the prevention of surgical site infections [67, 69], the objective of SAP is to achieve bactericidal concentrations of the antibacterial drug in tissues exposed to microbial contamination during surgery in order to prevent surgical site infections. The necessity of SAP is determined by the surgical wound class of the upcoming procedure: SAP is not required for "clean" surgical wounds that do not involve the placement of orthopedic implants and metallic hardware. SAP should be performed for: clean-contaminated and contaminated surgical wounds; and "clean" surgical wounds involving the placement of orthopedic implants and metallic hardware. For infected ("dirty") wounds, SAP is not indicated; instead, antibiotic therapy is administered.

• In patients with clubfoot, it is recommended to use first-generation (J01DB) and second-generation (J01DC) cephalosporins (#cefazolin**, #cefuroxime**) as the primary drugs for surgical antimicrobial prophylaxis in traumatology and orthopedics [67]. As an alternative in case of beta-lactam allergy, glycopeptide antibacterial drugs (vancomycin**), lincosamides (#clindamycin** 10 mg/kg) should be used [67]. The recommended single doses of the primary antibacterial drugs for SAP in traumatology and orthopedics are presented in Table 2.

Table 2. Recommended single doses of primary antibacterial drugs for SAP in traumatology and orthopedics [67, 71, 72]

GRL CLEL 5Recommendation Grade C (Level of Evidence – 5)

Comments: For patients with an unremarkable allergy history and without significant risk factors for methicillin-resistant staphylococci carriage, first-generation (J01DB) and second-generation (J01DC) cephalosporins (#cefazolin**, #cefuroxime**) are used for SAP [67], administered intravenously 30 to 60 minutes before skin incision. In case of beta-lactam hypersensitivity, vancomycin** should be prescribed, administered over at least 60 minutes with the intravenous infusion starting 2 hours before the incision, or #clindamycin** [67]. In the presence of significant risk factors for methicillin-resistant staphylococci carriage (MRS), the SAP regimen should include systemic antibacterial drugs (ATC: J01) with anti-MRS activity (vancomycin**). In most cases, a single preoperative dose of a systemic antibacterial drug is sufficient for effective prophylaxis. For prolonged procedures (exceeding 3 hours) or massive blood loss, an additional intraoperative dose of a systemic antibacterial drug (ATC: J01) should be administered (the approximate timing for intraoperative administration is 2 half-lives after the preoperative dose). Administration of systemic antibacterial drugs (ATC: J01) after wound closure in the operating room is impractical, even in the presence of indwelling drains.

A prospective randomized clinical trial showed that administering an antibacterial drug for SAP 1 minute after tourniquet application demonstrated a statistically significant advantage in reducing the incidence of surgical site infections, wound healing time, and overall patient satisfaction compared to administration before tourniquet application [70].

• In the surgical treatment of patients with clubfoot, it is recommended to perform surgical antimicrobial prophylaxis for a duration not exceeding 24 hours [73].

Leveled CGraded 5Level of recommendation evidence - C (level of data reliability – 5)

Comments: In accordance with the regulatory documentation in force in the Russian Federation (SanPiN 3.3686-21) and clinical guidelines [73], to achieve an effective concentration of the antibacterial drug in tissues and blood serum at the time of skin incision, antibiotics for surgical site infection prophylaxis should be administered intravenously 30 to 60 minutes before skin incision, or within 2 hours before incision in the case of vancomycin**. The maximum duration of prophylactic systemic antibacterial administration should not exceed 24 hours after the end of surgery. In most cases, a single dose of a systemic antibacterial drug is sufficient for effective prophylaxis. Additional doses should be administered during prolonged surgeries when the half-life of the administered systemic antibacterial drug has elapsed since the preoperative dose, creating a risk of the antibacterial drug concentration dropping below the bactericidal level and increasing the risk of surgical site infection.

