Congenital constriction bands

Abstracts

A síndrome da banda de constrição congênita é uma patologia rara, de ocorrência ocasional na natureza e não existe predisposição genética. É freqüentemente associada a amputações de dedos ou membros, sindactilia, acrossindactilia (sindactilia fenestrada), mal formações em face, tórax e/ou abdômen. O presente trabalho representa nossa experiência na abordagem e tratamento da Síndrome da Banda de Constrição Congênita. Foram tratados 10 pacientes atendidos em nosso serviço em um período de 20 anos.

Defeito Congênito; Mão; Bandas de Constrição

ARTIGO ORIGINAL

Congenital constriction bands

Arlindo G. Pardini Jr.^I; Marcos Antônio dos Santos^II; Afrânio D. Freitas^III

^IHead, Hand Surgery Service, Hospital Ortopédico, AMR

^IIResident, Hand Surgery Service, Hospital Ortopédico, AMR

^IIIHand Surgeon, Hospital Ortopédico, AMR, Belo Horizonte; Hospital Maria Amélia Lins (FHEMIG)

SUMMARY

Congenital Constriction Band Syndrome is a rare pathology, of occasional occurrence in the nature and genetic predisposition doesn't exist. It is frequently associated with amputations of fingers or members, sindactily, acrossindactily (fenestrated sindactily), malformations in face, thorax, and/or abdomen.

The present work represents our experience in the evaluation and treatment of the Constriction Congenital Band Syndrome. We have treated and assisted 10 patients in our service in a 20 year-old period.

Key Words: Congenital Defect, Hand, Constriction Band.

INTRODUCTION

The congenital constriction band syndrome is an unusual pathological condition and its etiology is still controverse.^{1,3,5,6,7,9,10,11} Clinical presentation is varied, from simple constriction rings in fingers and toes present in amputations, syndactyly and/or acrosyndactyly (fenestrated syndactyly) to associated malformations of the face, head and trunk, as cleft lip, anencephalia, encephalocele, hidrocephalus, microphtalmia, thoracoschisis, extrathoracic heart and gastroschisis.^{1,3,4,5,6,8,10}

The constriction band syndrome has a number of synonyms in the literature as: congenital constriction ring, annular constriction band, A.D.A.M. (amniotic deformity, adherences, mutilations) complex, amniotic band rupture complex, Streeter dysplasia, congenital annular defect and amniotic band syndrome.^1,2,10

There is consensus concerning the fact that it is a non-genetic pathology, occurring occasionally in the nature,^3,5,6,10,11 and that limb assymetric involvement is a rule; no similar lesions are seen in different individuals, and symmetric lesions do not occur in the same individual.^8,10,11 Incidence was mentioned in recently published papers as 1:2000 to 1:15000 live births. ^1,3,5,6,9,10 Male and female children^5,10 and all ethnic groups³ are equally affected. Among the children born with alterations caused by the congenital constriction band some authors observed high incidence of prematures and low weighted newborns.^1,10

Several theories were put forward to explain the amniotic bands etiopathogeny, and the most accepted are the intrinsic and the intrinsic theories. Streeter proposed an intrinsic theory defending that the congenital constriction band would represent an inherent defect of the embryogenic development. The constriction bands would be the result of a defect in the differentiation of the germinative plasma, which would form fibrotic bands and cause necrosis.^{1,2,3,5,9,10,11} Torpin defended an extrinsic theory: rupture of the amnion forms threads of amniotic tissue which intertwine with the fetus limbs causing constriction bands; considering that the fetus growth is quicker than the tissue, constriction, strangling, necrosis and amputation would occur^{1,2,3,4,5,9,10,11} Also supporting the intrinsic theory, it has been postulated that the moment during pregnancy when amniotic rupture occurs accounts for more or less severe lesions. Early ruptures would cause severe lesions in the fetus head, trunk and frequently would be the cause of abortion or stillbirths and amniotic ruptures; later ruptures would primarily result in involvement of the limbs.^3,10 One of the causes of amnion rupture during pregnancy, also supporting the intrinsic theory, has been considered the temporary oligodramnion.^1,3,4,10,11

It is common to observe at birth the association of areas strangled by constriction bands, stumps, syndactyly, acrosyndactyly and hypoplasia of fingers and toes^2,8,10,11 In the upper limbs, the distal regions are more affected, mainly the central fingers (II, III e IV) and in the lower limbs the hallux and the second toe are the most affected.^1,5,10,11 The thumb is rarely affected, since it is protected by the palm during pregnancy.¹ In syndactyly and acrosyndactyly caused by constriction bands, bone fusion is rarely observed, occurring only fusion in the soft parts.^10,11

