Correction of arthrogrypotic clubfoot with a modified Ponseti technique.

Surgical releases for arthrogrypotic clubfeet have high recurrence rates, require further surgery, and result in short, painful feet. We asked whether a modified Ponseti technique could achieve plantigrade, braceable feet. Ten patients (mean age, 16.2 months; range, 3-40 months), with 19 arthrogrypotic clubfeet, underwent an initial percutaneous Achilles tenotomy to unlock the calcaneus from the posterior tibia followed by weekly Ponseti-style casts. A second percutaneous Achilles tenotomy was performed in 53%. Mean number of casts was 7.7 (range, 4-12). From pretreatment to completion of initial series of casts, mean scores of Dimeglio et al. improved from 16 to 5 (ranges, 12-18 and 2-9, respectively), Catterall scores (as modified by Pirani and colleagues) from 4.8 to 0.9 (ranges, 1.5-6.0 and 0.0-2.0), and maximum passive dorsiflexion from -45 degrees (range, -75 degrees to -20 degrees ) to 10 degrees (range, 0 degrees to 40 degrees ). Ankle-foot orthoses maintained correction. At the minimum followup of 13 months (mean, 38.5 months; range, 13-70 months), the mean maximum dorsiflexion was 5 degrees (range, -20 degrees to 20 degrees ), two patients had posterior releases and no patient's ambulatory ability was compromised by foot shape. Arthrogrypotic clubfeet can be corrected without extensive surgery during infancy or early childhood. Limited surgery may be required as the children age.

Ex utero intrapartum treatment procedure in a patient with arthrogryposis multiplex congenita, using continuous spinal anesthesia and intravenous nitroglycerin for uterine relaxation.

The ex utero intrapartum treatment procedure allows for the controlled management of a potentially life-threatening difficult airway in the newborn. General anesthesia has previously been reported for the management of this procedure. We report the use of continuous spinal anesthesia in conjunction with intravenous nitroglycerin for the ex utero intrapartum treatment procedure in a woman with arthrogryposis multiplex congenita, a rare syndrome characterized by rigid joints and limb contractures.

[Lumbosacral agenesis. Presentation of one case. Literature review and embriological considerations (author's transl)]. / Agenesia lumbosacra. Presentación de un caso. Revisión de la literatura y consideraciones embriológicas.

A case of a complete lumbosacral agenesis is presented, whose etiological factors are unknown. Clinically, motor deficit corresponded to the agenesis level, but the sensibility and the autonomous nervous system functions, which were conserved, partially, in lower levels than the normal ones in the agenesis. The case report is completed with electromiography, mielography, rectal biopsy, urography, cistography, opaque enema and inborn infection study. There are three facts: a partially conserved sensibility in the lower extremities, normal bladder function and above all, the presence of nervous parasympatics normal fibers in rectum wall, in contrast with the serious motor alteration just in the same medullar levels. Explanation of these facts suggest origin of an embryological source. It is assumed that the noxa must have acted between the third and the fourth weeks of pregnancy at a caudal cordomesoblast level, supposing that it did not affect neural crest.