Search

Presents a letter to the editor of "Anaesthesia" concerning the hazards of submental tracheal intubation.

Megacystis-microcolon-intestinal hypoperistalsis syndrome is a multisystemic disorder in which impaired intestinal motor activity causes recurrent symptoms of intestinal obstruction in the absence of mechanical occlusion, associated with bladder distention without distal obstruction of the urinary tract. It is an uncommon disorder with high morbidity and mortality, and long-term total parenteral nutrition may be required. We describe an 18-year-old girl with long-term survival, followed-up by our service since birth. With medical, surgical and nutritional management she currently presents a good oral tolerance and good quality of life, only requiring parenteral nutrition during periods of decompensation.

Both endotracheal intubation and the laryngeal mask are already established and have their specific applications. The Combitube SA applied submentally is useful for patients with serious craniomaxillomandibular fractures, particularly those which disturb the occlusion and cause serious haemorrhage with attendant difficulties for intubation. Combitube SA used via the submental route will facilitate fracture reduction and temporary maintenance of fractures, so that bleeding, pain and airway problems may be managed more easily until alternative intubation has been achieved.

Introduction and Objectives: Children often consult for fictional or very exaggerated symptoms, grouped together as factitious disorders (TF), disorders due to somatomorphic pain (TDS), disorders due to somatization (TS) and conversion disorders (TC). Patients and method. We studied cases of TF, TDS, TS and TC evaluated by the neuropaediatric department of the Hospital Miguel Servet de Zaragoza between May 1990 and August 2001., Results: There were 134 children identified as having TF TDS TS TC: There were 91 girls (67.9%) and 43 boys (32.1%). They made up 2.47% of the 5,417 children included in the neuropaediatric data base. The mean age was 10 years and 9 months, ranging between 3 and 16 years with only 9 children aged under 7 years. The commonest syndromes were: paroxystic disorders, headache, other pain, paraesthesia, hypovision, and other visual alterations, paresias, tremors and other disorders of movement, disorders of gait, lack of air, hyperventilation and dysphagia. There was often more than one motive for consultation, with sometimes more than four. The commonest complementary tests done were: EEG, fundus oculi, cranial CAT scan, CPK, EMG/ENG, cranial MR, spinal MR, CSF and osseous gammography. No further studies were made of 12 children (9%)., Conclusions: The TF TDS TS TC are a common cause of assessment in neuropaediatric practice. It should be suspected in cases of multiple symptoms in children aged over 6 years, usually in girls. It is often necessary to carry out various complementary tests to rule out organic disorders.

Chickenpox in childhood is usually a self-limiting disease with relatively few and benign complications, although serious complications do occur. Renal involvement is uncommon during the course of this disease. We describe a 6-year-old girl who developed clinical glomerulonephritis, with arterial hypertension, hematuria and transient minor disturbance of renal function revealed by creatinine clearance 2 days before the appearance of papulovesicular skin lesions characteristic of chickenpox. The patient recovered spontaneously in 2 weeks.

Introduction: The commonest cause of malfunction of shunts for cerebrospinal fluid is obstruction of the catheter, which may be of subacute or sudden onset. When onset is sudden, the complication is serious as this implies risk of death or permanent damage to vision., Clinical Case: We describe the case of a 13 year old girl who, 8 days after insertion of a ventriculo-peritoneal shunt valve, complained of the sudden onset of headache, reduced level of consciousness, pupil changes and permanent loss of vision secondary to a bilateral occipital infarct., Conclusions: The visual pathways and oculomotor nerves are particularly sensitive to changes in intracranial pressure and displacement of intracranial structures. Therefore, neuro-ophthalmic signs are more sensitive than computerized tomography (CT) in detection of mechanical malfunction of the valve. Although CT shows structural anomalies, it cannot assess their function. The intracranial pressure may be high enough to cause damage to the visual pathways, although no enlargement of the ventricles can be seen on CT. A normal CT, or one similar to previous scans, does not rule out malfunction of the shunt. If clinical signs of raised intracranial pressure are observed, the possibility of mechanical malfunction of the valve should be considered and this should be explored as a neurosurgical emergency in view of the risk of death or permanent loss of vision.

