Regístrese
Buscar en
Neurología (English Edition)
Toda la web
Inicio Neurología (English Edition) First case of Moebius–Poland syndrome in child prenatally exposed to misoprost...
Journal Information
Vol. 26. Issue 8.
Pages 502-503 (October 2011)
Vol. 26. Issue 8.
Pages 502-503 (October 2011)
Letter to the Editor
DOI: 10.1016/j.nrleng.2011.01.008
Full text access
First case of Moebius–Poland syndrome in child prenatally exposed to misoprostol
Primer caso de síndrome de Moebius-Poland en niño expuesto prenatalmente a misoprostol
Visits
7742
H. Pachajoaa,??
Corresponding author
harrympl@yahoo.com

Corresponding author.
, C. Isazab
a Departamento de Ciencias Básicas Médicas, Programa de Medicina, Universidad Icesi, Cali, Colombia
b Facultad de Salud, Universidad del Valle, Cali, Colombia
This item has received
7742
Visits
Article information
Full Text
Bibliography
Download PDF
Statistics
Figures (2)
Full Text
Sir,

Moebius syndrome (OMIM 157900) is characterised by facial paralysis with impairment of ocular abduction. The facial nerve (cranial nerve VII) and the abducens nerve (cranial nerve VI) are the ones most commonly involved, but other cranial nerves can also be affected. The phenotype is variable and may include congenital, orofacial and limb defects.1

The Poland syndrome (OMIM 173800) is characterised by the presence of unilateral brachysyndactyly and ipsilateral aplasia of the sternal portion of the pectoralis major muscle. It is sometimes called Poland sequence, as it was initially described in Poland.2

The combination of Moebius and Poland syndrome is rarely seen. It has been estimated to occur in 1 in 500,000 people; in the literature we reviewed, we did not find this association with cases of prenatal exposition to misoprostol.3

We present a report on Moebius and Poland syndrome in a newborn prenatally exposed to misoprostol.

The patient, who was the son of an 18-year-old mother, was brought to consult for facial dysmorphism. However, on examination he was found to have bilateral facial paralysis, a rounded face, narrow palpebral fissures, cupid's bow lips, arched palate, micrognathia and hypoplasia with absence of pectoralis major nipple (Atelier), as well as proximal syndactyly of second and third fingers and bilateral clubfoot (Figs. 1 and 2). As an important background fact, we found that his mother had used 400g of misoprostol orally and vaginally at 5 weeks of gestation to bring about an abortion, but had had only slight bleeding.

Figure 1.

Note the typical phenotype of Moebius–Poland syndrome, hypoplasia of the chest with clubfoot Atelier.

(0.1MB).
Figure 2.

Note the bilateral facial paralysis, a typical characteristic of Moebius syndrome.

(0.06MB).

Misoprostol is a synthetic analogue of prostaglandin E1 approved by drug regulatory agencies in many countries for the prevention and treatment of gastric ulcers associated to the use of non-steroidal anti-inflammatories, due to its gastric acid antisecretory effect. Prenatal exposure to misoprostol has been associated to vascular disruption defects, mainly Moebius sequence and terminal transverse type limb defects.4,5

The abnormalities in the vascular structure could be secondary to teratogenic effects. Teratogens can act directly by reducing blood flow or blood vessel development, changing anatomy and/or structure.6,7 The vascular abnormalities of the right subclavian artery observed in Poland syndrome could be related to a vascular disruption caused by misoprostol during a critical period.5,8

The presentation of a case of Moebius and Poland syndromes associated to prenatal exposure to misoprostol has not been reported. However, this combination of two congenital defects has been reported as being associated to another vascular disruptor (such as cocaine).3

The presentation of these two defects in a same patient provides evidence that both pathologies present similar mechanisms, which are probably those of a vascular disruption.

Defects due to a vascular disruption are structural disturbances in development produced by vascular problems, such as severe intermittent vasoconstriction, abnormal vessel regression during the remodelling of the vascular system, arterial thrombosis or any other phenomenon that produces a lack of O2.9

The aetiology of Moebius and Poland syndromes is unknown; a possible vascular origin has been suggested for the two pathologies. Hypotheses suggest a transient ischemia, particularly in the vertebral arteries.10,11 Premature obstruction or regression of the terminal arteries of the trigeminal (V) nerve and/or delayed formation of the vertebral basilar system could lead to anomalies in cranial nerve development.12 In children with Moebius syndrome, cerebral necrosis has been reported together with capillary telangiectasia in the midbrain and bridge.13 Disruption of the subclavian artery occurs around week 6 of gestation and is related to Moebius–Poland syndrome; this same phenomenon is related to transversal limb defects and arthrogryposis.14 The disruption phenomenon can be secondary to a blood flow interruption secondary to an arterial spasm during the sensitive embryonic phase.15,16