3.2.2 Postoperative pain management

• To ensure adequate analgesia in patients following surgical treatment of clubfoot, it is recommended to use multimodal analgesia, which may include paracetamol**, NSAIDs (from group M01A Anti-inflammatory and antirheumatic products, non-steroids), and immediate-release opioids (ATC group code N02A) (ketorolac**, diclofenac**), taking into account age restrictions in the instructions for medical use; if such administration is not feasible, monomodal analgesia should be used [74, 75, 76, 77].

GCR BLOE 2Grade of Recommendations — B (Level of Evidence – 2)

Comments: Postoperative MMA may include psychotherapy, physical therapy, NSAIDs, oral paracetamol** (suspension for oral administration) or intravenous paracetamol** (infusion solution), regional anesthesia (single injection or peripheral nerve catheter placement), local injections, and opioids [75, 79].

In contemporary scientific literature, authors emphasize the use of the lowest effective doses of opioids for the shortest possible duration, avoiding the use of extended-release opioids.

It must be kept in mind that the duration of parenteral administration of most NSAIDs from the ATC group M01A "Anti-inflammatory and antirheumatic products, non-steroids" (ketorolac**, diclofenac**) is limited to two days in accordance with the instructions for medical use, which requires timely switching of the patient to oral administration, changing the drug, or coordinating further use with a medical commission. The specific choice of local anesthesia method and medicinal products is determined by the attending physician individually, in accordance with the patient's characteristics, local protocols, and the medical organization's equipment.

When prescribing analgesic therapy for children, a single dose should be chosen taking into account the child's weight and age. Preference should be given to analgesics with oral or rectal administration routes, avoiding intramuscular injections.

• When providing analgesia for children in the postoperative period, it is recommended to use multimodal analgesia, which may include combinations of oral or intravenous paracetamol**, NSAIDs (Diclofenac**, Ibuprofen**, Ketorolac**), opioid and opioid-like (Morphine**, Fentanyl**) analgesics in accordance with the age restrictions in the instructions for medical use [77].

__(UC R)__ C__(LEL)__ 5Level of Evidence Recommendation - C (Level of Evidence – 5)

3.3 Other Treatment

Historically, there are many methods of treating congenital clubfoot. Passive correction methods for congenital clubfoot include various types of serial casting. They are based on the principle of gradual parallel correction of all major components of the deformity (supination, adduction, cavus, varus, and equinus) using serial casts that do not allow limb movement during correction. There are also methods that combine elements of functional treatment and passive correction. Nevertheless, modern evidence indicates the overall superiority of the Ponseti method in treating children with idiopathic congenital clubfoot; therefore, the Ponseti method is recognized as the «gold standard» for treating congenital clubfoot [58], and deviations from its treatment protocol can lead to suboptimal treatment outcomes [40].

In cases of clubfoot relapse in older children, Ponseti management in some cases fails to correct the deformity. In such instances, corrective surgical interventions may be performed. To date, there is no unified approach to treating this type of deformity. Recommended treatments include posteromedial release of the foot joints, foot bone osteotomies, and external fixation devices (either as a standalone method or combined with release or osteotomies). In patients over 12 years of age, corrective arthrodesis of the hindfoot and midfoot joints may be performed.

Comments: to date, there is no sufficiently convincing comparative data confirming the priority efficacy of any of the above methods; therefore, their use is suggested based on the technical capabilities of the clinic and the individual experience of the surgeon [62, 63].

4. Medical Rehabilitation and Sanatorium-Resort Treatment

A critical part of Ponseti treatment is wearing foot abduction braces (orthopedic footwear). After clubfoot correction, the foot must be held in the corrected position for a specific period to prevent relapse. Refusing to wear braces or improper use of them is the most common cause of relapses. Braces must be fitted on the child immediately after the final cast is removed.

Comments: as supportive rehabilitation measures, manual reduction exercises and calf muscle stretching are prescribed (parents are advised to perform these before putting on the braces—stretching the calf muscle for two minutes). Squatting exercises must be performed without lifting the heels off the floor, as this leads to stretching of the tibialis posterior muscle. The exercise is performed for two minutes a day under parental supervision [64, 65, 66].

