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ABSTRACT: 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.
Anales de Pediatría 05/2004; 60(4):369-72. · 0.77 Impact Factor
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Anales espanoles de pediatria 12/1999; 51(5):540-2.
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ABSTRACT: 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.
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.
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.
Revista de neurologia 32(9):835-7. · 0.65 Impact Factor
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ABSTRACT: 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.
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%).
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.
Revista de neurologia 34(2):109-14. · 0.65 Impact Factor
