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.

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.

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.

[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.

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.

Near infrared spectroscopy in the management of status epilepticus in a young infant.

We report the medication management of electroencephalographic status epilepticus with subtle clinical manifestations in a young infant while simultaneously recording electroencephalographic activity and cerebral regional oxygen saturation (rSO(2)) index using near infrared spectroscopy (NIRS). We found that antiepileptic drugs equally influence the frequency of rSO(2) index fluctuations and electroencephalographic seizures. The purpose of this report is to illustrate the use of NIRS in the medication management of an infant with status epilepticus and subtle or no clinical manifestations; and to suggest that if future studies confirm our finding, NIRS may be reliably used to gauge the effects of antiepileptic medications in similar patients.

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.

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.

Bilateral decreased oxygenation during focal status epilepticus in a neonate with hemimegalencephaly.

Early surgical removal of a dysplastic hemisphere appears to be beneficial for neonates with hemimegalencephaly and medically resistant seizures. We analyzed the changes in the cerebral regional oxygen saturation index in a neonate with tuberous sclerosis and right hemimegalencephaly (1) during seven episodes of right hemisphere electroencephalographic status epilepticus with and without clinical manifestations and (2) after right hemispherectomy. The cerebral regional oxygen saturation index demonstrated marked fluctuations and progressive decline in both hemispheres during the episodes and normal values in the remaining hemisphere after surgery. We speculate that decreased oxygenation of the nonepileptic cerebral hemisphere in patients with hemimegalencephaly and medically resistant seizures can contribute to the production of global neurologic impairments in these patients and that the benefits of early hemispherectomy are due to the improved oxygenation of the nondysplastic hemisphere following surgery.

Cerebral regional oxygen fluctuations and decline during clinically silent focal electroencephalographic seizures in a neonate.

We describe a neonate with tuberous sclerosis complex and right frontal cortical dysplasia who underwent simultaneous near-infrared spectroscopy and electroencephalography (EEG) during repetitive clinically silent right frontal EEG seizures. The seizures produced a progressive decline in regional oxygen saturation index and wider regional oxygen saturation index fluctuations in the right hemisphere than in the left hemisphere. We conclude that recurrent clinically silent focal EEG seizures in this neonate were associated with lateralizing near-infrared spectroscopy changes suggestive of relative cerebral hypoxia.

Opsoclonus: an early sign of neonatal herpes encephalitis.

Preferential involvement of certain central nervous systems areas by specific viruses provides a valuable guide to the selection of antiviral agents. We report a neonate that developed opsoclonus 7 days prior to the diagnosis of herpes simplex type 2 cerebellitis. The course of the opsoclonus paralleled the clinical course and radiologic evolution of the infection. The purpose of this report is to describe opsoclonus as a possible early sign of herpes simplex type 2 central nervous system infection.

Cervicothoracic extradural arachnoid cyst: possible association with obstetric brachial plexus palsy.

The association of cervicothoracic extradural arachnoid cysts and obstetric brachial plexus palsy has not previously been reported. We report two patients with this association. The first patient is a 9-month-old boy with left obstetric brachial plexus palsy that developed bilateral leg weakness at 6 months of age owing to compression of the spinal cord by a C6 to T8 left cervicothoracic extradural arachnoid cyst. The second patient is a 3-year-old girl with bilateral brachial plexus palsy and spastic paraparesis who had magnetic resonance imaging at 3 days of age that showed intraspinal cord injury and a cervicothoracic extradural arachnoid cyst compressing the spinal cord. We believe that the association of cervicothoracic epidural arachnoid cysts and obstetric brachial plexus palsy in these patients was causal and recommend that the possibility of a cervicothoracic epidural arachnoid cyst be considered in patients with brachial plexus palsy and evidence of spinal cord injury.

Intrauterine shoulder weakness and obstetric brachial plexus palsy.

Obstetrical brachial plexus injury occurs when the forces preventing the stretch of the brachial plexus are overcome by the forces stretching it. This report describes an 8-day-old male delivered by uncomplicated cesarean section with right obstetrical brachial plexus palsy and congenital arm atrophy. The patient had a history of decreased right arm movement detected by fetal ultrasound at 18 to 20 weeks of gestation. The purpose of this article is to report that stretching of brachial plexus at birth sufficient to produce a plexus injury may occur in a patient with a vulnerable plexus even in the absence of traction during delivery.

Shoulder function following late neurolysis and bypass grafting for upper brachial plexus birth injuries.

Eleven children ranging in age from 9 to 21 months underwent late nerve reconstruction for persistent shoulder paralysis following an upper brachial plexus birth injury. Only neurolysis was performed in three patients. Neurolysis and nerve grafting bypassing the neuroma with proximal and distal end-to-side repairs was performed in the other eight. All patients were followed for 2 or more years. Two patients underwent a secondary procedure before their final follow-up evaluation. All infants demonstrated significant improvement when assessed by a modified Gilbert shoulder motion scale.

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.