5. Prevention and Dispensary Observation

Comments: the purpose of follow-up examinations is the timely detection of relapses and other foot problems. The following schedule of follow-up examinations is proposed:

First examination - 1 week after starting brace wear. Pay special attention to the child's tolerance of the braces.

Second examination - 1 month. It is necessary to assess the position of the foot in the braces.

Third examination - 1-3 months, depending on when the reduction of brace-wearing time is scheduled.

Examinations during the first year after treatment completion (i.e., after the removal of the last cast) - every 3 months. It is advisable to schedule follow-up examinations in accordance with the planned stages of changing the brace-wearing time.

Subsequent examinations - every 3-6 months.

Examinations after the completion of the brace-wearing period - annually until the end of the skeletal growth period (i.e., until 12-13 years of age).

6. Organization of Medical Care

Indications for hospital admission to a medical organization:

1) Planned surgical treatment for children with severe and complicated forms of congenital clubfoot.

Indications for discharging a patient from a medical organization:

1) Performed surgical treatment or refusal of surgical treatment for objective reasons;

2) Absence of: persistent pain syndrome; persistent hyperthermia; significant laboratory abnormalities; complications requiring inpatient treatment.

Criteria for Evaluating the Quality of Medical Care

References

Appendix A1. Working Group Composition

Blandinsky Valery Fedorovich, D.Sc. in Medicine, Professor, Head of the Department of Pediatric Surgery at the Federal State Budgetary Educational Institution of Higher Education "Yaroslavl State Medical University" of the Russian Ministry of Health.

Vavilov Maxim Alexandrovich, D.Sc. in Medicine, Professor at the Department of Pediatric Surgery at the Federal State Budgetary Educational Institution of Higher Education "Yaroslavl State Medical University" of the Russian Ministry of Health.

Vissarionov Sergey Valentinovich, D.Sc. in Medicine, Professor, Corresponding Member of the Russian Academy of Sciences, Director of the Federal State Budgetary Institution "H. Turner National Medical Research Center for Children's Orthopedics and Trauma" of the Russian Ministry of Health.

Kenis Vladimir Markovich, D.Sc. in Medicine, Professor, Deputy Director for Innovative Development and Regional Affairs at the Federal State Budgetary Institution "H. Turner National Medical Research Center for Children's Orthopedics and Trauma" of the Russian Ministry of Health.

Klychkova Irina Yuryevna, D.Sc. in Medicine, Head of the General Clinical Department at the Federal State Budgetary Institution "H. Turner National Medical Research Center for Children's Orthopedics and Trauma" of the Russian Ministry of Health.

Kokorev Alexey Ivanovich, Ph.D. in Medicine, Trauma and Orthopedic Surgeon, Head of the Department for Organization of NMRC Activities at the National Medical Research Center for Traumatology and Orthopedics named after N.N. Priorov of the Russian Ministry of Health.

Nazarenko Anton Gerasimovich, D.Sc. in Medicine, Corresponding Member of the Russian Academy of Sciences, Director of the Federal State Budgetary Institution "National Medical Research Center for Traumatology and Orthopedics named after N.N. Priorov" of the Russian Ministry of Health.

Novikov Vladimir Alexandrovich, Ph.D. in Medicine, Head of the Department of Neuroorthopedics, Systemic and Orphan Diseases at the Federal State Budgetary Institution "H. Turner National Medical Research Center for Children's Orthopedics and Trauma" of the Russian Ministry of Health.

Otdeleyonov Vitaly Alexandrovich, Ph.D. in Medicine, Clinical Pharmacologist, Head of the Drug and Medical Device Safety Management Department at the Federal State Budgetary Institution "National Medical Research Center for Traumatology and Orthopedics named after N.N. Priorov" of the Russian Ministry of Health.

Ochurenko Alexander Alekseevich, D.Sc. in Medicine, Professor, Head of the NMRC Functions Implementation Directorate at the Federal State Budgetary Institution "National Medical Research Center for Traumatology and Orthopedics named after N.N. Priorov" of the Russian Ministry of Health.

