Trimorphic extreme clubfoot deformities and their management by triple surgical skin expanders- DOLAR, DOLARZ and DOLARZ-E (evidence based mega-corrections without arthrodesis)

Congenital club foot is common, but remains unsolved and the literature quotes up to 95% incidence of residual adduction and inversion, Tarraf & Carroll [9]. There are numerous, diverse, confusing, terms in the literature, e.g. neglected, recurrent, relapsed, residual, resistant, recalcitrant, severe, rigid, syndromic, isolated, with/without surgical scars, from child to adults etc., having commonality in treatment failures. Millions of DALYs are wasted with clubfoot related GBD [17], especially in developing countries. The author has been researching in this grey area for more than 40 years, achieving a breakthrough. A generic name “extreme deformities” has been given to multiple terms describing severe/rigid deformities, having three hierarchic grades each 3D (trimorphic): Gr1 having partially flexible joints mostly children; Gr2 slightly mobile joints, mostly adolescents/adults and Gr3 with little or no mobility, mostly adults; with some overlapping. Incomplete understanding of complex 3D patho-anatomy, primarily of skin and consequently deeper tissues, has been considered responsible for all failures (Table 1).

This research is in three stages/generations, with distinct timeline and completely evidence based. First generation technique in two parts: Part 1 was started around the mid-1970s by studying the patho-anatomy of clubfeet in stillborn foetuses, from skin to deeper tissues with double purpose: Firstly, to clarify reported patho-anatomical controversies; some blaming muscle defects primarily [7, 22], others attributing bones [10, 19] or combined muscles/bones [3, 20, 24] and still others TN subluxation/dislocation [20, 24], but none mentioning skin; secondly, to consolidate opinion about anatomical hurdles causing failures. Fifteen clubfeet were dissected, 16 was the highest number reported till then [19], mostly one or two dissections. Skin contracture in 3D with smaller room was discovered as the primary cause and jammed soft tissues/osteo-articular structures as secondary, which was constantly reinforced with clinical innovations. Without skin expansion, deeper correction is unthinkable. Subcutaneous skin expanders implantation have been reported in clubfoot with limited role in children [18], not in extreme deformities. Part II: A new surgical technique, DOrsoLAteral Rotation skin flap (DOLAR) in Gr1 deformities, expanded skin in 3D, permitting extensive soft tissue release with comfortable realignment of deeper tissues. Anatomical dissections/clinical case series were documented in reputed peer reviewed journals [IJO1981;15-2;129-35 & JFAS(AP)2014;1(2):72-77] and author recieved the best original research award in 1988 from Indian orthopaedic association. Al-Khooly etal 2013 [26] cited with complete reference in their bibliography and successfully used surgical technique of author's 1st Gen i.e. DOrsolateral LAteral Rotation (DOLAR) skin flap (Internat orthop- SICOT 1987) for correction of resistant clubfeet, but maintained initially for 4 weeks with an external fixator instead of plaster cast, with excellent/good results. Others cited this report and used modified fascio-cutaneous flaps and reported with limited role in children only and not in extreme deformities [11,13].

Second generation technique: With increasing awareness, Gr2 extreme deformities in older children/adolescents/adults started presenting for treatment. The focus was again on further 3D skin expansion, incorporating one to three Z-plasties in DOLAR incision depending upon severity, with more extensive deeper dissection. Triple arthrodesis could be avoided in the majority. This was named the DOLARZ technique (DOLAR + Z-plasty). A large series was completed with long term follow up and highly satisfied patients. The author received another National Award from Indian Orthopaedic Association in 2013 and published an article in International journal [Int Orthop 1987;11(3):189-92.PMID:3623754].

Third generation technique: With more awareness, Gr3 deformities in children, adolescents and adults with scars/other complications, started presenting. Therefore, V-Y plasty was added to DOLAR + Z incision (three-in-one expansile incision), besides additional procedures: a small Thiersch graft, a distant relaxing incision and non-skin surgical steps. Hence, this was named DOLARZ-E, E stands for extended.

The literature is full of reports with innumerable conservative/surgical procedures and their modifications. Ponseti’s casting technique with percutaneous TA tenotomy [17], is good if started within the first month, with the right regimen and long term bracing in small children only, but not in extreme deformities. Others having limited role in children are PMR, PSTR and CSTR [23‐25], CC fusion [8], TMT mobilisation [12], TP, Tib.A transplants [7, 23], Illizarov techniques [6]. Complex problems of residual deformities management in childhood operated clubfeet with deteriorating function in adulthood have been highlighted [4]. In extreme deformities, arthrodesis or talectomy have been recommended [4, 17, 24]. Polydactyly and osseous coalitions [5, 21] can also occur with clubfoot, creating difficulties. The primary aim has been to treat, hitherto neglected “extreme deformities”, achieving a longer/flexible foot without fusion with high acceptability.

Surgery was performed in varied extreme deformities at all ages, with over 50% older children, adolescents and adults. The earliest age for surgery was about one year (walking age), with the majority of older children (8 years & above), adolescents/adults coming in recent years. Each case was assessed clinically and radiologically by TC angle [2] for TN subluxation/dislocation. Steindler’s plantar release was done in all; modified Evan’s procedure was done mostly after four years, sometimes earlier.

