Mermaid syndrome: virtually no hope for survival

Lutz, Nicolas; Meyrat, Blaise; Guignard, Jean‐Pierre; Hohlfeld, Judith

doi:10.1007/s00383-003-0992-7

Cited by 15 publications

(14 citation statements)

References 6 publications

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“…9 Once diagnosed, termination of pregnancy is advocated in view of the poor prognosis particularly when associated renal agenesis is present. 10 If the diagnosis is not made antenatally it becomes obvious at birth. The extent of the renal dysgenesis, which ultimately determines the prognosis, is less obvious.…”

Section: Clinical Presentationmentioning

confidence: 99%

“…Classically the clinical features of sirenomelia include fused lower limbs, imperforate anus and complete absence of urogenital system (bilateral renal agenesis, absent ureters, urinary bladder, absent internal and external genitalia). 1,3,[8][9][10][11][12] Invariably a single umbilical artery is present 1,9,11,15 , but other life threatening anomalies may be associated.…”

Section: Clinical Presentationmentioning

confidence: 99%

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Anaesthesia and Sirenomelia (Mermaid Syndrome)

Bosenberg

2005

Southern African Journal of Anaesthesia and Analgesia

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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.

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Section: Clinical Presentationmentioning

confidence: 99%

Section: Clinical Presentationmentioning

confidence: 99%

Anaesthesia and Sirenomelia (Mermaid Syndrome)

Bosenberg

2005

Southern African Journal of Anaesthesia and Analgesia

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“…(Lutz et al, 2004). 1,8 In 1961 Duhamel 4 described the spectrum of CRS from lower limb fusion to imperforate anus.…”

Section: Disscusionmentioning

confidence: 99%

“…It is a rare malformation sequence characterized by fusion of the lower limbs 1 and as well as involving multiple organ abnormalities. This rare congenital malformation is incompatible with life.…”

Section: Introductionmentioning

confidence: 99%

Caudal regression syndrome (sirenomelia) and its pathogenesis correlation: a case report

et al. 2015

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“…Bu sendromda bebege denizkizi görünümünü veren yapi §ik bacakiann yam sira, agir üreme-bo §altim, mide-bagirsak, kalpdamar ve merkezi sinir sistemi bozukiuklari bulunur (1,2). Bu bebekier çogunlukla ölü dogarlar veya dogumdan kisa sure sonra kaybedilirler (3).…”

Section: Giriçunclassified

Mermaid syndrome

Çelik¹,

Turhan²,

Gülaşı³

et al. 2013

Turk Pediatri Ars

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ÖzetSirenomeli olarak da adlandinlan denizkizi (mermaid) sendromu oidukça nadir görülen dogumsal bir bozukiuktur. Bu sendromda bebege denizkizi görünümünü veren yapifik bacakiann yam sira, agir üreme-bo §altim, mide-bagirsak, kalp-damar ve merkezi sinir sistemi bozukiuklari bulunur. Bu yazida yapi §ik bacakiann yam sira göbek kordonunda tek arter, böbrek yokiugu, akciger hipoplazisi, özofagus atrezisi, ventriküier septai defekt, anai atrezi, bagirsak atrezisi saptanan ve dogumunun beçinci saatinde kaybedilen denizkizi sendromu olgusu sunuimuçtur. (Türi< Ped Ar § 2013; 48:65-7) Anahtar sözcükler: Denizkizi, kaudal disgenezi, sirenomeli Summary Sirenomelia, also known as the mermaid syndrome, is a very rare congenitai anomaly characterized by iower iimb fusion and severe urogenital, gastrointestinal, cardiovascuier, central nervous system maiformations. We report a case of sirenomelia who had a singie umblicai artery, renal agenesis, pulmoner hypoplasia, esophageai atresia, ventricular septai defect, anai atresia, intestinal atresia and who was iost at fifth hour of life. (Turi< Arcti Ped 2013; 48; 65-7)

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Mermaid syndrome: virtually no hope for survival

Cited by 15 publications

References 6 publications

Anaesthesia and Sirenomelia (Mermaid Syndrome)

Anaesthesia and Sirenomelia (Mermaid Syndrome)

Caudal regression syndrome (sirenomelia) and its pathogenesis correlation: a case report

Mermaid syndrome

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