Acta Obstet Gynecol Scand 55: 179-182, 1976


CONGENITAL ANNULAR CONSTRICTIONS DUE TO AMNIOTIC BANDS M. Isacsohn, Y.Aboulafia, B. Horowitz and N. Ben-Hur From the Departments of Pediatrics (Head: S . Freier), Obstetrics and Gynecology (Head: Z . Palti) and Plastic Surgery (Head: N . Ben-Hur), Shaare Zedek Hospital, Jerusalem, Israel

Abstract. Amniotic bands which become involved with fetal parts, especially the extremities, are producd by rupture of the amnion during pregnancy with the consequent union of the extra-embryonic mesoderm in fibrous strings. An infant with the amniotic band syndrome, in whom an annular constriction of the left leg and right hand was found, is described. He was treated successfully by multiple Z-plasties and a plaster cast to correct the clubfoot. The oedema of the leg persisted for 5 weeks and then subsided completely. The role of amniotic abnormalities in the production of congenital malformations is reappraised. The microscopic and histological investigation of the placenta and membranes should accompany every case of fetal malformation in abortuses, stillbirths,-and affected newborn.

Although congenital fetal anomalies associated with abnormalities of the fetal membranes have been described in the literature for more than 300 years, there i s still some doubt that they are etiologically related (Willis, 1%2; Streeter, 1930). According to Torpin (1968) the amnion may rupture during gestation and the extra-embryonic mesoderm, together with the separated amnion, may form fibrous bands. Various fetal structures, such as the extremities or the umbilical cord, may be entangled, resulting in the production of annular constriction or amputations. If the umbilical cord is constricted, fetal death will result. The present report is of an infant with amniotic band syndrome, suffering from congenital annular constrictions of the left leg and the right hand, which were treated successfully. CASE REPORT 0. M. a 22-year-old primigravida, was admitted to the delivery room in her 39th week of pregnancy. Her

antenatal course had been uneventful and except for vitamin and iron supplements for prevention of anemia, no medication had been.administered. Her blood group was B.Rh.-positive. On examination, the abdomen corresponded to the gestational age, the fetal heart beats was normal, the cervix was dilated 2 cm, and the amniotic sac was ruptured. The delivery was uneventful except for somewhat slow progress (I5 hours from the time of arrival), for which an oxytocin drip was given at 7 cm dilatation of the cervix. A male weighing 3 OOO g was born with an Apgar score of 10 after one minute. The placenta was expelled 3 minutes later. On examination, the placenta appeared normal, though a band of dense tissue was found to emerge from the fetal surface of the placenta. This band surrounded the umbilical cord near its attachment to the placenta. The histological examination of the band showed dense connective tissue which was found in some areas (Fig. 1) to be necrotic. The dense tissue was covered by amniotic epithelium. Examination of the infant revealed a gestational age of 42 weeks. On the left lower leg, immediately above the ankle, a deep circular constriction was found. The skin and soft tissue was absent and the tibia and fibula were exposed. The left foot was completely flail and showed marked clubbing (Fig. 2) as well as severe oedemadistal to the constriction. The skin on the leg was warm. The nail and the third phalange of the second and the third fingers on the right hand were missing. The skin over the palm and fingers was wrinkled. Radiographic examination showed a mild deformity of the distal part of the left tibia and fibula. The distal phalanges of the second and third fingers of the right hand were absent. No other anomalies were observed. The child was operated on at the age of one day. Under tourniquet control, the medial half of the constriction was excised down to the bone and no muscles or tendons could be identified. Skin and subcutaneous tissue were sutured by multiple Z-plasties and the leg put into a plaster cast to correct the clubfoot. The would healed satisfactorily and oedema decreased steadily. Three weeks later, the remaining half of the annular constriction was operated on in a similar fashion. After excision of the constricted area, a Acra Obsret Gynecol Scand 55 (1976)


M . Isacsohn et al.

Fig. 1 . A band of dense tissue emerging from the fetal

surface of the placenta.

considerable amount of clear oedematous fluid gushed out from the wound edges. Again, no muscles or tendons could be detected. The postoperative course was uneventful and at the end of 2 weeks, the oedema of the foot had almost completely subsided.

sclerosis and sloughing off the tissue which results in formation of fibrous bands. Torpin (1968) suggested that amniotic bands arise from the early rupture of the amnion without injury of the chorion. Fibrous bands are found on the detached amnion and the chorion. Protruding fetal parts such as fingers and limbs, may be entrapped by the bands and gradually become constricted and even amputated. In addition, absorption of amniotic fluid by the amnion-denuded chorion may occur and contribute to the triad of defects associated with amniotic bands: intra-uterjne amputations, clubbing of the feet, and lymphedema distal to the side of constriction. The absence of the third phalange and the third finger of the right hand only, in our case, may be explained by an early amputation probably due to the same cause as the annular constriction of the leg, namely amniotic bands. Syndactyly and amputations of fingers due to amniotic bands have been reported in a human fetus at an age as early as 12 weeks (Ornoy et al., 1974). Congenital annular constrictions are conveniently classified into five degrees of severity. ( I ) Only a shallow groove is present, which usually requires no treatment. (2) The groove is deeper and encroaches to a varying extent on subcutaneous tissues or muscles; function is usually normal. (3) The constricting band deepens to the bone. Oedema of varying degree is present distal to the constriction as an expression of impaired venous and lymphatic return. Sensation may be disturbed and, if the band is on the leg, clubfoot is usually present. (4) Symptoms are the same as in (3), but show in addition, pseudarthrosis of the bones, tibia, or tibia and fibula. ( 5 ) Intra-uterine amputation.