Sapogovsky Andrey Viktorovich, D.Sc. in Medicine, Leading Research Fellow at the Clinic of Foot Pathology, Neuroorthopedics, and Systemic Diseases at the Federal State Budgetary Institution "H. Turner National Medical Research Center for Children's Orthopedics and Trauma" of the Russian Ministry of Health.

Conflict of interest: none.

Appendix A2. Methodology for Developing Clinical Guidelines

Target audience for these clinical guidelines:

1. Orthopedic surgeons,

2. Pediatricians,

3. Pediatric surgeons,

4. Physical therapy physicians.

Table 1. Scale for assessing levels of evidence (LE) for diagnostic methods (diagnostic interventions)

Table 2. Scale for assessing the levels of evidence (LE) for prevention, treatment, and medical rehabilitation methods, including those based on natural therapeutic factors (preventive, therapeutic, and rehabilitation interventions)

Table 3. Scale for assessing the grades of recommendation (GR) for prevention, diagnostic, treatment, and medical rehabilitation methods, including those based on natural therapeutic factors (preventive, diagnostic, therapeutic, and rehabilitation interventions)

Procedure for updating clinical guidelines.

The mechanism for updating clinical guidelines provides for their systematic revision at least once every three years, as well as upon the emergence of new evidence-based medicine data on the diagnosis, treatment, prevention, and rehabilitation of specific diseases, or in the presence of justified additions/comments to previously approved clinical guidelines, but no more frequently than once every 6 months.

Appendix A3. Reference Materials

1. Pediatric surgery: national guidelines / ed. by A.Yu. Razumovsky. - 2nd ed., rev. and supp. - Moscow : GEOTAR-Media, 2021. - 1280 p. : ill. - (National Guidelines series). - DOI: 10.33029/9704-5785-6-PSNR-2021-2-1-1280. - ISBN 978-5-9704-5785-6.

2. Vavilov M.A., Gromov I.V. [Treatment of congenital clubfoot in infants: study guide for students]. Yaroslavl: YSMU, 2022. 44 p.

3. https://global-help.org/publications/books/help_clubfoottreatmentmanualrussian.pdf

Appendix A3.1

Standard brace-wearing protocol (orthopedic shoes) for children with typical congenital clubfoot following its correction and in the absence of relapse signs.

1. Round-the-clock wear (23 hours a day, removed only for bathing) — 3 months.

2. Monthly reduction of brace-wearing time (1 month — 20–22 hours a day, 1 month — 18–20 hours a day, 1 month — 16–18 hours a day).

3. Brace wear during nighttime and daytime naps (14–16 hours a day — for several months, until independent walking begins).

4. Brace wear during nighttime sleep (12–14 hours a day) — up to 4–5 years of age.

Foot position in braces depending on the onset of independent walking:

Bilateral clubfoot:

Both feet fixed at 60–70° of abduction and 10–20° of dorsiflexion.

Unilateral clubfoot:

The corrected foot is fixed at 60–70° of abduction and 10–20° of dorsiflexion; the healthy foot is fixed at 40° of abduction and 10–20° of dorsiflexion if treatment is initiated before independent walking:

The distance between the heels of the shoes on the brace should approximately equal shoulder width.

Following successful and properly performed clubfoot treatment using the Ponseti method, special rehabilitation treatment is not required. Children wear normal shoes, participate in school physical education, and join sports sections.

Appendix B. Physician Action Algorithms

Appendix C. Patient Information

Information for parents about clubfoot and its treatment

The following information is designed to help you better understand your child's condition, the treatment methods, and how vital your role is in the treatment process. This section will help you navigate this.

Please note: Always discuss any problems, questions, or misunderstandings regarding the recommendations given to you with your doctor if you have them.

What is congenital clubfoot?

Congenital clubfoot is a deformity that develops in utero and is present at birth. Clubfoot can be unilateral or bilateral.

During intrauterine development, a normal foot becomes deformed and develops clubfoot. The tendons and ligaments of the posterior and medial parts of the foot become tight and taut. The muscles become shorter and smaller than normal. As a result, the posterior and medial parts draw closer together, and the foot turns downward and inward. The bones of the foot begin to deform following the muscles. The foot turns and becomes inelastic; the child cannot turn it into the correct position on their own.

