| 词条 | Constriction ring syndrome |
| 释义 |
| name = Constriction ring syndrome | synonyms = ADAM Complex, amniotic band sequence, amniotic band syndrome, amniotic disruption complex, amniochorionic mesoblastic fibrous strings, congenital amputation, constriction band syndrome, congenital constriction bands, Streeter bands, tissue bands | image = Amniotic band hand.jpg | caption = An infant's hand affected by constriction ring syndrome | pronounce = | field = | symptoms = | complications = | onset = | duration = | types = | causes = | risks = | diagnosis = | differential = | prevention = | treatment = | medication = | prognosis = | frequency = | deaths = }}Constriction ring syndrome (CRS) is a congenital disorder with unknown cause. Because of the unknown cause there are many different, and sometimes wrong names. It is a malformation due to intrauterine bands or rings that give deep grooves in, most commonly, distal extremities like fingers and toes. In rare cases the constriction ring can form around other parts of the fetus and cause amputation or even intrauterine death (see Types and Classification). The anatomy proximal to the site of constriction (or amputation) is developmentally normal.[1][2][3] CRS can be associated with other malformations with club foot being most common.[4] [5][6](see also Types and Classification). The precise configuration of the bands, lymphedema, and character of the amputations are not predictable and vary with each individual patient. Also more than one extremity is usually affected, and it is rare for only one ring to present as an isolated malformation with no other manifestation of this syndrome.[1][2][7][8] CauseThere are three different theories to the cause of the constriction ring syndrome. The first is the intrinsic theory, which was proposed by Streeter in 1930, implicates an anomaly in germ plasm resulting in the defects.[9] This theory is reinforced by the clinical presentation of the constriction rings with other internal visceral and systematic anomalies.[9] Because of these other anomalies the names “Constriction Ring Syndrome”, “Constriction Band Syndrome” and “Streeter Bands” are given to this defect/disease. The second theory postulates the involvement of an intrauterine disruption during pregnancy followed by a cascade of events involving amniotic rupture. When spontaneous rupture of the amnion occurs early in the second trimester, the separation of amnion from chorion produces many small, thin strands that can become entangled within digits and toes.[9][10] The names “Amniotic Band Syndrome”, “Amniotic Disruption Complex", "Amniochorionic Mesoblastic Fibrous Strings", are based on this theory. The third theory postulates the involvement of intrauterine trauma. Intrauterine trauma could be something like amniocentesis, or something like a fetal surgery. An intrauterine trauma could result in hemorrhage leading to acrosyndactyly. One study also showed the presence of bands as confirmed by sonography after fetal surgery.[9][11] Because of these different theories, there are many names for this syndrome. For a long time people believed the second theory about the amniotic rupture and strands. In the research cases not every child had a real (amniotic) strand. It could be that there has to be another explanation for the development of these anomalies. DiagnosisThe diagnosis of constriction ring syndrome can be confirmed with an ultrasonography. The clinical manifestations can be extremely variable. It could be a single or multiple manifestation. This can be confirmed at the end of the first trimester or at the beginning of the second trimester.[12] But not every patient will be diagnosed at that moment, most will get this diagnosis at birth. Differential diagnosisThe differential diagnosis includes;
“Adams-Oliver syndrome is often mislabeled as CRS and consists of cutis aplasia of the scalp in which a longitudinal defect can vary in size and can often be associated with full-thickness skullcap loss. The distal digital or toe hypoplasia-aplasia is often confused with CRS. Constriction rings with or without edema are not present. The digital or toe hypoplasia-aplasia usually contains diminutive nails or nail folds”.[7] ClassificationThe constriction ring syndrome is a complex collection of asymmetric congenital anomalies, in which no two cases are exactly alike.[15] This is why a classification is difficult to make. The most widely used classification system was proposed by Patterson. This classification system is based on the severity of the syndrome and is useful because, the different types require different treatments. Other clinicians have amended this scheme by separating the depth of the ring into mild, moderate, severe and amputation [2][24][16] and by further defining the presence or absence of lymphedema or soft tissue loss distal to the ring.[3] Expanding over subdivision in depth of the clefts for every classification is not necessary because the principles of treatment and technique for correction are the same [7] There are four categories:
Patterson divided the constriction ring associated with acrosyndactyly into three types:
