Syndactyly

Byline: D. Mendhekar, A. Dogra, V. Girotra

Maternal consumption of alcohol during pregnancy, especially in large amounts, may lead to significant abnormalities in the newborn infants known as fetal alcohol syndrome (FAS). It can also lead to less severe abnormalities, termed as fetal alcohol effects or exposure (FAE), which is more frequent than FAS. Surviving infants of an alcohol-drinking mother can evidence any combination of the components of a syndrome that in its full form can include mental retardation, microcephaly, a diminished physical growth, an atrial septal defect, syndactyly and facial abnormalities such as a flat bridge on the nose, an absent philtrum, and an epicanthal fold.

Drug and alcohol abuse in women and exposure of fetus to alcohol is a neglected topic in the Indian literature. So far not a single case report is available in the Indian literature on FAE or FAS.

This case highlights the effect of the exposure of the human fetus to alcohol.

Case report

A 26-year-old housewife from an urban slum area presented to us for detoxification. Her personal history revealed that she was married for the first time in 1995. However, she got divorced within 4 months due to a confrontation with the husband over her addiction to pethidine, which she had started injecting almost daily within one week of marriage due to frequent abdominal pain. At the end of 3 years, due to her poor economic condition, she switched over to alcohol. Initially for four months, she took whisky (about 230 ml/day) with occasional parenteral pethidine. Later, she switched to a local brand of alcohol called "desi pawwa" (illicit alcohol) about 150-200 ml/day. She got married for the second time in the year 2000, but she continued to take illicit alcohol (about 200 ml/day). Throughout the 9 months of pregnancy, she continued to consume alcohol without any period of abstinence despite strong resistance from her husband and knowing the risks to the fetus. On two occasions during the second trimester, she consumed as much as 400 ml of alcohol per day for 2 days without taking any food. Barring these two incidents, she had a positive attitude towards pregnancy and her nutritional care was good. She did not attend any antenatal clinic. She was admitted to the hospital only at the time of labor pains, where for the first time routine investigations were conducted and all reports were found to be within normal limits, including blood sugar and liver function tests. She delivered a full-term female baby, weighing 2.4 kg, without any peri-natal or postnatal complications. Even during her lactation period, she continued to consume the same quantity of alcohol. She attended a psychiatry clinic, along with her 2-year-old daughter, for detoxification. The child's father used to take alcohol on social occasions and upon evaluation, no psychopathology was observed in him. He would remain away from the house for most part of the day because of his work but upon coming back he would take due care of his family. Though he had cordial relations with his wife he would at times end up in a verbal argument with her because of her drinking habit. Apart from alcohol dependence, no other psychiatric problem was seen in the mother. She had been carrying out all the household chores including taking care of the child, well. There was no history of poor mother-child relationship or faulty parenting. Medical history of the child revealed delayed developmental milestones, particularly in the language and motor areas. The examination of the child showed decreased attention span and she was unable to speak more than 5 words. Further, motor incoordination was also noticed with hypotonia in all four limbs. She could walk only with support. Her play activity was solitary and she would not mix with her age-appropriate peers. She also exhibited lack of anxiety towards strangers and appeared a passive child.

Her height, weight and head circumference were 86 cm, 11 kg and 48 cm respectively. All these values were within the normal range for the child's age. Apart from a broad nose, no other congenital abnormality in the form of microcephaly, maxillary hypoplasia, joint abnormalities, epicanthal folds, short palpebral fissures, cleft palate, syndactyly etc. were noticed at the time of evaluation. Laboratory investigation of the child revealed normal hematological findings except for low hemoglobin (9 gm %). Her MRI head and echo cardiography findings were normal. Her thyroid function test too, was normal.

We do not have facilities for performing standardized neuropsychological battery for children of this age. On administering Vineland Social Maturity Scale, her age was calculated to be about 1 year. In view of the low hemoglobin level the child was prescribed Iron and B complex supplement but she did not turn up for subsequent follow-up visit.

Discussion

Alcohol and acetaldehyde can have deleterious effects on the developing fetus. These substances cross the placenta with ease; and in high enough doses, can produce fetal death and spontaneous abortion.

Western literature shows that approximately 30-35% of pregnant women drink alcohol during the course of pregnancy on a regular basis.[1] In India, alcohol content in illicit alcohol, estimated in 23 samples, was found to be in the range of 23-36 gm per 100 ml.[2] In this case report, probably the fetus was exposed to a moderate daily dose of alcohol. The child had developmental delay, which could not be attributed only to social, environmental, or nutritional factors and therefore the effect of toxin on the developing brain cannot be ruled out. Normal finding on the MRI of head does not rule out abnormalities at the micro level. The cause of the deficit is at a micro level for cases with relatively mild intellectual deficit, rather than at a macro level.[3] Burd et al [4] appropriately used the term fetal alcohol spectrum disorder precisely because of the presence of complex cognitive, behavioral and physical symptomatology in children with prenatal alcohol exposure.

The most important step in reducing the incidence of such disorders is to increase awareness among the public that FAS is a totally preventable syndrome. Women who desire to become pregnant should stop even social drinking because certain subtle neurodevelopment adverse effects to the fetus may be induced even before pregnancy is detected.

References

1. Rosenthal E. When a pregnant woman drinks. New York: Times Magazine; 1990.

2. Narawane NM, Bhatia S, Abraham P, Sanghani S, Sawant SS. Consumption of 'country liquor' and its relation to alcoholic liver disease in Mumbai. J Assoc Physicians India 1998;46:510-3.

3. Clark CM, Li D, Conry J, Conry R, Loock C. Structural and functional brain integrity in fetal alcohol syndrome in non-retarded cases. Pediatrics 2000;105: 1096-9.

4. Burd L, King MG, Martsolf JT, Kerbeshian J. Fetal alcohol syndrome: Neuropsychiatric phenomics. Neurotoxicol Teratol 2003;25:697-705.

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