When should clubfoot treatment be started?

• Shortly after birth (~ 1 month): — Best option.

• Before the child starts walking: — Highly effective.

• Walking child in early childhood: effective for correcting all or most deformities. Depending on the severity and concomitant pathology, older children may require surgery.

General overview of clubfoot treatment

The manipulations described below are standard procedures for infants and older children with congenital clubfoot. If your child is older or has a concomitant pathology, the treatment may differ somewhat.

1. Manipulation and casting to correct the deformity

• Over several weeks, the foot is gradually brought into the correct position by tissue stretching, and the bones of the foot assume the correct position.

• After each manipulation, the child's foot is placed in a plaster cast for 5–7 days to maintain the foot in this position and stretch the shortened ligaments, joint capsules, and tendons.

• These procedures are repeated 4–6 times. More severe clubfoot requires a greater number of casts.

2. Achilles tendon lengthening and plaster casting

• In most cases, full correction requires lengthening of the Achilles tendon. This procedure is performed under local anesthesia and takes only a few minutes. The Achilles tendon heals completely, allowing the foot to be dorsiflexed as much as necessary.

• Following the procedure, another cast is applied for 3 weeks.

3. Wearing foot abduction braces (orthopedic shoes) to maintain correction

• Once the final cast is removed, your child's feet are placed into braces, which must be worn for 23 hours a day for 3 months. Braces may only be removed for bathing.

• After 3 months, the time spent in braces will be gradually reduced. Eventually, braces will only need to be worn during sleep (12–14 hours a day) until 4–5 years of age.

• Wearing braces is a very crucial part of the treatment. Even if the feet look normal, if the child stops wearing the braces, it will lead to a return of the deformity, i.e., a relapse.

After 7 years of age, relapses are extremely rare.

4. Physical rehabilitation

• Various types of exercises can help maintain correction and promote the child's normal development.

• Various tendon and ligament stretches prescribed by the physician can be performed by the parents themselves.

To achieve the best treatment outcome, all recommendations must be strictly followed.

Recommendations and tips for casting

Every time during a cast change, bring some food or drink for your child, such as a bottle, or their favorite toy to distract them during the casting procedure.

1. After the first casting

Your child may feel uncomfortable. Plaster casts are quite heavy until they are completely dry.

• Roll up a small towel to make a bolster and place it under the knees until the cast dries.

• Distract the child with play, singing, feeding, or anything else they enjoy.

• A few hours later, the child can be picked up and carried while in the cast.

• Soon, once the cast is dry, allow the child to do whatever they usually like to do. Do not worry about damaging or soiling the casts, they are durable enough. In wet weather, make sure the cast is completely dry and hard before allowing the child to crawl.

• When the child is sleeping, always roll up a towel bolster and place it under the knees to reduce pressure on the heels.

2. While the child is in the cast

• Keep the casts clean. Never use water to clean the cast; only wipe away dirt.

• In cold weather, you can put socks over the dry cast to keep it clean and prevent the toes from getting cold.

• Use diapers to wrap the child's legs; the cast must not get dirty.

• Do not bathe the child while they are in the cast.

• To keep the child clean:

1. Lay the undressed child on a towel or sheet.

2. Wet your hands and apply soap.

3. Wash the child with your soapy hands.

4. Gently rinse off the soap using a washcloth, making sure the cast does not get wet.

5. Dry the child with a towel and dress them.

• Check the child's feet regularly.

Check the child's feet several times a day to ensure they are warm and of normal color. This is done by pressing on the child's toe and observing how it refills with blood. If circulation in the foot is good, the toes turn white upon pressure, then quickly return to pink.

• Call your attending physician if:

- The color of the toes does not return to normal.

- The toes are swollen.

- The toes are white, purple, or blue.

- The skin at the edges of the casts becomes very red, irritated, chafed, or develops sores.

- The cast is pinching tightly.

- The cast is cracked.

- The cast is heavily soaked.

