Regional anaesthesia for cleft lip surgery in a developing world setting.

BACKGROUND: The role of regional anaesthesia in cleft lip surgery in the developing world is not well documented. METHOD: A retrospective chart review of 100 patients aged >14 years who had cleft lip surgery during an Operation Smile South Africa (OSSA) volunteer surgical programme in Madagascar during 2007 and 2008. The nerve blocks used included a bilateral infraorbital nerve block, a dorsalnasal nerve block and a septal block supplemented with peri-incisional local in_ltration. Appropriateness of the regional anaesthesia alone for cleft lip surgery was determined by absence of any intraoperative complications, postoperative complications or conversions to general anaesthesia. RESULTS: Seventy-four patients commenced their operation under regional anaesthesia. There were no intraoperative or postoperative complications documented, and no patient required conversion to general anaesthesia. Two patients required additional analgesia in the immediate postoperative period. CONCLUSION: Regional anaesthesia for cleft lip surgery in patients >14 years of age was well tolerated and associated with few complications. It is a safe and effective option when used as the sole anaesthetic modality for cheiloplasty in the developing world.

Management of common bile duct stone late after laparoscopic Roux-en-Y gastric bypass for obesity.

Rapid weight loss following Roux-en-Y gastric bypass (RYGBP) for the treatment of obesity can increase the incidence of cholelithiasis formation. Nevertheless, routine simultaneous cholecystectomy at the time of bariatric surgery remains controversial. However, in case of delayed occurrence of common bile duct (CBD) stones, the difficulty to reach endoscopically the biliary tract after RYGBP should be kept in mind. We here report the case of a patient who presented with CBD stones seven years after gastric banding followed five years later by RYGBP without associated cholecystectomy. Our approach of transgastric laparoscopic assisted endoscopic retrograde cholangiopancreaticography followed by sphincterotomy and balloon stones extraction is illustrated.

Torsion of the gallbladder: two case reports.

Torsion of the gallbladder is a rare disease. It is simply defined as a rotation of the gallbladder on its mesentery along the axis of the cystic duct and cystic artery. The aetiology is unknown. However, several factors are postulated as playing causative roles. The disease symptoms mimic acute cholecystisis. The current value of radiological imaging is limited, but clinically, volvulus of the gallbladder is an acute surgical situation. It may be treated and diagnosed by a laparoscopic approach.

The association between developmental handicaps and traumatic brain injury during pregnancy: an issue that deserves more systematic evaluation.

AIMS: Trauma during pregnancy is commonly viewed as benign for the foetus when the delivery occurs normally. This study revisits that point of view. METHOD: We included eighteen patients having a neurological handicap with an anamnesis of an accident during pregnancy and a follow-up sufficient to determine a definite outcome. RESULTS: Pregnancy outcome and observed management. Foetal abnormalities were detected in six cases between the first and the thirteenth day after the trauma. Emergency delivery or rapid birth after signs of foetal distress occurred in five cases. One baby died soon after birth. One-third of cases were not submitted to any investigation. VARIOUS NEUROLOGICAL HANDICAPS WERE RECORDED: Congenital microcephaly (three patients), congenital hydrocephalus (three), Infantile cerebral hemiplegy (six), quadriplegy with severe encephalopathy (four), diplegy (one), clumsiness with cerebellar atrophy (one), Moebius syndrome (one), mental retardation with autistic features (two), learning disability (one) auditory agnosia (one). Cerebral imaging showed macroscopic abnormalities in fourteen patients, evoking various pathogenetic hypotheses. CONCLUSION: The association between maternal trauma and foetal brain lesions lacks sufficient investigation in many cases. Prospective studies are needed to clarify both medical and legal issues. Guidelines are proposed for obstetrical and paediatric management after significant maternal trauma.

[Recent progress in pediatric neurological rehabilitation]. / Quoi de neuf en rééducation neurologique infantile?

