Anaesthesia and Sirenomelia (Mermaid Syndrome)

Abstract

fied, but the liver and spleen were normally situated. X-ray of the lower limbs showed a normal looking pelvis, femurs, tibiae and fibulae, and abnormal feet. Full blood count, urea and electrolytes were normal.Neither the paediatric surgeon nor anaesthesiologist had prior experience of this clinical problem and on a Sunday, access to a reference library was not possible. A colostomy, and if possible a stoma for urinary drainage, was planned.Anaesthesia was induced with halothane and intubation achieved without muscle relaxants. In view of the easily palpable sacral hiatus, a caudal block using 2.5ml 0.25% bupivacaine was performed. A block to the level of T8 was achieved.Laparotomy revealed bilateral renal agenesis, an absent urinary collecting system and no bladder. The rudimentary kidneys seen on ultrasound were in fact the adrenal glands. In view of hopelessness of these findings further surgery was abandoned. The gravity of the situation was explained to the mother. The infant was kept comfortable and discharged at mother's request to die at home.A second case of sirenomelia was admitted to the paediatric surgical unit at King Edward VIII Hospital a year later. In view of the hopeless prognosis, no surgery was offered. SynopsisA 2.4kg one day old, the product of a 36-week monozygotic twin pregnancy born to a 24 yr old primigravida by C-section for foetal distress, presented for laparotomy. The child's mother had attended antenatal clinic once during her otherwise uneventful pregnancy. No antenatal investigations were performed. The monozygotic twin sibling, a male weighing 2.65kg, was completely normal.On examination of the abnormal twin, the most striking feature was the complete fusion of the lower limbs from buttock with no perineum to the heels, and a single 5th toe (sympus); there were also no external genitalia or urogenital orifice, and an imperforate anus. The feet were inverted, the popliteal region was dimpled and the sole of the feet faced forward. The feet were fused along the lateral border and resembled a flipper (dipus) (Figure1 and 2). The rest of the examination, from the umbilicus upwards, was completely normal.Preoperative ultrasound of the abdomen revealed what was thought to be rudimentary kidneys. No bladder could be identi-A Bösenberg Figure 1. Monozygotic male twins. The typical features of sirenomelia are present in one -fused lower limbs (dipus) with flipper like feet, absence of external genitalia, imperforate anus and normal upper torso. Features of Potter's facies are not present. Figure 2. Lateral and posterior views of the sirenomelia. The sacral hiatus was easily palpable despite the abnormal buttock. The feet are rotated inwards and fused along their lateral borders with a single 5th toe.

Anesthetic Management of a Patient With Sirenomelia A Case Report

“…(6) Sirenomeli etiyolojisi multifaktöriyel olup; kaudal mezoderm blastogenezis yetersizliği, vasküler çalma fenomeni, amniyotik bantlar veya oligohidramniosa bağlı mekanik sıkıştırma, kadmiyum, retinoik asit gibi teratojenler, annede diyabet ve genetik mekanizmalar sayılabilir. (1) Prenatal dönemde sirenomeli tanısında kullanılabilecek bilinen bir serum belirteci henüz yoktur ve teşhisinde prenatal ultrasonografi kullanılır. (6) Yaptığımız literatür taramasında sirenomeli'nin anestezi yönetimi ile ilgili yazılar sınırlı idi.…”

“…Fakat doğum sonrasında bu olgularda renal displazinin şiddetine bağlı olarak metabolik bozukluklar (hiperkalemi, hiperglisemi, asidoz), hematolojik bozukluklar (anemi, trombosit fonksiyon bozukluğu) sık görülür. (1) Böbrek işlevleri bozuksa anestezi indüksi-yonunda ve idamesinde nefrotoksik olmayan ajanların kullanılması önerilmektedir. İnhalasyon ajanı kullanılacak ise desfluran ve izofluran tercih edilmelidir.…”

“…Nonspesifik doku esterazlarının doğumda iyi geliştiği bildirilmekte olup, bu nedenle opioid olarak remifentanil kullanılabilir. (1) Sirenomeli olgularında nöroaksiyal blokaj yöntemlerinin vertebra anomalileri nedeniyle olanaksız olduğu bu nedenle kaçınılması gerektiği bildirilmektedir. (5,9) Bösenberg (1) ise laparatomi planlanan sirenomeli olgusunda halotan ile anestezi indüksiyonundan sonra sakral hiatus kolaylıkla palpe edildiğinden % 0,25'lik bupivakain ile kaudal blokaj yaptığını bildirmiştir.…”

“…(1) Alt ekstremitelerin kısmen veya tamamen gelişememesi veya füzyonuna renal agenezi, sakrum, rektum ve mesanenin yokluğu eşlik edebilmektedir. Kalp, böb-rek, karın duvarı, göğüs ve alt omurga kusurları, tek umblikal arter, imperfore anüs ve genital organların yokluğu diğer eşlik eden bulgulardır.…”

“…Monozigotik ikizlerin birinde görülme sıklığı tekiz bebeklere göre 150 kat daha fazladır. (1,3) Olguların % 16'sında annede diyabet mevcuttur. (4) Bugüne kadar literatürde 300 olgu bildirilmiştir.…”