Our Experience With The Thalidomide Children: An Interim Report

Maurice Mongeau, M.D.


Following the thalidomide tragedy, three Canadian centres were selected for the evaluation, habilitation, and follow-up of children born with malformations secondary to thalidomide: the Manitoba Rehabilitation Centre in Winnipeg, the Crippled Children's Centre in Toronto, and the Rehabilitation Institute of Montreal. The habilitation programme at the Rehabilitation Institute of Montreal was inaugurated in 1963 and has provided treatment for 34 thalidomide children.

Thirty-five cases with congenital anomalies associated with thalidomide were referred to the Institute by the Ministry of Health, Province of Quebec. One child died before examination; two were evaluated but were withdrawn from the recommended programme by their parents. The elimination of these three children left a net case load of 32 for assessment. Two cases were subsequently referred from other provinces, raising the total to 34 cases. In each case, the ingestion of thalidomide by the mother during pregnancy was confirmed through an inquest conducted by a governmental agency.

Evaluation

The standard evaluation of children with congenital malformations treated at the Institute consists of:

  1. A complete case history of the child and the mother.

  2. Complete physical examinations by the physiatrist, the pediatrician, and the orthopedist.

  3. Radiological studies of the skeleton.

  4. Laboratory studies (e.g., hematology) .

  5. An electroencephalogram, an electrocardiogram, and an intravenous pyelogram.

  6. A social evaluation.

  7. A psychological evaluation of the child and of both parents.

  8. A psychiatric interview of both parents.

  9. An occupational therapy evaluation.

  10. Added consultations if required .

  11. An orthotic and/or a prosthetic evaluation.

  12. Photographs and slides.

  13. A team discussion following the completed evaluation to develop a detailed habilitation programme.

All but one of the children treated at the Centre were born in 1962. Their present ages range from 3 to 5 years of age. Eight of the children (23.5%) are male and 26 (76.5%) are female.

Classification and Diagnosis

Since all the malformations secondary to thalidomide have been quite similar regardless of the country in which they occurred, a thalidomide syndrome has been described. This syndrome consists of one or more of the following congenital malformations: phocomelia, paraxial hemimelia, congenital hip dislocation, polydactylia and syndactylia, malformation of the external ear with or without congenital deafness, unilateral facial palsy, hemangioma, and malformations of the internal organs, mainly of the heart and the urinary system.

Examination of the 34 children disclosed 109 malformations, an average of 3.21 per child (see table). Twenty-six of the children displayed defects in the upper and/or lower extremities (76.5%). Twenty showed malformations in the upper extremities only, all of which were bilaterally symmetrical except two cases involving the hands. Three displayed symmetrical and bilateral deformities in the lower extremities only. Three children had involvement of all four extremities, one with quadruple pho-comelias, one with quadruple transverse hemimelias, and the third with symmetrical phocomelias of the upper extremities and asymmetrical deformities of the lower extremities. In general the classification system of Frantz and O'Rahilly was adopted, although we have used the terms "dys-cheiria" and "dyspodia" to indicate malformations of the hands and feet.

Some interesting observations recorded were:

  1. Limb malformations were bilateral and symmetrical.

  2. Malformations involving the ears were asymmetrical and essentially unilateral.

  3. Facial palsies were unilateral and appeared on the same side as the malformed ears.

  4. All the phocomelic children and the three cases of paraxial hemimelia radial had no thumb. For grasping, the interossei are utilized instead of the flexors of the fingers; objects are grasped between the last two ulnar fingers in 31 of the 34 malformed hands.

  5. All the phocomelic children had only partial hands, and those hands may be divided by the number of fingers:

    • 2 hands with 1 finger 6 hands with 2 fingers

    • 15 hands with 3 fingers

    • 2 hands with 4 fingers

    • 3 hands with 5 fingers (polydactylia, as there is absence of the thumb. )

Habilitation Programme

The habilitation programme should be a team effort which recognizes the essential role played by the parents, whose evaluation and possible treatment is a necessary first step. The help of a social worker and a psychologist in such a habilitation programme is mandatory. Observation showed that initially the parents were in a state of shock and grief, unable to accept the fact of a deformed child. In some cases refusal to accept the handicap extended to a rejection of the child, while in others the reaction was one of over-protection.

Occupational therapists play a very active part in the evaluation and the treatment of these children, while the cooperation of the nursing staff is also essential.

Following is an outline of our habilitative procedures:

Classification 1

Malformation of the upper extremities

  1. Of the 14 children with upper-extremity phocomelia, one of whom had quadruple limb deficiencies, 12 were initially fitted and trained with conventional prostheses, with activated hooks as terminal devices (Fig. 1 and 2 ). Of the two remaining, one was not treated because of family refusal to cooperate; the other has been treated elsewhere.

  2. Of the three cases with upper extremity paraxial hemimelia, one has been treated with good results by passive stretching and splints to stabilize the wrists. Two were operated upon in an attempt to correct wrist deformities, with poor results.

  3. The only child with transverse hemimelia was fitted with standard prostheses with hooks as terminal devices.

  4. Of the five cases of dyscheiria, three have been treated conservatively to date; two have been operated upon.

Malformation of the lower extremities

  1. One of the four children with lower-extremity phocomelia also had phocomelia of the upper limbs. She was fitted initially with a special bucket on a pair of short skis to develop balance, and was then fitted with a swivel walker.

  2. In the other three cases the phocomelic feet were amputated and the children are now walking with prostheses.

  3. The case with transverse hemimelia of the four extremities was fitted with four conventional prosthetic appliances, with excellent results.

Malformed ears

One case was operated during the first month of life; the remaining nine were evaluated by a plastic surgeon, but no surgery has been performed to date.

Deafness

The five cases of congenital deafness were evaluated and are being treated in a Montreal audiology clinic.

Facial palsy

The 11 cases have been followed and reviewed. Continuous improvement has been noted without any surgical intervention.

Cardiac malformation

The three cases of cardiac malformation have been closely super>vised; no surgical treatment has yet been recommended.

Urogenital malformation

One case with rectovaginal communication was operated on successfully.

Role of the Prosthetist

The prosthetist should be a member of the rehabilitation team. All of the prostheses for these children were constructed in our Prosthetic Department. Children with upper-extremity phocomelia were equipped with polyethylene plastic shoulder caps. As a rule, the shoulder consisted of a passive conventional joint limited to flexion and extension. The elbow incorporated a friction "turntable" mechanism which provided internal and external rotation. Only one lateral side bar was used in order to provide maximal space for the phocomelic hand. The majority of the wrist units used were "friction type," and the terminal device was a hook.

All children with limb malformations were fitted initially with conventional prostheses. However, at present several children with bilateral upper-extremity phocomelia are using prostheses activated by carbon dioxide in order to improve their function (Fig. 3 and 4 ). We are also planning to use more sophisticated prostheses utilizing electricity or electronics.

Conclusions and Recommendations

  1. Congenitally deformed children should be evaluated as soon as possible after birth, and the habilitation programme must be initiated at an early age.

  2. The parents need support and guidance to achieve acceptance of a deformed child and to cooperate in a habilitation programme.

  3. The early prescription of a prosthesis is desirable.

  4. A good follow-up is essential, as the habilitation programme should be continued until adult life.

  5. Severe congenital amputees should be referred and evaluated in clinics where facilities exist for a complete evaluation, for an active habilitation programme, and for follow-up.

  6. Team work is essential for the evaluation, during the active habilitation programme, and for follow-up.

Maurice Mongeau, M.D. is the Clinic Chief of the Children's Amputee Clinic Rehabilitation Institute of Montreal Montreal, Quebec