Well padded BK POP cast is given with foot in under-correction to forestall circulatory embarrassment, fully corrected at two/three fortnightly casts. Total immobilisation is for 2 to 3 months.

Assessment criteria: Patient satisfaction level; based on foot length, flexibility, looks and function has been considered for grading results, rather than any scoring system, because “each clubfoot is different” with evidence based countless variables due to severity, rigidity, differing deformity components at birth, varying with age, diverse surgical scars, additional congenital defects, above complicating issues and radiological under-corrections with normal looking operated foot. There are three patient satisfaction grades: excellent, good and fair/ poor. Results: In about 40 years, 1080 surgeries have been performed with adequate follow up in 800 feet, from six months to 30 years, and in 600 ft more than five years with an overall average of 12½ years. There was excellent/good patient satisfaction in 96%, which included insignificant marginal skin necrosis in 3%, not affecting the results (evidence based- BJPS , 1981, Apr;34(2):215-20). There was fair/poor patient satisfaction in 4% with complications of recurrence/overcorrection with or without infection.

Heterogeneous 3D skin contractures, have been considered as the most important obstacles in good correction. These may be only congenital (primary) or complicated with scars (secondary). In a smaller container, the contents have to be squeezed in, like a gymnast in small box or a closed contractured fist. Rotation flap, Z & VY-plasties are popular plastic surgery procedures and have been innovatively used as three-in-one skin expanders in clubfoot, enlarging the skin chamber longitudinally, horizontally and vertically. Addition of V-Y plasties in Gen3 technique is also evidence based on plastic surgery reports PMID 7236984 and many more.

These procedures offer numerous advantages: excising mega scars, better deeper release, restoring osteo-articular relationships, closing DL wedge defect, minimising osseous resections for maximum foot length, retaining mobility without arthrodesis. Unusual neglected cases, common in developing countries are also correctible to improve their psycho-socio-economic status, helping in reducing GBD.

Subtotal subtalar release: Multiple steps of extensive soft tissue/osseous release, meant for repositioning of talus vis-à-vis other bones is acronymed as SSR. Leaving some TC attachments (subtotal) in extreme deformities is considered better than Turco’s PSTR (1971) partial (without lateral) release or Simon’s CSTR, complete release (1985). In SSR, talus is adequately manipulable, more than partial, but less than complete and cannot go out of control. A new perspective for equinus, i.e. flexion deformities at five levels, described above, is important for total correction. Rarely, old standing Gr3 deformity in adults with EQ5 in one case, required ITFS division to retrace talus, enabling lesser osseous resections with excellent result (Table 1: case 14, Fig. 5d1,d2).

Unusual complications: AMC defies all corrections [1], a complicated adult AMC with plantar horn, was corrected by these techniques (Table 1: case 12 ci-viii). Another unique case, probably the first one in a literature review, with eight toes, IT & TMT coalitions and many other defects (octopus clubfoot) has been corrected with a highly satisfied patient in spite of 2½” limb shortening (Fig. 4 Table 1: case 8bi-ix) Fig. A case of stress fracture too has been corrected with high patient satisfaction (Table 1:case 9, Fig. 4ci-iv). GR, a compensatory postural mechanism in under-corrected clubfoot with unsightly gait, may have severe knee symptoms and early OA and need correction. Two cases with significant knee symptoms had correction of feet, knees also got corrected (Table 1:case 7, Fig. 2e&f. Traumatic ischaemic rigidity or osseous coalitions with healed scars, are easily corrected by these techniques (Table 1, Fig. 5: cases 14 di,ii/case 15, ei-v). They all need correction, having tremendous physico-psycho-socio-economic problems for family and society.

Assessment criteria have been based on patient satisfaction level, because “each clubfoot is different” with myriad variables depending upon: deformity components, age, weight bearing, syndromic/ others, previous surgeries, complications, radiological osteo-articular differences (Table1,Case2,CC & TN dislocations). No prevalent assessment criteria can be applicable.

Its strengths are: 1. Evidence based surgical techniques, consistently reinforced by clinical innovations for more than 40 years; 2. Long term follow up in a large diverse series with high percentage of excellent & good results and no adverse effect on corrections; 3. High patient satisfaction with a longer, flexible without fusion, good looking, better functioning foot and improved socio-economic status; 4. Effective for every type, rarest of the rare clubfoot, at all ages and; 5. The latest innovative addition of V-Y plasty (remaining superficial to deep fascia) in Gen3 technique is completely evidence based with minimal chances of skin necrosis with experience. There are many reports from India and abroad in plastic surgery journals proving good capillaries, arterioles, venules, lymphatics and neural networks deep and superficial to deep fascia with numerous perforators (Ponten B 1981 PMID7326984, Bhattacharya V doi. https://​doi.​org/​10.​4103/​0970-0358.​73424) with cross references. Meticulously performed, the Three-in-One techniques, are completely evidence based achieving the best possible cosmetic and functional corrections with minimal chances of failures.