Long-Term Hand and Shoulder Function in Children following Early Surgical Intervention for a Birth-Related Upper Brachial Plexus Injury.

Purpose To better understand the long-term hand and shoulder outcomes of upper brachial plexus birth injuries. Methods We evaluated shoulder and hand function in 32 patients (13 males; 19 females) with a C5/C6 birth injury history). All patients had undergone primary nerve surgery as infants, and 12 underwent a simultaneous shoulder procedure as they presented with a fixed internal rotation contracture of the shoulder. On average, all patients were evaluated and examined 15 years postoperatively. The shoulder function was evaluated using the Miami Shoulder Scale. Hand function was measured by the 9-hole peg test (9-HPT) and statistical analysis included comparison of 9-HPT time against normative data using the Student's t -test. Results The cohort includes 22 right-hand-dominant and 10 left-hand-dominant patients. Mean age at surgery was 10 months; mean age at follow-up was 15 years ± 2 years 2 months. Cumulative shoulder function was "good" or "excellent" (Miami score) in 23 patients. For 9-HPT, 23 out of 32 patients seen had an involved hand with a significant alteration in function. Conclusion Early nerve surgery in cases of upper brachial plexus birth injuries result in the desired outcome. To ensure timely and targeted therapy for any residual deficits, it is imperative that limitations in hand function among children with an Erb's palsy.

Four Anchor Repair of Jersey Finger.

Background: Various surgical techniques for treating avulsions of the flexor digitorum profundus tendon at the distal phalanx have been published but no ideal technique has emerged. We introduce a new all-internal 4-anchor flexor tendon repair technique and evaluate outcomes in three clinical cases. Methods: In this retrospective case series, we reviewed three patients that sustained an avulsion of the flexor digitorum profundus tendon at the distal phalanx. All patients were surgically treated with the four-anchor repair technique. Two titanium anchors were inserted into the distal phalanx and two all-suture anchors were inserted distal to the first set of anchors. The tendon was then attached to these four anchors using a Krackow stitch pattern and the anchors were sown to each other. Active flexion and extension of the proximal and distal interphalangeal joint were measured at 3-month, 12-month, and 5-year follow-up. Postoperative complications were documented. Results: All patients achieved excellent clinical outcomes according to assessment criteria. At 3-month follow-up, all patients regained full flexion; two patients had full extension, while one patient was 3 degrees short of full extension. At 12-month follow-up, all patients had full flexion and extension. Five-year follow-up demonstrated the same results with no loss of function, sensation or grip strength. The repairs healed without rupture, and no complications were reported. Conclusion: The 4-anchor flexor tendon repair is a viable surgical technique for zone 1 flexor digitorum profundus tendon repair or reconstruction. Further studies are needed to replicate these promising results and biomechanically validate this technique.Level of Evidence: IV.

Lower extremity nerve trauma.

Peripheral nerve injuries of the lower extremity (LE) are frequently encountered in orthopaedic practice. They can be traumatic or iatrogenic. Proper and timely diagnosis and treatment are the keys to optimizing outcomes. This paper reviews and discusses the basic anatomy and physiology of nerve injury and the current literature on the incidence, pathogenesis, diagnosis, management and outcomes of sciatic, femoral, peroneal, and tibial nerve injuries. The purpose of this review is to suggest a protocol for evaluation and management of LE nerve injuries.

Hand function in children with an upper brachial plexus birth injury: results of the nine-hole peg test.