Patterson I; There are simple constriction rings which are strands most commonly around the distal extremities as fingers and toes. In general, the thumb is not likely to be affected by a constriction ring because the fetus typically holds the thumb in tight adduction flexion, making entanglement with strands less likely.[1] These malformations need to be surgically removed which must be executed in different stages and can done by different techniques (see also treatment). Patterson II; The CRS involves strands which obstruct the lymphatic vessels and thus causing fluid retention, distal of the affected extremity. This utters itself with swollen parts distal of the constriction. Patterson III; In this form there is a complex form of syndactyly named acrosyndactyly, the fingers (or toes) were initially separated but due to the constriction they are formed back together. Sometimes multiple fingers can be involved. The distal fusion between digits or toes never initially involves a skeletal coalition.[7] The digits are usually hypoplastic if multiple digits are involved. When the constriction cuts off the blood supply to the fingers, the fingers can form a peak with the most palmar digit being the index finger. Normal neurovascular bundles are not present in the distal parts.[7] Hands with fused fingers need to be released in phases to preserve the distal blood supply. Paterson IV; One of the most severe consequence of constriction strains is probably intrauterine amputations, this is where the constriction goes as deep as the bone and cuts of the blood supply of the proximal extremity. The result will be that the developing toe or finger will become ischemic and will fall of. Because the end result is a transverse amputation that cuts off the vascular supply to the developing extremity,[1] the actual constriction ring is not seen [7] This can result in different outcomes;
Intrauterine death; In extremely rare cases a strain can form around the umbilical cord and cut off the blood supply to the fetus which will result in intrauterine death. Malformation associated with constriction ring syndrome; The percentage of associated anomalies varies from 40% to as high as 80% [15][17][18] Constriction ring deformities are as common on the lower extremity as on the upper, almost all of these involve the musculoskeletal system, with clubbed feet being the most common in up to 30% of reported cases [6][11][17][19] Large reported series reveal an incidence between 5% and 15% of craniofacial malformations with clefting of the lip or palate.[13][20][21][22][23][24][25] TreatmentSurgical correction is recommended when a constriction ring results in a limb contour deformity, with or without lymphedema.[26] Surgical techniqueAt the beginning of the surgery a tourniquet will be applied to the limb. A tourniquet compresses and control the arterial and venous circulation for about 2 hours. The constriction band must be dissected very carefully to avoid damaging the underlying neurovasculature. When the constriction band is excised, there will be a direct closure. This allows the fatty tissue to naturally reposition itself under the skin.[27] “With complete circumferential constriction bands, it is recommended that a two-stage correction approach be used. At the first operation, one-half of the circumference is excised and the other one-half can be excised after three to six months. This will avoid any problems to the distal circulation in the limb, which may already be compromised. Lymphedema, when present, will significantly improve within a few weeks of the first surgery.” [27] For the direct closure of the defect after dissecting a constriction band there are two different techniques:
In rare cases, if diagnosed in utero, fetal surgery may be considered to save a limb that is in danger of amputation or other deformity. This operation has been successfully performed on fetuses as young as 22 weeks.[28] The Melbourne's Monash Medical Centre in Australia, as well as multiple facilities in the United States of America, have performed successful amniotic band release surgery. EpidemiologyThe reported incidence of constriction ring syndrome varies from 1/1200 and 1/15000 live births.[18] The prevalence is equally in male and female.[11] Fetomaternal factors like prematurity, maternal illness, low birth weight and maternal drug exposure are predisposing factors for the constriction ring syndrome.[11] No positive relationship between CRS and genetic inheritance has been reported.[7] References1. ^1 2 3 {{cite journal |author=Patterson T |title=Congenital ring constrictions |journal=Br J Plast Surg |issue=4 |pages=1–31 |year=1961}} 2. ^1 2 {{cite journal |vauthors=Ogino T, Saitou Y |title=Congenital constriction band syndrome and transverse deficiency |journal=J Hand Surg Br Vol |volume=12 |pages=343–348 |year=1987 |doi=10.1016/0266-7681(87)90185-9}} 3. ^1 {{cite journal |vauthors=Weinzweig N, Barr A |title=Radial, ulnar and median nerve palsies caused by a congenital constriction band of the arm: single stage correction |journal=Plast Reconstr Surg |volume=94 |pages=872–876 |year=1994 |doi=10.1097/00006534-199411000-00022}} 4. ^{{cite journal |author=Montogomery W |title=Observations on the spontaneous amputation of the limbs of the fetus in utero with an attempt to explain the occasional cause of its production |journal=Dublin J Med Chem Sci |issue=1 |pages=140 |year=1832}} 