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AN INTERESTING CASE OF ADAM COMPLEX

Meena Jadhav, Ajay Mehta
Hon. Sonologist, N. Wadia Maternity Hospital, Mumbai.

An 18 year old primigravida was advised USG at 20 weeks which showed a possibility of membranous VSD. After caesarean delivery baby expired within few movements. Being a rare case presented here.

INTRODUCTION

Adam complex also known as ‘amniotic band syndrome’ or ‘amniotic rupture sequence’, is a non-recurrent, sporadic condition, which occurs with an incidence of 1 in 1,200 to 15,000 cases.

Its aetiopathogenesis can be explained on the basis of

1. Torpin’s theory of early amniotic rupture
2. Streeter’s hypothesis of intrinsic defect in germ plasma, or by the
3. Vascular disruption theory.

CASE REPORT

The patient was an 18 year old primigravida, with no other high risk antenatal factors.

USG done at 20 weeks gestational age showed marked dorsal kyphosis of spine, a narrow thorax, and a possibility of membranous VSD. Repeat USG at 25 weeks gestational age failed to give any additional information. As there was no definite diagnosis on USG, the patient was not advised termination of pregnancy.

The patient had regular antenatal care until 38 weeks when she presented in labour.

In labour, she presented with a brow presentation, and an arrest of descent was noted.

Due to persistion was performed. ent brow presentation an emergency caesarean sectA female baby, 1,340 gms, with multiple defects and a short umbilical cord was born. The baby expired within a few moments after birth.

A partial autopsy was done on the baby, the findings were:-

• External examination

— Severe kyphoscoliosis of spine
— Exomphalos major
— Grossly deformed lower limbs
— Multiple bands of adhesion between limbs and back of sacrum and back of neck
— Amputation of one toe

• Internal examination

Through the ruptured sac of exomphalos major revealed.

— Ileal atresia

The above case stresses the importance of an early USG diagnosis, which is important for appropriate management of such cases. However, USG diagnosis is also limited in case of multiple complex congenital defects. In such cases, a good postmortem examination is important for accurate diagnosis and genetic counseling of future pregnancies.

DISCUSSION

Amniotic band syndrome is a sporadic condition that occurs in approximately 1:1200 to 1:15,000 livebirths and that may result in amputation, constriction and other deformities of the foetus. [1]

The genesis of amniotic band syndrome is debated. Streeter (1930) and others maintain, that localised failure of germ plasm usually is responsible for the abnormalities. Torpin (1968) and others, say that the lesions are a consequence of early rupture of the amnion, which then forms adherent tough bands, that constrict and at times actually amputate an extremity of the foetus.

Evidence from the internal anomalies suggests that, in most cases reviewed, damage occurred in a definable time period, probably prior to 26 days post-conception and before the establishment of effective embryonic circulation. Most defects are explicable in terms of interference with neuropore closure, malmigration of cephalic neural crest tissue, and damage to the mesonephros, consistent with local interference of the graded expression of organizational genes, resulting in a local defect in the organization of the embryo. [2]

There is ultrasound evidence in literature supporting the hypothesis that vascular disruption from death of a co-twin, or from in utero embolic infarcts can cause terminal limb reduction defects and ring constrictions, similar to those of ‘amniotic band disruption sequence’, in the absence of an abnormal amnion. [3]

Two cases have been reported in literature, where minimally invasive surgical techniques were used to interrupt the amniotic bands, thus avoiding spontaneous amputation of the extremity. These cases represent the 1st prenatal surgical intervention successfully used to treat constricting amniotic bands in humans. [1]

Similar proportions of male and female infants are affected, although the syndrome is more common in mothers younger than 25 years of age and in first births. A heightened awareness of the syndrome should enhance the identification of amniotic band syndrome, which has implications for genetic counselling and our understanding of the aetiology and pathogenesis of this condition. [4]

REFERENCES

  1.  Ultrasound. Obstet Gynecol Nov. 1997; 10 (5) : 316-20.
  2.  Am J Med Genet Oct. 1, 1992; 44 (3) : 280-7.
  3.  Am J Med Genet Nov. 14, 1992 44 (5) : 598-604.
  4.  Paediatr-Perinat-Epidemiol Oct. 1993; 7 (4) : 345-403.

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