AIM: The aim of this study was to evaluate hand function in children with Erb upper brachial plexus palsy. METHOD: Hand function was evaluated in 25 children (eight males; 17 females) with a diagnosed upper (C5/C6) brachial plexus birth injury. Of these children, 22 had undergone primary nerve reconstruction and 13 of the 25 had undergone simultaneous and/or secondary shoulder procedures. Hand function was evaluated using the nine-hole peg test at a mean age of 9 years (SD 2y 2mo), and compared with the contralateral, uninvolved hand. Results were compared with age- and sex-matched population norms, and correlated with shoulder outcomes using the Gilbert and Miami scores. RESULTS: Although shoulder function was graded as good or excellent in 24 of 25 children, hand function as measured by the nine-hole peg test was significantly altered in the involved hand in 80% (p=0.008). On average the participants took 18.8% longer to complete the task with the involved hand; this was significantly different from the expected difference of 7.2% (p=0.008). INTERPRETATION: Hand function is impaired in individuals with upper brachial plexus birth injury. These results suggest that from the initiation of treatment in this population, attention should be paid to recognizing and focusing therapy on subtle limitations of hand function.

Hypoplasia of the trapezius and history of ipsilateral transient neonatal brachial plexus palsy.

We present two children with hypoplasia of the left trapezius muscle and a history of ipsilateral transient neonatal brachial plexus palsy without documented trapezius weakness. Magnetic resonance imaging in these patients with unilateral left hypoplasia of the trapezius revealed decreased muscles in the left side of the neck and left supraclavicular region on coronal views, decreased muscle mass between the left splenius capitis muscle and the subcutaneous tissue at the level of the neck on axial views, and decreased size of the left paraspinal region on sagittal views. Three possibilities can explain the association of hypoplasia of the trapezius and obstetric brachial plexus palsy: increased vulnerability of the brachial plexus to stretch injury during delivery because of intrauterine trapezius weakness, a casual association of these two conditions, or an erroneous diagnosis of brachial plexus palsy in patients with trapezial weakness. Careful documentation of neck and shoulder movements can distinguish among shoulder weakness because of trapezius hypoplasia, brachial plexus palsy, or brachial plexus palsy with trapezius hypoplasia. Hence, we recommend precise documentation of neck movements in the initial description of patients with suspected neonatal brachial plexus palsy.

Outcome following spinal accessory to suprascapular (spinoscapular) nerve transfer in infants with brachial plexus birth injuries.

The purpose of this study is to evaluate the value of distal spinal accessory nerve (SAN) transfer to the suprascapular nerve (SSN) in children with brachial plexus birth injuries in order to better define the application and outcome of this transfer in these infants. Over a 3-year period, 34 infants with brachial plexus injuries underwent transfer of the SAN to the SSN as part of the primary surgical reconstruction. Twenty-five patients (direct repair, n = 20; interposition graft, n = 5) achieved a minimum follow-up of 24 months. Fourteen children underwent plexus reconstruction with SAN-to-SSN transfer at less than 9 months of age, and 11 underwent surgical reconstruction at the age of 9 months or older. Mean age at the time of nerve transfer was 11.6 months (range, 5-30 months). At latest follow-up, active shoulder external rotation was measured in the arm abducted position and confirmed by review of videos. The Gilbert and Miami shoulder classification scores were utilized to report shoulder-specific functional outcomes. The effects of patient age at the time of nerve transfer and the use of interpositional nerve graft were analyzed. Overall mean active external rotation measured 69.6°; mean Gilbert score was 4.1 and the mean Miami score was 7.1, corresponding to overall good shoulder functional outcomes. Similar clinical and shoulder-specific functional outcomes were obtained in patients undergoing early (<9 months of age, n = 14) and late (>9 months of age, n = 11) SAN-to-SSN transfer and primary plexus reconstruction. Nine patients (27%) were lost to follow-up and are not included in the analysis. Optimum results were achieved following direct transfer (n = 20). Results following the use of an interpositional graft (n = 5) were rated satisfactory. No patient required a secondary shoulder procedure during the study period. There were no postoperative complications. Distal SAN-to-SSN (spinoscapular) nerve transfer is a reliable option for shoulder reinnervation in infants with brachial plexus birth injuries. Direct transfer seems to be the optimum method. The age of the patient does not seem to significantly impact on outcome.

Agenesis of the corrugator supercilii: a benign condition.

