thiopental and Appendicitis

Robinow syndrome, originally described in 1969, consists of mesomelic brachymelia, short stature, genital hypoplasia and characteristic facies. Associated organ system involvement may include developmental delay, congenital heart disease, obstructive disorders of the urinary tract and renal cysts. As there is a potential for airway problems related to the facial features, such as midface hypoplasia and micrognathia, perioperative management of these patients is important. The authors present an 8-year-old boy, previously diagnosed with Robinow syndrome, who was admitted for an emergency surgical procedure. The perioperative implications of this disorder are reviewed.

Topics: Abnormalities, Multiple; Anesthesia; Anesthetics, Inhalation; Anesthetics, Intravenous; Appendectomy; Appendicitis; Child; Face; Fentanyl; Genitalia, Male; Growth Disorders; Humans; Isoflurane; Laparoscopy; Limb Deformities, Congenital; Male; Syndrome; Thiopental

The acute porphyria is an autosomal dominant disorder of the héme biosynthesis enzyme. The aim of this work is to determin a diagnostic step in order to anticipate porphyric crises. We report the observation of a child which was hospitalized in the pédiatric service three years ago for peripheric arthralgies and myalgies where the diagnosis of an acute porphyria was not posed. He wase admitted in our service for appendicitis, he was operated, the anesthesie was carried out by the thiopenthal and the succinylcholine. At the first post-operative day, a respiratory insufficiency was the cause of death. The rate of coproporphyrines and of uroporphyrines in the urine had confirmed the diagnosis of acute porphyria. The positive diagnostic of acute porphyria is difficult, the association of abdominal, psychological and neurologic signs must suggest the diagnostic, all the more, if the urines have a dark colour. The presence of uroporphyrines and coproporphyrines and certain precursors in the urines or in the high-rated stools confirm the diagnostic. The family survey and dosage of porphyric compounds in the sickness-bearing patient's family members allow to reveal certain asymptomatic forms of the heriditary acute porphyria, it is caused by certain products used in neuropsychiatry, in aneasthesic or in certain circunstances, such as, the infection or the trauma. The treatment of the acute porphyria is an emergency wich requires the transfert of the patient to a unit of intensive care.

Topics: Acute Disease; Anesthetics, Inhalation; Anesthetics, Intravenous; Appendicitis; Child; Diagnosis, Differential; Fatal Outcome; Halothane; Humans; Pedigree; Porphyria, Acute Intermittent; Postoperative Complications; Prognosis; Respiratory Insufficiency; Thiopental

Topics: Adjuvants, Anesthesia; Adolescent; Adrenal Gland Neoplasms; Anesthesia, General; Appendicitis; Humans; Hypertension; Male; Nitrous Oxide; Pheochromocytoma; Preanesthetic Medication; Thiopental

Topics: Anesthesia; Anesthesia, Inhalation; Anesthetics; Appendectomy; Appendicitis; Drug Therapy; Halothane; Humans; Metabolism; Porphyria, Acute Intermittent; Porphyrias; Porphyrins; Succinylcholine; Thiopental