Supercharged End-to-Side Anterior Interosseous to Ulnar Motor Nerve Transfer for Hirayama Disease: A Case Report.

Hirayama disease is a rare condition of cervical myelopathy. Its early identification and correction can optimize functional outcomes. However, late presentation and some more severe cases may be associated with loss of hand function. Among the cases described, there are no reports of nerve transfers for this condition. We presented the first case report of a Hirayama disease of isolated ulnar nerve impairment managed with nerve transfer. Electroneuromyography showed isolated preganglionic involvement of C7, C8, and T1, with no sensory changes. The patient underwent nerve transfer with anterior interosseous nerve to ulnar nerve supercharge end-to-side, recovering hand function in 7 months.

VEPTR: Are We Reducing Respiratory Assistance Requirements?

BACKGROUND: The assisted ventilation rating (AVR) indicates the degree of external respiratory support required in children with thoracic insufficiency syndrome (TIS) and early onset scoliosis. For skeletally immature patients with TIS, the vertical expandable prosthetic titanium rib (VEPTR) device can be used to improve lung volume and growth. We hypothesized that patients who underwent early thoracic reconstruction by VEPTR treatment had an improved respiratory status. METHODS: Preoperative and postoperative AVR ratings were prospectively collected in a multicenter study group and compared to determine change after VEPTR treatment. Patients under 10 years of age at initial implant with minimum of 2-year follow-up data were included. Patients were excluded if there were incomplete data or if initial AVR was normal (breathing on room air). Statistical analysis was performed on groups which had stable, declined, and improved AVR at final follow-up. RESULTS: Database search yielded 77 patients with initial abnormal AVR. Average follow-up was 5.6 years. The most frequent primary diagnoses were congenital scoliosis (n=14) and spinal muscular atrophy (n=14). In total, 19 (24%) demonstrated improvement, 9 (12%) patients deteriorated, and 49 (64%) remained at the same level. The average preoperative major curve in those with improvement (58.4 degrees) and those with no change (63.5 degrees) was less than in those with deterioration (85.5 degrees) (P=0.014). The average age in years at implant of those with improvement (4) was less than those declined (6.7) and those with no change (5.5). In total, 16 (84.2%) of those that improved had a normal AVR and did not require respiratory support at last follow-up. CONCLUSIONS: There is evidence that a subset of patients with early onset scoliosis and TIS who received early thoracic reconstruction with VEPTR treatment show complete resolution of pulmonary support at final follow-up. In total, 89% of 79 patients did not experience respiratory deterioration. A total of 24% (n=19) had a positive change with over 84% (n=16) of this group no longer requiring support. LEVEL OF EVIDENCE: Level III-prognostic.

Síndrome de Werdnig-Hoffmann / Werdnig-Hoffmann disease

Introducción: El síndrome de Werdnig-Hoffmann o atrofia espinal tipo I forma parte de las atrofias musculares espinales y es la más grave de las tres formas clínicas existentes. Tiene carácter hereditario autosómico recesivo, no tiene tratamiento, es de carácter progresivo y por lo general culmina con la muerte del paciente entre el primero y segundo año de vida. Objetivo: Describir la conducta de la vía respiratoria anatómicamente difícil conocida en un paciente con síndrome de Werdnig-Hoffmann operado de litiasis renal derecha. Caso clínico: Paciente masculino de 39 años de edad, nivel de escolaridad superior, con diagnóstico de litiasis obstructiva en riñón derecho, propuesto para realizar una nefrolitotomía percutánea. Los exámenes en la consulta de anestesia diagnosticaron una vía respiratoria anatómicamente difícil. Pese a contar con la colaboración del paciente, personal entrenado, equipamiento necesario y proceder según los algoritmos recomendados en la literatura, se necesitó una vía quirúrgica para realizar la operación. Se efectúo el proceder quirúrgico propuesto sin complicación y el paciente salió del quirófano despierto y consiente. Conclusión: De requerirse otra intervención quirúrgica, sería necesario iniciar la intubación mediante fibroscopía óptica para evitar el edema de las vías respiratorias. De no obtenerse una vía respiratoria segura por este método, el paciente precisaría una vía aérea quirúrgica(AU)

Introduction: Werdnig-Hoffmann disease or spinal atrophy type I is part of the spinal muscular atrophies and the most serious of the three clinical forms in existence. It is an autosomal recessive hereditary condition, with no treatment, progressive in nature and usually culminates with the death of the patient between the first and second year of life. Objective: To describe the behavior of the anatomically difficult airway identified in a patient with Werdnig-Hoffmann disease operated for right renal lithiasis. Clinical case: Male patient at age 39, higher education level, with a diagnosis of obstructive lithiasis in the right kidney, proposed to be performed a percutaneous nephrolithotomy. The exams in the anesthesia consultation provided diagnosis of an anatomically difficult airway. Despite having the cooperation of the patient, trained personnel, necessary equipment and proceeding according to the algorithms recommended in the literature, a surgical approach was needed to perform the operation. The proposed surgical procedure was carried out without complications and the patient left the operating room awake and conscious. Conclusion: In case that another surgical intervention is required, it would be necessary to initiate intubation by optical fibroscopy in order to avoid edema of the respiratory tract. In case a safe airway is not obtained by this method, the patient would need a surgical airway(AU)