We report 2 neonates with frontonasal masses. The frontonasal masses were only present while the neonates were crying. The rest of the general examination and the neurological examination of the neonates were normal. The first patient had an extensive neuroimaging evaluation that included skull radiograph, computed tomography (CT) and magnetic resonance imaging (MRI) of the brain, and ultrasound of the frontonasal mass. The second patient was evaluated with ultrasound of the frontonasal mass. The mother of the second patient had no frontonasal creases and was unable to frown. In both patients, the ultrasonographic studies revealed nonspecific soft tissue thickening in the region of the glabella only while crying. The ultrasonographic findings and the similarity between the mother's findings and those of adult patients receiving botulinum toxin injection to the corrugator supercilii muscle point to the absence of this muscle as the cause of the frontonasal mass in these patients.

[Updating neonatal neurometabolic screening]. / Estado actual de la pesquisa neurometabólica neonatal.

The purpose of neurometabolic screening is the earliest possible detection of treatable disorders. The disorders currently offered to general population screening are phenylketonuria, congenital hypothyroidism, galactosemia, congenital adrenal hyperplasia, cystic fibrosis, biotinidase deficiency, maple syrup urine disease, sickle cell disease, homocystinuria. The methods used for these evaluations are mass spectroscopy and genetic testing.

[Juvenile myasthenia gravis]. / Miastenia gravis juvenil.

Juvenile myasthenia gravis is a chronic autoimmune disorder which occurs when serum antibodies combine with nicotinic acetylcholine receptors at the muscle membrane of the motor endplate imparing the neuromuscular transmission. It results in early muscle fatigability with progression to a complete paralysis during repetitive muscle contraction (movements) or steady muscle contraction (postures), and less common persistent paralysis at rest. The cranial nerves, mainly the one innervating the extraoccular and palpebral levator, are the most susceptible to permanent weakness and paralysis at rest. Initial clinical presentations are generalized, ocular and respiratory forms. The diagnosis is suspected through medical history of abnormal fatigability and corroborated by physical examination, repetitive nerve stimulation of an affected but not complete paralyzed muscle, correction of fatigability by the intravenous administration of acetylcholine esterase inhibitors, and by the presence of serum acetylcholine receptors antibodies (ACRA). The long term treatment is symptomatic (acetylcholine inhibitors) and etiopathogenic (immunosupresor drugs, plasmapheresis, intravenous gamma globulin and thymectomy. Spontaneous or post symptomatic and etiopathogenic treatment remissions occur from 1 to 10 years. Fatality is rare but children are at high risk during myasthenia crisis.

Miastenia gravis juvenil / Juvenile myasthenia gravis

La miastenia gravis juvenil (MGJ) es un trastorno crónico auto inmune en el cual existen anticuerpos séricos que al unirse a los receptores de acetilcolin nicotínicos de la membrana muscular de la placa motora alteran la transmisión neuromuscular. El resultado es fatiga muscular precoz con progresión a la parálisis durante estados de contracción muscular iterativos (movimientos) o sostenidos (posturas) y más raramente parálisis permanente durante el reposo. Los músculos inervados por los nervios craneales, especialmente los extraoculares y elevadores de los párpados, tienen más tendencia a la debilidad muscular persistente que los inervados por otros pares craneales y las extremidades. Las formas clínicas de presentación son generalizadas, oculares y respiratorias. El diagnóstico se sospecha mediante la anamnesia, la fatiga anormal se comprueba mediante el examen físico y la estimulación eléctrica iterativa del nervio que inerva al músculo afectado pero no paralizado. Se corrobora mediante la administración de inhibidores de la acetilcolin esterasa (IACE) que al aumentar la cantidad de acetilcolin en la hendidura sináptica, corrigen la fatiga o la debilidad muscular transitoriamente. Se hace el diagnóstico de certeza mediante la demostración sérica de anticuerpos contra los receptores de acetilcolin (ACRA). El tratamiento es a largo plazo sintomático con IACE y etiopatogénico con inmunosupresores, plasmaféresis, gamma globulina endovenosa y timectomía. El curso es crónico. La remisión espontánea o después de tratamiento sintomático o etiopatogénico ocurre entre 1-10 años respectivamente. La mortalidad es prácticamente nula aun durantes las crisis miastenias gracias a la educación de padres, pacientes y público en general sobre el tema, al desarrollo del sistema de respuesta rápida de auxilio domiciliario y las unidades de cuidados intensivos y el empleo de la ventilación asistida profiláctica, plasmaféresis y administración endovenosa de gamma globulina.