The fibrous bands can be superficial or deep and may totally or partially embrace an affected limb or finger. Superficial bands normally do not cause neurovascular damage or difficulty to lymphatic return, avoiding lymphedema. Deep bands, on the other hand, can cause neurovascular damage of variable intensity, demanding at times urgent surgical intervention due to intensification of the progressive distal edema in the compression area which presents vascular involvement.^1,8,9,10,11 Peripheral nerve lesions due to deep constriction bands (in general proximal to the wrist), have been reported in the literature and are classified as axonotmesis and neurotmesis. The segment of the lesioned nerve is the nervous tissue under the fibrous band. The nervous tissue proximal and distal to the constriction band presents normal macroscopic aspect.^7,10

Early diagnosis of the congenital constriction band syndrome can be made using ultrasound after the first three months of pregnancy.^3,6,10

Treatment, in the cases when surgical intervention is necessary, is made using Zplasty, Wplasty^{1,5,8,9,10,11} or ressection of the fibrous ring with flap rotation of the subcutaneous fat and closure of the skin.^8,11 All the procedure can be carried out in a single surgical intervention^5,9 or, as most authors recommend, half of the circumference is liberated from the constriction ring in one occasion and the other half in a second occasion^1,8,10,11 six to twelve weeks after the first intervention.¹¹

MATERIAL AND METHODS

Ten patients studied and analyzed in the Hand Surgery Service, Hospital Ortopédico, from 1979 to 1999 (twenty years) represent our experience in approaching and treating the Congenital Constriction Band Syndrome.

Three patients were females (30%) and seven were males (70%). The localization of the constriction bands was more frequent in the distal segments of the limbs, mainly fingers II, III and IV, involving also the hand (at the metacarpus), one case, the forearm, two cases, and the arm, one case (Figs. 1,2,3,4). We detected bilateral incidence in four patients (40%), three in the right limb (30%), and three in the left limb (30%). Involvement of the thumb was observed in one patient (Fig. 5). Involvement of the lower limbs was observed in two patients (20%) (Fig. 6).

No consanguinity was present among the parents. Normal to term delivery was the rule for seven children (70%); only one (to term) Cesarian section and one normal premature delivery. One of the children was adopted and no data about delivery or gestation were available. Mean gestation time was 41 weeks, from 36 to 42 weeks. No accidental or deliberate intake of drugs during pregnancy was reported, except drugs currently administered during gestation prescribed by the obstetrician. One mother reportedly had a lower limb X-ray at the end of the third month of pregnancy.

Deformities associated to the Congenital Constriction Band Syndrome were amputation of fingers, six cases (Figs. 1,2,⁷,⁸); syndactyly, five cases (Fig. 9); radio-ulnar synostosis, one case; brachisyndactyly, one case (Fig. 1); triphalangeal thumb, one case; and gigantism in one finger, one case.

The pathologies associated to the constriction band were interventricular communication (C.I.V.), one case; and radial nerve paralysis, one case (Fig. 4).

Nine patients were white (90%) and one black (10%).

Average time from birth to corrective surgery, in the cases when surgical correction of the constricting rings was necessary, was 9.1 months, the earliest case 2 months and the latest 23 months after birth. Surgery was not necessary in one patient, and two were submmitted to surgical procedures other than to liberate the constricting ring. In five patients, other procedures were effected to correct syndactyly.

Surgical treatment to liberate constriction rings was based on stretching the skin and subcutaneous Zplasty (formation in "Z" of the fibrous bands) (Fig. 10).

The indication for surgical treatment was obtained following standards based on the clinical assessment of the patients (Table I). Usually, there were not alterations proximal to the constriction band. Alterations were present under the constriction ring (one case of deep and circular constriction band with radial nerve lesion) and distal to it (Fig. 4). In the cases of superficial or deep constriction rings (complete or incomplete) without lymphedema or neurovascular alterations, the conservative treatment was chosen with periodic evaluations. In cases of superficial or deep constriction rings (complete or incomplete) with neurovascular lesion and/or progressive lymphedema, surgical treatment was indicated.

Surgical approach was effected through liberation of the dorsal half of the constriction band circumference in the complete cases, and liberation of the volar half was carried out 12 weeks after the first surgery.