References
[1]
H.T. Verzijl, G.W. Padberg, M.J. Zwarts.
The spectrum of Moebius syndrome: an electrophysiological study.
Brain, 128 (2005), pp. 1728-1736
[2]
A.S. Shalev, J.G. Hall.
Poland anomaly—report of an unusual family.
Am J Med Genet, 118A (2003), pp. 180-183
[3]
S. Puvabanditsin, E. Garrow, G. Augustin, R. Titapiwatanakul, K.M. Kuniyoshi.
Poland-Möbius syndrome and cocaine abuse: a relook at vascular etiology.
Pediatr Neurol, 32 (2005), pp. 285-287
[4]
T. Da Silva Dal Pizzol, F.P. Knop, S.S. Mengue.
Prenatal exposure to misoprostol and congenital anomalies: systematic review and meta-analysis.
Reprod Toxicol, 22 (2006), pp. 666-671
[5]
C. Isaza, W. Saldarriaga, H. Pachajoa.
Uso inadecuado de misoprostol.¿Un problema de salud pública?.
Colomb Med, 39 (2008), pp. 61-65
[6]
M.I. Van Allen.
Fetal vascular disruptions: mechanisms and some resulting birth defects.
Pediatr Ann, 10 (1981), pp. 219-233
[7]
J.N. Bouwes Bavinck, D.D. Weaver.
Subclavian artery supply disruption sequence: hypothesis of a vascular etiology for Poland, Klippel-Feil, and Moebius anomalies.
Am J Med Genet, 23 (1986), pp. 903-918
[8]
F.J. Los, H. Brandemburg, M.F. Niermeijer.
Vascular disruptive syndromes after exposure to misoprostol or chorionic villus sampling.
[9]
W.S. Webster, D. Abela.
The effect of hypoxia in development.
Birth Defects Res (Part C), 81 (2007), pp. 215-228
[10]
M.J. Marques-Dias, C.H. Gonzalez, S. Rosemberg.
Möbius sequence in children exposed in utero to misoprostol: neuropathological study of three cases.
Birth Defects Res (Part A), 67 (2003), pp. 1002-1007
[11]
M. Issaivanan, V.S. Virdi, V.R. Parmar.
Subclavian artery supply disruption sequence-Klippel-Feil, Möbius anomalies.
Indian J Pediatr, 69 (2002), pp. 441-442
[12]
J.N. Bavinck, D.D. Weaver.
Subclavian artery supply disruption sequence: hypothesis of a vascular etiology for Poland, Klippel-Feil, and Möbius anomalies.
Am J Med Genet, 23 (1986), pp. 903-918
[13]
O.F. D’Cruz, C.N. Swisher, S. Jaradeh, T. Tang, R.J. Konkol.
Möbius syndrome: evidence for a vascular etiology.
J Child Neurol, 8 (1993), pp. 260-265
[14]
S. St Charles, F.J. Dimario, M.L. Grunnet.
Möbius sequence: further in vivo support for the subclavian artery supply disruption sequence.
Am J Med Genet, 47 (1993), pp. 289-293
[15]
J.-P. Bouvet, D. Leveque, F. Bernetieres, J.J. Gros.
Vascular origin of Poland syndrome? A Comparative Rheographic Study of the Vascularisation of the Arms in Eight Patients.
Eur J Pediatr, 128 (1978), pp. 17-26
[16]
G.M. Beer, P. Kompatscher, K. Hergan.
Poland's syndrome and vascular malformations.
Br J Plast Surg, 49 (1996), pp. 482-484

Please cite this article as: Pachajoa H, Isaza C. Primer caso de síndrome de Moebius-Poland en niño expuesto prenatalmente a misoprostol. Neurología. 2011;26:502-3.

Copyright © 2010. Sociedad Española de Neurología
Article options
Tools
es en pt

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?

Você é um profissional de saúde habilitado a prescrever ou dispensar medicamentos

es en pt
Política de cookies Cookies policy Política de cookies
Utilizamos cookies propias y de terceros para mejorar nuestros servicios y mostrarle publicidad relacionada con sus preferencias mediante el análisis de sus hábitos de navegación. Si continua navegando, consideramos que acepta su uso. Puede cambiar la configuración u obtener más información aquí. To improve our services and products, we use "cookies" (own or third parties authorized) to show advertising related to client preferences through the analyses of navigation customer behavior. Continuing navigation will be considered as acceptance of this use. You can change the settings or obtain more information by clicking here. Utilizamos cookies próprios e de terceiros para melhorar nossos serviços e mostrar publicidade relacionada às suas preferências, analisando seus hábitos de navegação. Se continuar a navegar, consideramos que aceita o seu uso. Você pode alterar a configuração ou obter mais informações aqui.