- You notice an unpleasant odor coming from under the cast.

- You cannot see the toes (the cast has slipped) – call the physician immediately.

Tips for wearing foot abduction braces (orthopedic shoes)

After the last cast is removed, braces are fitted. It takes a few days for the child to get used to them, usually 5–7 days. Discomfort comes from the removal of the familiar casts, as well as from the inability to move each leg independently.

Never remove the braces in response to the child's crying – they need time to adapt!

The first few days are critical for the child's further adaptation to the braces. If you remove the braces in the first few days, your child will learn that crying can free them from the braces, and this will cause you numerous problems with wearing them.

You must know that refusing to use the braces in the correct position and for the required duration is the main cause of deformity recurrence!

Putting on the braces should become a normal and routine part of the child's daily schedule.

1. How to put on the braces

• Make sure the skin is clean and completely dry before putting on the braces.

• Never use alcohol-based liquids to treat red skin areas; this will only make the problem worse.

• Put the first boot on the more affected foot. Gently dorsiflex the foot and then place the heel into the boot first. Hold the foot in the correct position. Fasten the middle strap first, then fasten the other straps on the boot. Do the same for the second boot.

• Make sure the heel is properly seated in the boot. Use the special viewing holes in the boot to verify that the heel is in the correct position. You should see through the special hole that the heel is resting on the bottom of the boot.

• You must help your child get used to the braces. Play with them and teach them how to move both legs while wearing the braces.

2. Wearing braces 23 hours a day for 3 months

• Remove the braces only for bathing, for no more than 1 hour a day.

3. Reducing brace time to nighttime sleep until 4–5 years of age

• Change the brace-wearing schedule from 23 hours a day to nighttime sleep only with the permission of the attending physician.

• Never allow your child to stand in the braces.

• Perform ligament and tendon stretching exercises if recommended by your doctor.

• Put comfortable, soft shoes on your child when they are walking.

• Do not forget about regular check-ups, even if everything is going well. Always check the braces!

• Your child only needs a boot size upgrade in their braces when their toes completely extend past the edges of the sole.

• Never stop using the braces before your doctor gives you permission.

4. Common problems

• If the child's foot slips out of the braces, check whether the straps on the boots are fastened tightly enough. If you cannot resolve the issue of feet slipping out of the boots, consult your doctor.

• If the child is frequently fussy, make sure there are no chafing or blisters on the heels; if there are, consult your doctor.

• If the child takes off the boots:

a) Put socks on over the boot. This might not stop them, but it will make removing the boot much more difficult.

b) Keep putting the braces on!

• If you notice that the foot deformity is returning, consult your doctor as soon as possible. Do not wait for the next scheduled appointment.

• If the braces break, call your attending physician. New braces must be ordered.

Appendix G. Assessment Scales, Questionnaires, and Other Evaluation Tools

Appendix G1. Severity assessment scale for congenital clubfoot deformity (Pirani scale)

Russian title: Pirani score.

Original title (if applicable): Pirani Score.

Source (official developers' website, validation publication): [18]https://www.physio-pedia.com/Pirani_Score#cite_note-:2-5

Type: rating scale.

Purpose: assessment of foot deformity severity in congenital clubfoot, monitoring the results of conservative treatment (Ponseti method), determining indications for tendo-achilles tenotomy, assessing the completion of the casting phase and the transition to bracing.

Objective of application:

Quantitative assessment of the severity of foot deformity.

Interpretation of treatment outcomes at various stages.

Determining the need for surgical intervention (tenotomy).

Determining the time of completion for cast correction.

Scientific research on the effectiveness of treatment methods.

Assessment frequency: before treatment, during treatment (at each cast change), after treatment completion during follow-up examinations.

Content (template):

Pirani Scale

Chart completed during treatment using the Ponseti method

Key (interpretation):

General principles of assessment using the Pirani score:

• 6 clinical signs of a clubfoot are compared with a normal foot;

• 3 signs indicate hindfoot deformity;

• 3 signs indicate midfoot deformity.