RESULTS

The aim of this study was to point out our experience in approaching, assessing and treating this rare condition which overtakes the doctors in their practice accompanied by aflicted and worried parents due to the deformities which are present and the possibility that they occur again in a future pregnancy.

Clinical history of pregnancy, delivery, consanguinity, use of drugs or incidents during pregnancy were minutely investigated.

We found a discreet predominance (2:1) of male children. White children were also more commonly seen (nine white and one black).

The association of constriction bands with syndactyly is very frequent and the treatment of this condition can be postponed until a more adequate age, about one year.

We made an inventory of all lesions present as well as of the associated conditions, scheduling the treatment. Priority was given to liberation of the constriction rings coexistent with nervous, vascular lesions or progressive lymphedema; one child was operated on when two-month old due to lesion in the radial nerve (Fig. 4). Pre-operatively we observed that this lesion was restricted to the tissue immediately below the constriction and in spite of the segmental neurectomy followed by neurorraphy under a magnifying glass, we did not succeed and, a posteriori, tendinous transferences were effected (Fig. 11).

Our option was liberation of the constriction rings in two separate interventions with a 12-week interval and no early or late post-operative neurovascular complications were observed.

DISCUSSION

The Congenital Constriction Band Syndrome is a rare condition and its etiology is not completely defined.^{1,3,5,6,7,9,10,11} The clinical presentation is extremely varied, with cases of face, thorax and/or abdomen associated lesions.^{1,3,4,5,6,8,10} Frequently, this condition is associated to the amputation of fingers, syndactyly, acrosyndactyly and lower limb involvement. ^2,8,10,11

We agree with several authors that this condition lacks a genetic component and that it occurs sporadically. ^3,5,6,10,11

Although the literature states that the incidence is equal in males and females^5,10 and in all ethnic groups,³ we found a higher occurrence in male and white children.

In our series of patients, the most frequently found associated deformities were amputations, mainly of the II, III and IV fingers, and syndactyly. The thumb was affected in only one patient. These findings are consistent with those in the literature.^1,5,10,11

We agree that evaluation and classification of the constriction rings concerning the total or partial involvement of the limb, and whether there is involvement of deep planes, are matters of fundamental importance. This procedure permits to evaluate the presence of neurovascular injury and consequently distal involvement and the need of surgical intervention^1,8,9,10,11 The nerve lesion found in our patient was neurotmesis, consistent with the literature which mentions these lesions as neurotmesis and axonotmesis.^7,10

Though considered effective,^3,6,10 we do not have experience with ultrasound to prenatally diagnose the Congenital Constriction Band Syndrome.

Surgical treatment, when necessary, was effected using Zplasty. We did not experience Wplasty or ressection of the fibrous ring with flap rotation of subcutaneous fat as some authors describe^1,5,8,10,11 We do not agree in liberating all fibrous ring in a single surgical procedure as some preconize,^5,9 but instead in liberating the dorsal half of the constriction ring in one occasion, to improve the venous and lymphatic drainage, and the other half 12 weeks later. This procedure is also recommended in the literature,^1,8,10,11

CONCLUSION

The Congenital Constriction Band Syndrome is a rare pathological condition, of occasional occurrence in the nature and without genetic involvement. It is frequently associated with intrauterine amputations of fingers or limbs, syndactyly and acrosyndactyly (fenestrated syndactyly). There can be involvement of the thorax, abdomen and face. Assymetry of the lesions is a rule and involvement of the lower limbs may occur. The fingers II, III and IV are the most affected; the thumb is generally spared. Deep lesions near the wrist can cause nerve lesions.

Indication to surgical approach is made after assessing the child and observing neurological, vascular or lymphatic involvements.

Treatment of the cases with surgical indication is based on the constrictive rings liberation through Zplasties, allowing improvement of the skin and subcutaneous tissue extension.

One must have in mind that other surgical procedures may be necessary to correct associated deformities, mainly syndactyly and acrosyndactyly.

REFERENCES

This study was carried out in: Hospital Ortopédico - AMR - Rua Prof. Otávio Coelho de Magalhães, 111 - Belo Horizonte - MG - CEP 30210-300

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• 6. Quintero, R.A., Morales, W.J., Kalter, C.S. et al: In utero lysis of amniotic bands. Ultrasound Obstet. Gynecol 10: 316-320, 1997.
• 7. Uchida, Y. & Sugioka, Y.: Peripheral nerve palsy associated with congenital constriction band syndrome. J. Hand Surg. (Br) 16: 109-112, 1991.
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Publication Dates

• Publication in this collection
  17 May 2006
• Date of issue
  June 2001