New challenges in rehabilitation for children with neurological diseases directly depend on advances made in medical research and on the quality of the environment. This is relevant to motor function as a whole, to new therapeutic avenues in spasticity, to global approaches in the evaluation of cognitive and learning disabilities, as well as curative perspectives in neuromuscular disease. Networking with the family and other actors in the environmental field is essential to achieve a better social integration. A true collaboration between physicians and pediatricians is necessary to work toward more progress.

[Foix-Chavany-Marie syndrome in a child caused by a head trauma]. / Syndrome de Foix-Chavany-Marie d'origine traumatique chez l'enfant.

We report the case of a traumatic Foix-Chavany-Marie syndrome (SFMC) which is the cortico-subcortical type of suprabulbar palsy. A 10-year-old boy was brain injured in a traffic accident in August 1996. He was found comatous (initial GCS = 6) without any focal neurological deficit. The hemodynamic situation was stable even though he presented two wounds of the scalp and a hemoperitoneum that required intensive perfusions. The initial CT scan elicited a frontal fracture, ischemo hemorrhagic lesions of the right frontopolar and anterior temporal cortex. On the second day, he developed on the left side a subdural collection and a extradural hematoma which was surgically withdrawn. The comatous state ended on the ninth day. On examination, The child was awake and alert, able to understand spoken and written language but unable to speak. There was masticatory diplegia: the mouth was half open, the patient was drooling, chewing was impossible. The most striking feature was the automatic voluntary dissociation which might be observed on laughing, crying and yawning. The patient was unable to initiate swallowing but reflex swallowing was preserved once food was placed into the pharynx. The child had a deficit of voluntary control of muscles supplied by nerves V, VI, IX, X, XI. These clinical features are the hallmarks of SFMC. The first case was reported in 1837 by Magnus. The syndrome was described by Foix Chavany et Marie in 1926, and called SFMC by Weller (1993). His literature review of 62 SFMC allowed the differentiation of five clinical types: the classical and most common form associated with cerebrovascular disease, a subacute form caused by central nervous system infections, a developmental form, a reversible form in children with epilepsy and a rare type associated with neurodegenerative disorders. Bilateral opercular lesions was confirmed in 31 of 41 patients who had CT or MRI performed, and by necropsy in 7 of 10 patients. As previously reported, the outcome was poor for this boy who recovered very limited orofacial motor abilities. The medical functional readaptation was long et tedious and took in consideration the fact that the speech disturbance was anarthria and not an aphasic or an apraxic one and the age of onset of this acute acquired syndrome.

[Neurogenic bladder in children with acute transverse myelopathy]. / Troubles sphinctériens de la myélopathie aiguë transverse de l'enfant. Aspects diagnostiques, pronostiques, rééducatifs.

BACKGROUND: Neurogenic sphincter dysfunction may result from acute transverse myelopathy. The aim of this paper is to study the course of such a dysfunction and to propose management techniques. PATIENTS AND METHODS: The files of 21 children admitted at the mean age of 8 years 5 months (2 to 14 years, 8 months) for acute transverse myelopathy were retrospectively studied. RESULTS: Bladder sphincter dysfunction occurred in the first days of disease in 85% of these patients. Abnormal perception of micturition was one of the most constant and specific symptoms. Anorectal function was also impaired. Complete regressive course was noted in 38% of patients, minor sequellae in 39% and major sequellae beyond 6 months of course in 23%. None upper tract deterioration was noted after 3 years of course. Factors of favorable prognosis were early motor function recovery (especially recommencement of walking before 20 days) and early management of bladder dysfunction (inability to void had better prognosis than urinary incontinence). CONCLUSION: Early systematic bladder drainage in case of inability to void might be essential for improved prognosis.

Nongalenic cerebral arteriovenous malformations in neonates and infants. Review of 26 consecutive cases (1982-1992).

