| This case illustrates 2 main points. Firstly, fetal infection can mimic exactly both the immediate and delayed signs of perinatal asphyxia. Secondly, the placenta may hold the key to the diagnosis of sepsis which may be made difficult in the neonate by labour ward practices such as the use of intrapartum and immediate newborn antibiotics. We strongly support the recommendation that newborn blood and fetal membrane cultures should always be obtained in babies with a diagnosis of 'intrapartum asphyxia and fetal distress' (1). To this we would add the recommendation that placental histology be performed in these circumstances. ( info) |
2/181. Rare delivery complication caused by an undiagnosed uterine septum. The role of a uterine septum, and thus, metroplasty in an infertile woman is a debatable issue. A rare complication of fetal malpresentation and impaction in the uterine cavity due to undiagnosed uterine septum in a 24-year-old primigravida who conceived after 3 years of primary infertility is reported. This case highlights that uterine anomalies should be looked for in patients with infertility and reproductive failures, and should be corrected before conception by metroplasty in order to improve reproductive outcome. ( info) |
3/181. Homologous bone graft for expansion thoracoplasty in Jeune's asphyxiating thoracic dystrophy. adult tibial shaft from a bone graft bank was used as a solid homologous bone graft for midsternal expansion thoracoplasty in an infant with Jeune's asphyxiating thoracic dystrophy. The technique appeared successful, but the child grew out of her chest in her second year of life. Expansion thoracoplasty for Jeune's disease probably should only be reserved for children who survive their first year of life without major surgery. ( info) |
| PURPOSE: This article represents the first report in the ophthalmology literature of an association between pseudotumor cerebri (PTC) and recombinant human growth hormone (rhGH). DESIGN: Noncomparative case series. PARTICIPANTS: Three children receiving rhGH for short stature with turner syndrome, Jeune syndrome, or down syndrome. methods: Children underwent full ocular examination. After papilledema was identified, patients underwent lumbar puncture and imaging with either magnetic resonance imaging or computerized tomography. Treatment was under the guidance of the primary physician or neurosurgeon. The rhGH was discontinued in all children. MAIN OUTCOME MEASURES: visual acuity and evaluation of the optic nerve for resolution of papilledema were followed at each examination. RESULTS: In all three cases, papilledema resolved with the cessation of rhGH, and treatment with acetazolamide or prednisone. visual acuity was unchanged in case 1, decreased by two to three lines in case 2, and was inconsistent in case 3. One child (case 2) required a ventriculoperitoneal shunt for persistent elevation of intracranial pressure. CONCLUSION: There appears to be a causal relationship between the initiation of rhGH with the development of PTC. Children should have a complete ophthalmic evaluation if they report headache or visual disturbances. Baseline examination with routine follow-up should be instituted when children cannot adequately communicate. ( info) |
5/181. prenatal diagnosis of thoracopelvic dysplasia. A case report. BACKGROUND: Thoracopelvic dysplasia, a variant of asphyxiating thoracic dysplasia (Jeune syndrome), is an uncommon skeletal disorder characterized by a small thorax, pelvic abnormalities and other complex, combined anomalies, including hypomelia, polydactyly and renal anomalies. CASE: A 32-year-old woman, gravida 1, para 0, was referred at 27 weeks' gestation due to polyhydramnios. Sonography revealed hydramnios, low fetal thoracic circumference (TC) and abdominal circumference (AC) ratio (0.78), skull and skin edema, increased nuchal translucency (7 mm), micrognathia, low-set ears, left cardiac deviation (66 degrees), overriding fingers, and club and rock-buttock feet. amniocentesis revealed a normal karyotype (46, XY). Asphyxiating thoracic dysplasia was considered. At 40 weeks' gestation, a male infant was delivered vaginally. Besides the prenatal findings, cryptorchidism and high-arched palate were noted. radiography of the infant revealed a narrow, funnel-shaped thorax and small pelvis with short, flared iliac bones; poorly developed acetabulum; and small, shallow sciatic notch. No dyspnea was observed at five months postpartum. CONCLUSION: Thoracopelvic dysplasia should be considered when a low TC/AC ratio (< 0.8) is observed. In this case the final diagnosis was made after detailed exclusion of other disorders combined with observation of a small thorax. prenatal diagnosis of thoracopelvic dysplasia is possible. ( info) |
6/181. malaria in the neonate: report of 2 cases. Two cases of neonatal malaria are reported, both presenting with signs similar to neonatal sepsis. The first baby responded to oral chloroquine (CQ) with fever and parasite clearance times of 48 h and 60 h respectively. The CQ treatment failed in the second baby and was subsequently treated successfully with oral Halofantrine hydrochloride. These cases indicate a need for routine blood film for malaria parasite as part of initial screening for neonatal sepsis. ( info) |
7/181. Fetal arrhythmia is not always a simple vagotonia. Fetal arrhythmia (extrasystoles) is usually caused by vagotonia and thought to be innocuous. Four case reports are presented in which arrhythmia accompanied myocardial localization of tuberous sclerosis, fetal sepsis, fetal hypoxia and acidemia. ( info) |
8/181. Parasagittal cerebral injury: magnetic resonance findings. Parasagittal cerebral injury is a specific pathologic lesion that can be found in full-term newborn babies suffering from hypoxic-ischemic encephalopathy. It is defined by the presence of cortical and subcortical white-matter necrosis involving the parasagittal and superomedial areas of the cerebral convexities. We report on two patients who showed parasagittal cerebral injury on magnetic resonance imaging. In both cases antecedents of hypoxic-ischemic encephalopathy were noted. In one of the patients basal ganglia involvement was also detected by cranial magnetic resonance imaging. In the follow-up studies the presence of upper-limb pyramidal signs and dyspraxia were two of the more pronounced symptoms. We will discuss the usefulness of neuroimaging, especially magnetic resonance imaging with coronal views, in the diagnosis of the parasagittal cerebral injury. ( info) |
9/181. Jeune syndrome (asphyxiating thoracic dystrophy) associated with hirschsprung disease. We describe two children with diagnostic features of Jeune syndrome who also had hirschsprung disease. An association between the two conditions has not previously been described and has implications both for clinical management and for further study. ( info) |
10/181. Jeune syndrome and liver disease: report of three cases treated with ursodeoxycholic acid. Three children with Jeune syndrome (asphyxiating thoracic dystrophy) had clinical and laboratory evidence of liver disease. In two patients the disease evolved to biliary cirrhosis, whereas in the third it was recognized when extensive fibrosis was developing. In the three patients, treatment with ursodeoxycholic acid appeared to control the progression of the hepatic dysfunction. ( info) |