DISCUSSION The formation of amniotic bands and the entanglement of various parts of the fetus, especially the extremities, with consequent developmental deformities of the fetus are ascribed to the complete or the partial separation of the amnion from the chorion. The spectrum of the deformities varies from minor defects such as syndactyly to complete amputation of a limb or even fetal death due to constriction of the umbilical cord. According to Streeter (1930) amniotic bands are due to developmental defects occumng at the forIllation of the germ disc, and the fetal meKhranes. Imperfect development leads to a localized Acta Obstet Gynecol Scand 55 (1976)

Fig. 2. A deep circular constriction is seen on the left lower leg with clubbing and edema of the leg.

Congenital annular constrictions

Fig. 3. The nail and the third phalange of the second and third finger of the right hand are missing.

An interesting question is how the part distal to the constriction gets its blood supply in degrees (3) and (4). Stevenson (1946) postulated blood supply through the bones, although it seems more probable that collateral circulation is established through branches of the peroneal and anterior tibial vessels which, in their distal parts, lie immediately over the bones and the interosseous membrane. Several cases of amniotic band deformities have been reported in children born to mothers who have taken L.S.D. (lysergic acid diethylmide) (Zellveger et al., 1967). Although Tjio et al. (1%9) could not find definite evidence to prove that L.S.D. damages human chromosome, chromosome aberrations have been described in human fetuses which have been exposed to L.S.D. in utero. In our case, a normal chromosomal karyotype 46 XY was found. An antivitamin compound, Citral, when injected into a 3-day-old chick embryo, was found to induce congenital malformations, some of them associated with amnniotic fibrous bands and adhesions between the amnion and the embryo (Abramovici, 1972). The teratogenic mechanism appears to be a local effect of Citral that acts simultaneously on embryonic tissue and the adjacent area of embryonic membranes. Degrees (4) and (5) of the congenital annular constriction require early surgical treatment to improve circulation and to reduce the oedema which, in time, might become so severe as to obstruct the anatomical features of the affected extremity. Longstanding edema may lead to induration, infection and gangrene. After excision of the constricted ring, the skin


and soft tissue are united in zig-zag fashion by rotating multiple local triangular flaps into the suture line (multiple Z-plasties), thus avoiding a long straight scar which, by postoperative constriction, could recreate the original constriction. With constriction degrees (3) and (4), the operation is done in two stages, with an interval of several weeks, so as not to compromise the already precarious blood supply to the distal part. Thus, the operation reduces considerably the distal oedema, or even eliminates it completely. Additional orthopedic procedures are required to treat the clubfoot (casts, tendon lengthening, arthrodesis). After the reduction of the oedema by Z-plasties, the degree of sensory disturbance usually determines the ultimate fate of the affected part, since a completely anesthetic foot is prone to repeated ulceration, infection, and might eventually require amputation.

ACKNOWLEDGEMENT We wish to thank Dr Asher Ornoy, Department of Anatomy, Hebrew University Hadassa Medical School, for valuable guidance and criticism, and Dr L. Dolbergfor radiological interpretation.

REFERENCES 1. Abramovici, M.: The Teratogenic Effect of Cosmetic

Constituents on Chick Embryo, Drugs and Fetal Developments (ed. Klingberg, Abramovici & Chemke), p. 161, Plenum Press New York, 1972. 2. Blackfield, H. M. & House, D. P.: Congenital constricting band of the extremities. Plast Reconstr Surg 8: 101, 1951. 3. Baker, C. J. &Rudolf, A. J.: Congenital ring constrictions and intrauterine amputations. Am J Dis Child 121: 393-400, 1971. 4. Glessner, J . R.: Spontaneous intrauterine amputation. J Bone Joint Surg45-A:351, 1%3. 5. Chemke, J., Graf, G., Horwitz, N. & Liban, E.: The amniotic band syndrome. Obstet Gynecol 41: 332, 1973. 6. Ornoy, A., Skeles, E. & Sadovsky, E.: Amniogenic bands as a cause of syndactily in a young human foetus. Teratology 9: 129, 1974. 7. Sarnat, B. G . & Kagan, B. N.: Prenatal constricting band and pseudoarthosis of the lower leg. Plast Reconstr Surg47: 547, 1971. 8. Streeter, G. L.: Focal deficencies in fetal tissues and their relations to intrauterine amputations. Contrib Embryol22: 1, 1930. 9. Stevenson, Th. W.: Release of circular constricting scar by Z plast. Plast Reconstr Surg I: 39, 1946. Acta Obstet Gynecol Scand 55 (1976)


M. Ismsolin

rt (11.

10. Tjio, J . H.,Pahnke, W. M. & Kurlanda, A.: L.S.D. and chromosomes. JAMA 210: 849, 1%9. 1 I . Torpin, R.: Fetal Malformations Caused by Amnion Rupture During Pregnancy. Charles C. Thomas Publisher, Springfield,, Ill., 1%8. 12. Zellveger, H., MacDonald, J . S. & Albo, J.: Is lysergic acid diethylamide a teratogen? Lancet 2; 1066, 1967.

Acta Obstet Gynecol Scand 55 (1976)

1974 SubmittcdforpublicationAu~usr4.

M . Isacsohn Shaare Zedek Hospital P.O.B. 293 Jerusalem Israel

Congenital annular constrictions due to amniotic bands.

Amniotic bands which become involved with fetal parts, especially the extremities, are produced by rupture of the amnion during pregnancy with the con...
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