We present 26 consecutive cases of nongalenic pial arteriovenous malformations (PAVMs) diagnosed in the neonatal period or in infancy. No diagnosis was made antenatally. Presenting symptoms in neonates were systemic cardiac manifestations (54%), seizures (31%), and hemorrhages (15%). In infants, hemorrhagic strokes and hydrodynamic disorders (external or internal hydrocephaly, macrocephaly, atrophy) both occurred in 38% of cases. Systemic cardiac manifestations and seizures were rare at that age (respectively 16% and 8%). Sixty-two percent of neonates and 31% of infants already had neurocognitive disorders (assessed by pediatric neurocognitive testing: Brunet-Leizine and Denver tests) when referred. The venous drainage and its anomalies (ectasias, stenoses, thromboses) were the main causes of symptoms. Atrophy and leukomalacic lesions occurred rapidly; they express local hydrovenous disorders and are specific to this population group. Untreated neonates and infants have a poor prognosis. Endovascular treatment, although partial and challenging in all instances, represents the treatment of choice in our series. Of the eight neonates treated, one improved to normal (12.5%), while four remained stable (50%): two neurologically normal, two with mild neurological deficit. Three (37%) died despite embolization (heart failure, multiorgan failure, postoperative death). Transient neurological complications occurred in two cases (25%): hemiparesis in one patient with a rolandic and in one with a thalamic AVM. Of the eight infants successfully embolized, one was significantly improved (12.5%) and is now neurologically normal, while five remained stable (62.5%): four neurologically normal, one with mild neurological deficit. One died between two sessions of embolization from intracerebral hemorrhage (12.5%). Hemianopsy occurred in one case (12.5%) after embolization of an occipital AVM. In one additional case in a normal child we failed to embolize the last small pial AVM of four after the three others had spontaneously thrombosed. With a minimal follow-up of 18 months and a maximum of 7 years, the review of our series shows 53% of the initial group of neonates and infants growing neurologically normal after therapeutic management in our institution; 23.5% died despite treatment, and the remaining 23.5% present minor neurological deficit. When targeted at the points of angioarchitectural weakness, embolization contributes to stabilizing a lesion. It should be undertaken rapidly to avoid loss of brain substance secondary to hemorrhage, atrophy, or leukomalacia, and to allow neurocognitive recovery and normal brain maturation. In our experience, these lesions are the most aggressive ones for the maturing brain, and the most difficult to approach technically. They represent a new therapeutic field and have their own specific anatomy and physiology.

Cerebral arteriovenous malformations in children. Management of 179 consecutive cases and review of the literature.

Over the past 10 years (1982-1992), we have been actively involved in the management of 179 cerebral arteriovenous malformations (CAVMs) in children and infants. Seventy-seven were true vein of Galen malformations (VGAMs) and 102 were pial AVMs (PAVMs), i.e., developed in the subpial space. Hemorrhage occurred as the first symptom in 50% of the children with pial AVMs, but was present in none of the VGAM cases. Only 31 children were found to be unsuitable for endovascular treatment, and in 124 cases embolization was indicated as the primary treatment (104 embolization performed). Only 21 children underwent a direct surgical approach (none in the VGAM group). In the embolized group in whom treatment has been completed (n = 56), 8 children died, 39 have an anatomical cure, and 34 are clinically normal. In the group under treatment (n = 48), 16 are not normal. The problems are timing and the aims (total or partial treatment) of the therapeutic procedures. In the nonembolized group (n = 31), 8/13 of the pial lesions were operated on (no mortality, 2 patients with moderate neurological deficits). In the VGAM group 13/18 died and 4 had spontaneous thrombosis (only 1 is neurologically normal). In the nonembolized group 13 lesions have been completely excluded, but only 5 patients are neurologically normal. This fact again stresses the need for prognostic evaluation before treatment and a clear definition of the treatment aims. Analysis of a large number of published series on the management of children with AVMs (1017 cases) reveals inconsistencies that hamper proper evaluation and comparison. In our experience, endovascular treatment always seems to be the best primary treatment in both VGAMs and PAVMs. However, management of children with these lesions requires a large multidisciplinary team, which is the only way of offering the most suitable and effective treatment, the sole guarantee of a good result.

[Angiokeratoma and fucosidosis. Immunohistochemical and ultrastructural study]. / Angiokératomes et fucosidose. Etude immunohistochimique et ultrastructurale.