Each symptom is scored as follows:

0 = no foot deformity;

0.5 = moderate foot deformity;

1 = severe foot deformity.

The higher the total score, the more severe the deformity.

Notes:

1. Posterior skin crease above the ankle joint (reflects the degree of equinus contracture).

To assess it, the foot must be held in a position of moderate correction while visualizing the posterior region of the heel.

0 points: Multiple shallow creases are visible that do not alter the contour of the heel region. The creases smooth out upon dorsiflexion of the foot.

0.5 points: One or two deep creases that slightly alter the contour of the heel.

1 point: One or two deep creases that significantly alter the contour of the heel.

2. "Empty heel" also reflects the degree of equinus contracture. If the talus is in an equinus position, the calcaneus also assumes an equinus position. In this case, the calcaneal tuberosity is displaced upward relative to the soft tissues of the heel region. After correction of the equinus position of the talus, the calcaneal tuberosity returns to its normal position.

To check this sign, the foot must be held in a position of moderate correction. The examiner's fingers are placed on the bisector of the angle formed by the plantar surface of the foot and the posterior surface of the lower leg, applying slight pressure to the tissues.

0 points: The calcaneus is easily palpable (palpatorily comparable to palpation of the temporal region).

0.5 points: The calcaneus is palpable deep within the soft tissues (palpatorily comparable to palpation of the tip of the nose).

1 point: Palpatory sensation of an "empty heel." Bone structures are not palpable (palpatorily comparable to palpation of the tip of the thumb).

3. Fixed equinus also reflects the degree of equinus contracture.

To assess it, the knee joint must be held in full extension and the foot in slight supination. The foot is then gradually brought into maximum dorsiflexion. When viewed from the lateral side, the angle between the axes of the lower leg and the foot is determined.

0 points: Normal dorsiflexion – greater than 5°.

0.5 points: The foot is brought only to the neutral position (range from 5° plantar flexion to 5° dorsiflexion).

1 point: The foot is not brought to the neutral position (no more than 5° of plantar flexion).

In addition to the Pirani scoring, it is advisable to note the angle of maximum dorsiflexion of the foot.

4. Medial crease of the foot reflects the degree of tissue contracture along the medial surface of the lower leg and foot.

The foot is held in a position of moderate correction while examining the medial border of the foot.

0 points: Multiple shallow skin creases along the medial border of the foot that do not alter its contour.

0.5 points: One or two deep creases that slightly alter the contour of the foot.

1 point: One or two deep creases that significantly alter the contour of the foot.

5. Lateral part of the talar head — reflects the degree to which the navicular bone is centered on the talar head. In congenital clubfoot, the lateral part of the talar head does not articulate with the navicular bone. As the deformity is corrected during treatment stages, the navicular bone repositions and covers the lateral aspects of the talar head.

To check this sign, the foot must first be placed into the deformed (clubfoot) position, and the lateral aspects of the talar head are palpated. Afterwards, the examiner uses the other hand to abduct the foot while palpating the degree of reduction of the navicular bone.

0 points: The lateral aspects of the talar head disappear from palpation due to complete coverage by the navicular bone.

0.5 points: The lateral aspects of the talar head are partially palpable due to incomplete coverage by the navicular bone.

1 point: The lateral aspects of the talar head are freely palpable even at maximum foot abduction due to a fixed medial subluxation of the navicular bone.

In addition to scoring using the Pirani scale, it is advisable to note the angle of foot abduction.

6. Curvature of the lateral border of the foot — reflects the degree of tissue contracture along the medial surface of the lower leg and foot. The degree of curvature can reflect the severity of this contracture.

This sign is determined by examining the foot from the plantar aspect. A straight object (e.g., a pencil) is placed along the lateral border of the rearfoot.

0 points: The lateral border of the foot has a straight contour from the heel to the head of the fifth metatarsal bone.

0.5 points: The lateral border of the foot is moderately curved. The apex of the curvature is located distally at the level of the metatarsal bones.

1 point: The lateral border of the foot is significantly curved. The apex of the curvature is located at the level of the calcaneocuboid joint.