A 4-year-old male with Nager's syndrome phenotypically manifested by: cleft palate, bilateral microtia, severe soft palate and inferior fibroid cleft, absence of bilateral hypoplasia malar, mandibular hypoplasia, colob
The lower eyelid coloboma and cleft palate had been previously treated and the presence of significant structural organic anomalies had been ruled out.
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She also had preaxial, upper and lower extremities hypoplasia of the thumbs in both hands.
The radial deficiency that accompanies this type of syndromes is usually bilateral and asymmetric, and thus occurred in our patient who had type V thumb hypoplasia in the right hand and type IV Koth popular classification used in left hand agreement (5).
Preaxial involvement of the lower limbs in Nager's syndrome distinguishes it from others such as Treacher-Collins syndrome, without limb involvement, or postaxial Miller's syndrome, in which the involvement is
In our case, the lower limbs were also compromised, presenting partial agenesis of the bilateral tibia.
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The most indicated treatment in this case was thumbization in both hands.
Currently, the procedure is performed at an early age, between 6 and 18 months of age, with the objective of achieving postoperative rehabilitation before the development of the opposing clamp.
In our patient we perform the thumb in a relatively late way, at 4 years of age, because that was the age with which he was referred to our Hand Clinic from his reference hospital (6).
We performed a right hand thumb procedure under general anesthesia without complications using the Buck-Gramcko technique originally described in 1964 (7) and taking into account the modifications of the technique published in 1971 (8).
It basically consists of:
1) Keep the head of the second metatarsal.
2) To resect the epiphysis of the second metatarsal head.
3) Mutation of specific muscle functions.
4) Rotate the index at an angle of 160o in the longitudinal axis.
5. Establishment of an index equal to or greater than 40o of palm formation;
6. Turn the head of the second metatarsal at 70-80o.