INTRODUCTION: Angiokeratoma can lead to diagnoses other than Fabry's disease. We report a case of angiokeratoma in a child with fucosidosis. CASE REPORT: A 7-year-old child with psychomotor retardation presented angiokeratoma located on the penis. Uptake of type I Ulex Europaeus Agglutinin antilectin antiserum was intense in the endothelial structure. This antibody is specific for alpha-L-fucose residues which were thus found in large quantities in the vacuoles of the ultrastructure. The patient also had a major deficiency in leukocyte, serum and fibroblast alpha-fucosidase. COMMENTS: This is a typical case of fucosidosis, a rare hereditary disease with autosomal recessive transmission due to generalized deficiency in alpha-L-fucosidase. Diffuse angiokeratosis should suggest, other than Fabry's disease, fucosidase and other enzyme deficiencies including sialidase, GM1 gangliosidase as well as Kanzaki's disease.

[Syndrome of delayed maturation of the sucking-swallowing reflex]. / Syndrome de retard de maturation de la succion-déglutition.

BACKGROUND: Neonates with neurological diseases often have difficulty in sucking or swallowing. This report describes such difficulties in a group of infants with normal development, suggesting late maturation of sucking and swallowing. POPULATION AND METHODS: Seven infants (four girls, three boys) had suffered from aspiration since their first day of life (four cases) with severe asphyxia (two cases), late repeated episodes of airway obstruction by accumulated secretions (two cases), and weak isolated sucking and swallowing (one case). The lack of sucking and swallowing or difficulty with them were the main manifestations in these newborns: the face lacked expression and the tongue movements were abnormal with, in two cases, hyper-extension of the neck. A second phase during the first months of life was marked by episodes of bradycardia and/or drowsiness. A third phase during the first years of life was characterized by repeated episodes of respiratory infections, recurrent stridor and accumulation of saliva. These manifestations required prolonged hospitalization (mean: 2.5 months), nasogastric feeding, and in two cases, surgery. The investigations during the acute phases included a cineradiographic study of swallowing, laryngoscopy, monitoring of the distal esophagus pH, a barium swallow, polygraphic recording during sleep and MR imaging of the brain stem. The difficulties in sucking or swallowing disappeared before the age of 6 months (two cases), between 6 and 12 months (one case), between 1 and 5 years (four cases) without any sequelae or speech disorders. CONCLUSION: These transient sucking or swallowing difficulties suggest late maturation of praxis. They required specialized prolonged education after careful, essentially clinical, investigation.

Central nervous system lesions in hypomelanosis of Ito: an MRI and pathological study.

A severe form of hypomelanosis of Ito is reported, which presented as fetal macrocephaly and neonatal epileptic encephalopathy. Lymphocyte karyotypes were normal. MRI showed an absence of delineation between cortical grey matter and white matter. The prominent neuropathological finding was an abnormal cortical morphogenesis, with the co-existence of cells migrating normally and cells exhibiting arrêt en route or even the complete absence of migration. Intense astrocytic reaction with moderate dystrophic features was present. Juxtaposition of two migration behaviours in the neural cells paralleled the cutaneous findings and reinforced the hypothesis of a genetic chimerism.

[Atypical electroencephalographic activity in terminal phase subacute sclerosing panencephalitis]. / Activité électroencéphalographique atypique lors d'une panencéphalite sclérosante subaiguë en phase terminale.

The authors describe the evolution of the EEG during a 3-yr period in a case of subacute sclerosing panencephalitis (SSPE) revealed by epilepsy in a 9 yr-old boy. During the first 2 yr, the EEG was markedly abnormal (diffuse spike-waves, delta rhythms). During the static period, it looked like a continuous non-convulsive status epilepticus. The typical periodic abnormal activity only appeared at the terminal stage, and after a Valium test. Then slow wave complexes persisted on a dysrhythmic background. This type of EEG has never been described; our study stressed the efficacy of the Valium test in this particular case.