Juvenile myasthenia gravis is a chronic autoimmune disorder which occurs when serum antibodies combine with nicotinic acetylcholine receptors at the muscle membrane of the motor endplate imparing the neuromuscular transmission. It results in early muscle fatigability with progression to a complete paralysis during repetitive muscle contraction (movements) or steady muscle contraction (postures), and less common persistent paralysis at rest. The cranial nerves, mainly the one innervating the extraoccular and palpebral levator, are the most susceptible to permanent weakness and paralysis at rest. Initial clinical presentations are generalized, ocular and respiratory forms. The diagnosis is suspected through medical history of abnormal fatigability and corroborated by physical examination, repetitive nerve stimulation of an affected but not complete paralyzed muscle, correction of fatigability by the intravenous administration of acetylcholine esterase inhibitors, and by the presence of serum acetylcholine receptors antibodies (ACRA). The long term treatment is symptomatic (acetylcholine inhibitors) and etiopathogenic (immunosupresor drugs, plasmapheresis, intravenous gamma globulin and thymectomy. Spontaneous or post symptomatic and etiopathogenic treatment remissions occur from 1 to 10 years. Fatality is rare but children are at high risk during myasthenia crisis.

Cortical dysplasia and obstetrical brachial plexus palsy.

We report 2 patients with obstetrical brachial plexus palsy, ipsilateral leg weakness, and contralateral motor cortical dysplasia. To our knowledge, this is the first description of such an association. In both cases, the diagnosis of obstetrical brachial plexus palsy was established clinically shortly after birth and later confirmed neurophysiologically. Motor cortex dysplasia was diagnosed by magnetic resonance imaging (MRI). The association of obstetrical brachial plexus palsy and contralateral motor cortex dysplasia, a condition known to produce congenital hemiparesis, raises the possibility that the cortical dysplasia was a predisposing factor for obstetrical brachial plexus palsy in these cases.

Resolution of brachial plexus palsy due to hemangioma after intravenous corticosteroid therapy.

The authors report a 7-day-old girl born with a hemangioma involving the right side of the scalp and neck who developed ipsilateral brachial plexus palsy at 5 days of age. Imaging studies confirmed the presence of a cavernous hemangioma in the vicinity of the brachial plexus. She was treated with intravenous corticosteroids and her palsy resolved in 5 days. Only 1 prior case of hemangioma producing brachial plexus palsy has been reported; this patient was not treated with corticosteroids and had a partial recovery. The authors believe that the quick recovery after initiating systemic steroids was due to relief of nerve compression (neuropraxia) resulting from shrinkage of the cavernous hemangioma. The purpose of this article is to describe an uncommon cause of neonatal brachial plexus palsy and to report the effectiveness of early intravenous corticosteroid treatment in a patient with brachial plexus palsy due to a cavernous hemangioma.

Hemispherectomy in early infantile epileptic encephalopathy.

The authors report the successful surgical treatment of early infantile epileptic encephalopathy and suppression burst in 2 children with hemispheric dysplasia. At surgical evaluation, focal epileptic patterns were recorded from the abnormal hemisphere, and ictal single-photon emission computed tomography demonstrated ipsilateral regional hyperperfusion. Functional hemispherectomy was performed after electrocorticography revealed near-continuous spike and slow-wave discharges over the affected hemisphere. Histopathology revealed Taylor-type cortical dysplasia. Postoperatively, both patients are seizure free on antiepileptic drug monotherapy and have shown improved developmental status. Their favorable outcome demonstrates that children with early infantile epileptic encephalopathy due to unilateral malformations of cortical development may benefit from early surgical intervention.

