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Journal of Indian Association of Pediatric Surgeons
Medknow Publications on behalf of the Indian Association of Pediatric Surgeons
ISSN: 0971-9261 EISSN: 1998-3891
Vol. 12, Num. 2, 2007, pp. 102-103

Journal of Indian Association of Pediatric Surgeons, Vol. 12, No. 2, April-June, 2007, pp. 102-103

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Placento-cranial adhesion: A report of two cases

Ghritlaharey RajendraK, Kushwaha AnandSingh, Chanchlani Roshan, Nanda Monika

Department of Paediatric Surgery, Gandhi Medical College and Associated, Kamla Nehru and Hamidia Hospitals, Bhopal - 462 001, MP
Correspondence Address:Department of Paediatric Surgery, Gandhi Medical College and Associated, Kamla Nehru and Hamidia Hospitals, Bhopal - 462 001, MP drrajendrak1@rediffmail.com

Code Number: ip07037

Keywords: Body stalk anomaly, limb body wall complex, placento-cranial adhesion, short umbilical cord

Case Reports

Case 1

A preterm, eight hour-old male with cranio-facial deformities was born normally at a hospital to a G 1 P 0, 22 year- old mother on June 24, 2006. The mother had no significant antenatal history. The child weighed 1.9 kg including the placenta, which was adherent to the head. Also evident were associated exencephaly, cleft of face, lip and palates and bilateral anophthalmos. Constriction marks over the right arm, hypoplasia of the right radius and ulna and oligodactyly in both hands were also present [Figure - 1]. There were no thoracic or abdominoschisis and both lower limbs as well as other systemic examinations were normal. He survived for only 48 h after birth.

Case 2

A full-term male was born to a G 3 P 2, 25 year-old mother by lower segment Caesarean section (LSCS) performed due to obstructed labour on August 27, 2006. Antenatal history was nothing significant. He weighed 2.5 kg including placenta, which was adherent to his head. Also evident were associated exencephaly and cleft of lip and palate on the right side [Figure - 2]. He also had multiple constriction marks over both lower limbs and associated bilateral congenital talipes equino varus (CTEV). Other systemic examinations were normal. He survived for only 12 h after birth.

Discussion

Limb body wall complex (LBWC) is a rare, complicated, polymalformative fetal malformation syndrome with the essential features of: (a) exencephaly / encephalocele with facial clefts, (b) thoraco- and / or abdominoschisis and (c) limb defects. [1] There is no sex or familial predilection and two phenotypes have been described as placento-cranial and placento-abdominal adhesions. [1] The LBWC is also described as body stalk anomaly, cyllosomas and pleurosomas with a reported incidence of 1:4000 to 0.7:10,000 births. [1],[2]

In a fetopathologic evaluation with amniotic bands, Moerman et al. described three types of lesions as: (1) constructive tissue bands, (2) amniotic adhesions and (3) more complex lesions such as LBWC. Most of the cranio-facial defects (encephalocele and / or facial clefts) occurring in these fetuses are the result of a vascular disruption sequence with or without cephalo-amniotic adhesion. [3]

The diagnosis of this entity is based on the presence of two of the three features described above. Ultrasonographic detection of abdominoschisis, scoliosis, a single umbilical artery, a short umbilical cord, an extremely elevated level of maternal serum alpha-fetoprotein and abnormalities of the lower extremities are the keys to early diagnosis. [1],[4]

Major structural defects associated with LBWC include limb defects, scoliosis, craniofacial defects, cardiac defects, bowel atresia, renal agenesis / dysplasia, urogenital anomalies, absent or short umbilical cord etc. [1],[2],[4],[5] LBWC should be differentiated from more common anterior abdominal wall defects such as gastroschisis, omphalocele, etc.

As the existence of a fetus with LBWC is always endangered and universally fatal; it necessitates an early antenatal diagnosis and medical termination of the pregnancy. The risk of recurrence being negligible, the prognosis for a later pregnancy remains positive. [1],[2],[4],[5],[6],[7] Both of our patients showed the placento-cranial phenotype. Neither any further investigations nor postmortem examinations have been done in these two cases because of the fatal nature of the disease.

References

1.D'Souza J, Indrajit IK, Menon S. Limb body wall complex. MJAFI 2004;60:77-80.  Back to cited text no. 1    
2.Gupta S, Upreti L, Bhargava SK, Jain S, Verma S. Radiological quiz - Obstetric - Limb body wall complex or the body stalk anomaly. Indian J Radiol Imag 2002;12:429-31.  Back to cited text no. 2    
3.Moerman P, Fryns JP, Vandenberghe K, Lauweryns JM. Constructive amniotic bands, amniotic adhesions and limb body wall complex: Discrete disruption sequences with pathogenetic overlap. Am J Med Genet 1992;42:470-9.  Back to cited text no. 3  [PUBMED]  
4.Managoli S, Chaturvedi P, Vilhekar KY, Gagane N. Limb body wall complex. Indian Pediatr 2003;40:891-4.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.Catte LD, Waterschoot T, Mares C, Goossens A, et al . Umbilical cord, short umbilical cord syndrome. 1993-03-19-21 Umbilical cord, short umbilical cord syndrome.© de Catte Available from: http://www.thefetus.net.  Back to cited text no. 5    
6.Nevils BG, Maciulla JE, Izquierdo LA, et al . Umbilical cord, short umbilical cord syndrome. 1993-04-16-15 Umbilical cord, short umbilical cord syndrome © Nevils Available from: http://www.thefetus.net.  Back to cited text no. 6    
7.Cuillier F. Limb body wall complex. 2004-08-16-08 Limb body wall complex © Cuillier Available from: .  Back to cited text no. 7    

Copyright 2007 - Journal of Indian Association of Pediatric Surgeons

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