Introduction: Varicella in childhood is a self-limiting disease which usually follows a benign course. However, a considerable number of complications occur, particularly involving the nervous system. On rare occasions hemiparesis has been reported following an ischemic cerebrovascular accident related to varicella., Clinical Case: We report the case of a 4 year old boy who presented with right hemiparesis eight days after the onset of varicella. CAT and MR showed a cerebral infarct involving the left caudate nucleus, putamen and internal capsule. Other possible causes of cerebral infarct were ruled out. Four years later he had slight residual right hemiparesis. The pathogenesis may have been due to vasculitis of the intracranial arteries caused by direct invasion of the vessels by virus from the nearby nerve tissue. Another theory suggest similarities with moyamoya disease, suggesting that lesions of the head and neck stimulate the superior cervical ganglion and this, together with the production of circulating immuno-complexes may be the cause of the vasculopathy of the intracranial blood vessels., Conclusions: In most cases of cerebrovascular accidents occurring in childhood the cause is unknown. However, it is important to discover the cause since the risk of recurrence often depends on the primary disease. Once other commoner causes have been excluded, one should ask whether there is a history of varicella, since the prognosis is generally good and recurrence uncommon.

Aplasia cutis congenita is a rare condition characterized by the congenital absence of epidermis, dermis and, in some cases, subcutaneous tissues. It was first described by Cordon in 1767 and more than 500 cases have been reported since, with an estimate incidence of 3 in 10,000 births. The lesions may occur on any body surface although localised agenesis of the scalp is the most frequent pattern. In approximately 20% of cases underlying bone defects are also found. Aplasia cutis congenita occurs as an isolated defect or with other associated anomalies. There is no unifying theory for the pathogenesis and large scalp defects present a management dilemma. We report a newborn with a large scalp defect in the midline at the vertex without associated malformations. There was no significant family history. Skull and extremities radiographs, chromosome analysis, cerebral and abdominal sonography were normal. Two methods of treatment were used: a conservative approach consisting of daily antiseptic dressing to allow scalp epithelialization improved conditions for secondary surgery at 30 days of life, closing the defect with local rotational flaps. The postoperative course was uneventful and an excellent cosmetic result was achieved.

Introduction: Pelizaeus-Merzbacher's disease involves extensive demyelination of the Central Nervous System. This is due to a defect in the gene for proteolipoproteins, found on the X chromosome. It appears early as marked axial hypotonia, stridor, nystagmus and anomalous movements of the head, and later as variable pyramidal, extrapyramidal and cerebellar involvement., Clinical Case: A three year old boy, with no unusual family history, was seen for psychomotor retardation. He had marked hypotonia, absence of evidence of social contact, inspiratory stridor, nystagmus and horizontal nodding movements of the head. MR at 4 months showed absence of supratentorial and infratentorial myelinization. Analysis of the gene for proteolipoproteins showed a specific mutation on the exon 5 C227Y. At eight and a half years there was persistence of the severe axial hypotonia with minimal visual function and social contact was maintained through hearing., Conclusions: It is possible to make an early diagnosis of Pelizaeus-Merzbacher's disease in the first weeks of life, on the typical clinical picture and MR findings of marked extensive hypomyelination, although assessment of myelination is difficult at this age. Early diagnosis is very important, since molecular analysis of the proteolipoproteins gene permits confirmation of the diagnosis, identification of heterozygotes and the establishment of prenatal diagnosis.

Introduction: Epidural haematoma is a complication which may occur after apparently minor head injury. It may lead to the sudden onset, after a variable lucid interval, of transtentorial uncal hernia with consequent risk to life. If drainage is done early, the prognosis is excellent., Clinical Case: We present a case of right parietal epidural haematoma in a 5 year-old-boy following a minor head injury, with no fracture of the skull. After a clear interval of 40 hours his consciousness deteriorated, there was right mydriasis and extension hypertonia of arms and legs. Following surgical removal there was evidence of an extensive cerebrovascular accident in the territory of the middle cerebral artery., Conclusions: Although they are infrequent, infarcts have been described in relation to epidural haematoma in all cerebral territories, predominantly in the distribution of the posterior cerebral artery. In the territory of the middle cerebral artery this may be due to traction and stenosis or occlusion of the small perforating branches, secondary to the displacement of midline cerebral structures or the space-occupying effect of epidural haematoma. All head injuries, including minor injuries mean a risk of serious hemorrhagic complications, the prognosis of which depends on the rapidity with which they are treated., Guidelines: Adapted to each centre are necessary for a common problem, usually without complications, but which may have unpredictably fatal results. Head injury is a dynamic process and therefore requires close clinical observation, which cannot be substituted by any complementary test.