[Sleep disorders in the newborn]. / Enfermedades del sueño en el recién nacido.

Sleep disorders are classified in dyssomnias, parasomnias, sleep disorder associated with medical and psychiatric disorders and proposed sleep disorders. Only the parasomnias have been studied as such in the newborn period. The parasomnias that occur in this age group are infant sleep apnea, congenital central hypoventilation syndrome, sudden infant death syndrome, and benign neonatal sleep myoclonus. Infant sleep apnea includes three entities: (1) apnea of prematurity, (2), apparent life threatening episodes with apnea and (3) obstructive sleep apnea. Congenital central hypoventilation syndrome can be associated with other autonomic system illness, such as Hirschsprung disease (Haddad syndrome) and neuroblastoma. The implementation of the supine sleep position and smoking free homes has diminished the frequency of sudden infant death syndrome. Benign neonatal sleep myoclonus should be considered in all newborns with a normal exam between the episodes when they always occur during sleep. This entity may be mistaken for status epilepticus, because it is associated with increases in heart rate. Benzodiazepines prolongs the duration of the episodes.

[Medical and surgical indications of evoked potentials]. / Indicaciones médicas y quirúrgicas de los potenciales evocados.

Evoked potentials (EP) are more sensitive than specific, safe, objective and reproducible neurophysiologic techniques to assess integrity of peripheral and central sensory and motor pathways. The integrity of pathways are determined by measurement the latency of the recorded and digitally averaged electrical events elicited after adequate stimulation. EP are medically and surgically recommended in conditions in which the diagnoses, determination of neurological outcome in comatose and under anesthesia patients, and determination of the course and evaluation of treatment are uncertain with neurologic examination and other neurodiagnostic studies.

Fetal deformations: a risk factor for obstetrical brachial plexus palsy?

The purpose of this report is to discuss the association of brachial plexus palsy and congenital deformations. We reviewed all charts of patients less than 1 year of age with obstetrical brachial plexus palsy evaluated by one of the authors (IA) between January 1998 and October 2005 at Miami Children's Hospital Brachial Plexus Center. Of 158 patients with obstetrical brachial plexus palsy, 7 had deformations (4.4%). Deformations were present in 32% of patients delivered by cesarean section, but in only 2% of patients delivered vaginally. The deformations were ipsilateral, involving the chest in two patients, distal arms in two patients, proximal arm in one patient, ear in one patient, and the leg in one patient. All patients with deformations had unilateral Erb's palsies. None had a history of maternal uterine malformation. Two presumptive mechanisms of injury, one causing the deformation (compressive forces) and one causing brachial plexus palsy at the time of delivery (traction forces), were present in all cases. The higher incidence of deformation in patients with obstetrical brachial plexus palsy born by cesarean sections and the presence of two presumptive mechanisms in all of the cases presented here raises the possibility that fetal deformations are a risk factor for obstetrical brachial plexus palsy.

Characteristic brain magnetic resonance imaging (MRI) findings in neonates with tuberous sclerosis complex.

We describe the brain magnetic resonance imaging (MRI) findings in eight neonates with tuberous sclerosis complex to further delineate the spectrum of characteristic findings in these patients. In addition to the previously described characteristic brain MRI findings in neonates, which included cortical tuber, transmantle dysplasia, subependymal nodules, cerebral subependymal giant cell astrocytomas, white-matter anomalies, and hemimegalencephaly, we found one neonate with cerebellar subependymal giant cell astrocytomas and one patient who had a normal MRI and computed tomographic scan of the brain at 4 days of age but subsequently was found to have cortical tubers and subependymal nodules by MRI.