5. ^{{cite journal |vauthors=Light TR, Ogden JA |title=Congenital constriction band syndrome. Pathophysiology and treatment |journal=Yale J Biol Med |issue=66 |pages=143–155}} 6. ^1 {{cite journal |author=Pillay VK |title=Intrauterine amputations and annular limb defects in Singapore |journal=J Bone Joint Surg Am |issue=47 |pages=514–519 |year=1965}} 7. ^1 2 3 4 5 6 7 8 {{cite book |vauthors=Gupta A, Kay SP, Scheker LR |title=The Growing Hand: Diagnosis and Management of the Upper Extremity in Children |chapter= 205 |pages=185–212}} 8. ^{{cite journal |vauthors=Moses J, Flatt AE, Cooper R |title=Annular constricting bands |journal=J Bone Joint Surg Am |issue=61 |pages=562–565 |year=1979}} 9. ^1 2 3 {{cite journal |author=Upton J |title=Constriction Ring Syndrome |journal=J Plastic surgery |pages=185–213 |year=2006}} 10. ^{{cite journal |vauthors=Graf JL, Bealer JF, Gibbs DL |title=Chorioamniotic membrane separation: a potentially lethal finding |journal=J Fetal Diagn Ther |issue=21 |pages=255–258 |year=2006|display-authors=etal}} 11. ^1 2 3 4 {{cite journal |vauthors=Foulkes GD, Reinker K |title=Congenital Constriction Band Syndrome: A seventy-Year Experience |journal=Journal of Pediatric Orthopaedics |volume=14 |pages=242–248 |doi=10.1097/01241398-199403000-00021}} 12. ^{{cite journal |author=Nardoza LMM |title=Prenatal Diagnosis of Amniotic Band Syndrome in the Third Trimester using 3D Echo |journal=J Clin Imaging Sci |issue=2 |pages=22 |year=2012}} 13. ^1 {{cite journal |vauthors=Van Allen MI, Curry C, Gallagher L |title=Limb body wall complex: I Pathogenesis |journal=American Journal of Medical Genetics |volume=28 |pages=529–548 |year=1987 |doi=10.1002/ajmg.1320280302}} 14. ^{{cite journal |vauthors=Van Allen MI, Curry C, Walden CE |title=Limb body wall complex: II Limb and spine defects |journal=American Journal of Medical Genetics |volume=28 |pages=549–565 |year=1987 |doi=10.1002/ajmg.1320280303}} 15. ^1 {{cite journal |vauthors=Temtamy SA, McKusick V |title=Digital and other malformations associated with congenital ring constrictions |journal=Birth Defects Orig Artic Ser |issue=14 |pages=547 |year=1978}} 16. ^{{cite journal |vauthors=Garza A, Cordero JF, Mulinare J |title=Epidemiology of the early amnion rupture spectrum of defects |journal=Am J Dis Child |volume=142 |pages=541–544 |year=1988 |doi=10.1001/archpedi.1988.02150050079037}} 17. ^1 2 {{cite journal |vauthors=Light TR, Ogden JA |title=Congenital constriction band syndrome: Pathophysiology and treatment |journal=Yale J Biol Med |issue=66 |pages=143–155 |year=1993}} 18. ^1 {{cite journal |vauthors=Kawamura K, Chung KC |title=Constriction Band Syndrome |journal=Hand Clin |volume=25 |pages=257–264 |doi=10.1016/j.hcl.2008.10.007}} 19. ^{{cite journal |author=Poswillo D |title=Observation of fetal posture and causal mechanism of congenital deformity of palate, mandible and limbs |journal=J Dent Res |volume=45 |pages=584–596 |year=1966 |doi=10.1177/00220345660450032301}} 20. ^{{cite journal |author=Causabon J |title=Congenital band about the pelvis |journal=Plast Reconstr Surg |issue=71 |pages=120–122 |year=1983 |doi=10.1097/00006534-198301000-00027}} 21. ^{{cite journal |vauthors=Coady MS, Moore MH, Wallis K |title=Amniotic Band Syndrome: the association between rare facial clefts and limb ring constrictions |journal=Plast Reconstr Surg |issue=101 |pages=640–649 |year=1988 |doi=10.1097/00006534-199803000-00010}} 22. ^{{cite journal |vauthors=Eppley BL, David L, Li M |title=Amniotic band facies |journal=J Craniofac Surg |issue=9 |pages=360–365 |year=1998|display-authors=etal}} 23. ^{{cite journal |vauthors=Hudgins RJ, Edward MS, Ousterhout DK, Golabi M |title=Pediatric neurosurgical implications of the amniotic band disruption complex |journal=Pediatric Neursci |volume=12 |pages=232–239 |year=1985–1986 |doi=10.1159/000120257}} 24. ^{{cite journal |author=Van der Meulen J |title=The amniotic band syndrome |journal=Plast Reconstr Surg |issue=13 |pages=1087–1090 |year=1999 |doi=10.1097/00006534-199903000-00068}} 25. ^{{cite journal |vauthors=Jones K, Smith DW, Hall BD |title=A pattern of craniofacial and limb defects secondary to aberrant tissue bands |journal=J Pediatr |volume=84 |pages=90–95 |year=1974|display-authors=etal |doi=10.1016/s0022-3476(74)80559-7}} 26. ^1 {{cite journal |vauthors=Tan P, Chiang Y |title=Triangular flaps: A modified technique for the correction of congenital constriction ring syndrome |journal=Hand Surgery |volume=16 |pages=387–393 |year=2011 |doi=10.1142/s021881041100576x}} 27. ^1 {{cite journal |vauthors=Choulakian MY, Williams HB |title=Surgical correction of congenital constriction band syndrome in children: Replacing Z-plasty with direct closure |journal=Can J Plast Surg |issue=16 |pages=221–223 |year=2008}} 28. ^{{Cite news |url=http://www.abc.net.au/news/stories/2008/06/08/2268374.htm |title=Surgeons save unborn baby's legs |publisher=Australian Broadcasting Corporation |work=News Online |date=2008-06-08}} External links{{Medical resources| DiseasesDB = 32457 | ICD10 = {{ICD10|P|02|8|p|00}} | ICD9 = {{ICD9|762.8}} | ICDO = | OMIM = 217100 | MedlinePlus = 001579 | eMedicineSubj = orthoped | eMedicineTopic = 561 | MeshID = D000652 }} 2 : Congenital disorders|Syndromes |
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