Rıdvan DURAN, Üffet VATANSEVER, NÜKHET ALADAĞ ÇİFTDEMİR, Aslı ERKEN, Betül ACUNAŞ

Amniotik bant sekanslı Bir olgu sunumu

Amniotik bant sekans (ABS) ekstremite amputasyonları, konstriksiyon bantları, psödosindaktili, multipl kraniofasyal, visseral ve karın duvarı defektleri ile karakterize, sporadik doğuştan anomaliler içerisinde yer alan bir durumdur. Doğum öncesi kontrollerini düzenli olarak yaptırmamış bir anneden 30 haftalık, 1310 gr olarak doğan ve doğumda resüsitasyon gerektiren preterm kız bebek, multipl doğuştan anomalileri olması nedeniyle kliniğimize sevk edildi. Fizik muayenede antimongoloid görünüm, iki taraşı büyük ve düşük kulak vardı. Sağ elde ikinci ve dördüncü parmaklarda distal interfalangeal eklem hizasında boğum vardı, üçüncü parmak proksimal interfalangeal eklemden ampute idi. Sağ ayakta pes kalkaneus, birinci parmak uç kısmında boğum ve diğer parmaklar arasında sindaktili vardı. Sol ayakta pes ekinovarus mevcuttu ve beşinci parmak dışında diğer parmaklar ampute idi. Diğer dış ve iç organ sistemlerinde tutulum yoktu. Plastik cerrahi ile konsülte edildi. Olgu, ailesinin isteği üzerine postnatal yedinci günde taburcu edildi.

Anahtar Kelimeler:

Süt çocuğu, yenidoğan, hastalıklar, Amniyotik bant sendromu, Ergen, Kadın, Ergenlikte gebelik

Amniotic band sequence: A case report

Amniotic band sequence (ABS) is a sporadic congenital anomaly characterized by amputation of limbs, constriction bands, pseudosyndactylism, multiple craniofacial, visceral and abdominal wall defects. A female preterm infant of 30 weeks, weighing 1310 g and born to a mother who did not receive regular antenatal follow- up, was born with multiple congenital anomalies requiring resuscitation at birth was referred to our clinic. Her physical examination revealed antimongoloid slant, bilateral large and low-set ears, presence of circular constriction bands on second and fourth distal interphalangeal joints and absence of 3rd digit by the amputation of the proximal interphalengeal joint on the right hand, pes calcaneus deformity, constriction band on toe and syndactly of other digits of right foot, equinovarus deformity and amputation of all digits other than 5th digit of left foot. No other external and internal organ involvement was noted. Plastic surgery consultation was made. She was discharged on postnatal day 7 upon the request of her parents.

Keywords:

Infant, Newborn, Diseases, Amniotic Band Syndrome, Adolescent, Female, Pregnancy in Adolescence,

PDF

___

1) Torpin R. Amniochorionic mesoblastic fibrous strings and amnionic bands: associated constricting fetal malformations or fetal death. Am J Obstet Gynecol 1965;91:65-75.
2) Pedersen TK, Thomsen SG. Spontaneous resolution of amniotic bands. Ultrasound Obstet Gynecol 2001; 18:673-4.
3) Bodamer OA, Popek EJ, Bacino C. Atypical presentation of amniotic band sequence. Am J Med Genet 2001;100:100-2.
4) Keswani SG, Johnson MP, Adzick NS, Hori S, Howell LJ, Wilson RD, et al. In utero limb salvage: fetoscopic release of amniotic bands for threatened limb amputation. J Pediatr Surg 2003;38:848-51.
5) Muraskas JK, McDonnell JF, Chudik RJ, Salyer KE, Glynn L. Amniotic band syndrome with significant orofacial clefts and disruptions and distortions of craniofacial structures. J Pediatr Surg 2003;38:635-8.
6) Benacerraf BR, (editor). Ultrasound of fetal syndromes. Philadelphia: Churchhill Livingstone; 1998.
7) Sentilhes L, Verspyck E, Eurin D, Ickowicz V, Patrier S, Lechevallier J, et al. Favourable outcome of a tight constriction band secondary to amniotic band syndrome. Prenat Diagn 2004;24:198-201.
8) Conrad M, Ezaki M. Oligodactyly-diagnoses and patterns of malformation. J Am Soc Surg Hand 2002; 2:110-20.
9) McGuirk CK, Westgate MN, Holmes LB. Limb deficiencies in newborn infants. Pediatrics 2001;108:E64.
10) Patterson TJ. Congenital ring-constrictions. Br J Plast Surg 1961;14:1-31.
11) Hunter AG, Carpenter BF. Implications of malformations not due to amniotic bands in the amniotic band sequence. Am J Med Genet 1986;24:691-700.