Bronchial trifurcation in a congenital pulmonary venolobar syndrome.

Congenital malformations of the tracheobronchal tree and the related arterial blood supply are a complex group of lesions in which there are abnormalities of the venous drainage and lung parenchyma. These malformations are examples of congenital pulmonary venolobar syndrome (CPVS). Tracheal trifurcation is an extremely rare anomaly associated with CPVS. We report on an unusual case of lower right extralobar sequestration connected to the trachea, plus a type I posterior laryngeal cleft, an aberrant systemic artery, and an anomalous route of the phrenic nerve. This paper discusses the place of this unusual abnormality in the spectrum of congenital bronchopulmonary vascular malformations.

Cystic dysplasia of the testis with ipsilateral renal agenesis. A case report and review of the literature.

While cystic dysplasia of the testis is rare, the awareness of the benign and malformative nature of this lesion is important to consider among other cystic testicular and para testicular lesions, first neoplasm. Its sonographic appearance and the recognition of simultaneously occurring renal anomalies should allow for prompt recognition in children with scrotal swelling or testicular enlargement. We describe a 10-year-old boy with right testicular swelling who underwent orchiectomy for cystic dysplasia of the testis.

Highly differentiated teratoma and fetus-in-fetu: a single pathology?

A case of sacrococcygeal teratoma is presented with characteristics of fetus-in-fetu. This pseudo-fetus presented a rudimentary single cavity heart, which beat at a different rate to that of the affected infant. X-ray examination showed no spinal column. This case confirms that fetus in fetu can be a remarkably complex, well-differentiated, highly organized teratoma.

Congenital diaphragmatic hernia: antenatal prognostic factors. Does cardiac ventricular disproportion in utero predict outcome and pulmonary hypoplasia?

UNLABELLED: Despite regular progress in neonatal intensive care, congenital diaphragmatic hernia (CDH) diagnosed antenatally is still associated with up to 80% mortality. It is impossible to predict which fetus with CDH will survive or not. OBJECTIVE: To identify reliable antenatal predictors of outcome and of pulmonary hypoplasia (PH) in fetuses with CDH. DESIGN: Retrospective study. SETTING: Paediatric intensive care unit of a university children's hospital. PATIENTS AND METHODS: Antenatal parameters and presence of left ventricular hypoplasia in utero were compared retrospectively to outcome and to presence of PH in 32 consecutive newborn infants with antenatally diagnosed CDH. Antenatal parameters included: gestational age at diagnosis, herniated organs, associated malformations and presence of polyhydramnios. Size of the cardiac ventricles, the aorta (Ao) and the pulmonary artery (PA) were obtained by fetal echocardiography, from which we calculated a cardioventricular index (left ventricle/right ventricle, LV/RV) and a cardiovascular index (Ao/PA). Delivery was planned in order to provide ventilatory and hemodynamic management. In case of death, PH was assessed according to the following criteria: the lung weight/body weight index and the radial alveolar count. For statistical comparisons, patients were separated into two groups: the hypoplasia group (H) and the non-hypoplasia group (NH). RESULTS: Thirty-two pregnancies were delivered. Twenty-six newborns died (81%), 6 survived (19%). When comparing non-survivors to survivors, predictors of poor outcome were: mean gestational age at diagnosis (23 vs 28 weeks, p = 0.002), intrathoracic stomach (20 vs 1 s, p = 0.01) and associated malformations (6 vs 0). Cardiac ventricular disproportion, expressed by the LV/RV ratio, appeared to correlate well with a poor outcome (0.63 in non-survivors vs 0.93 in survivors, p = 0.03) and with PH (0.63 in the H group vs 0.95 in the NH group, p = 0.03). CONCLUSIONS: Our study confirmed the factors for a poor prognosis associated with CDH previously described in the literature, but none with a consistent demonstration of accuracy. LV hypoplasia may be a more accurate predictor of outcome and of PH but it has to be assessed by prospective studies with larger samples. Further basic science and Doppler-flow studies may be helpful to understand the natural history and pathophysiology of LV hypoplasia in CDH.

Congenital diaphragmatic hernia: antenatal prognostic factors : Does cardiac ventricular disproportion in utero predict outcome and pulmonary hypoplasia?

UNLABELLED: Despite regular progress in neonatal intensive care, congenital diaphragmatic hernia (CDH) diagnosed antenatally is still associated with up to 80 % mortality. It is impossible to predict which fetus with CDH will survive or not. OBJECTIVE: To identify reliable antenatal predictors of outcome and of pulmonary hypoplasia (PH) in fetuses with CDH. DESIGN: Retrospective study. SETTING: Paediatric intensive care unit of a university children's hospital. PATIENTS AND METHODS: Antenatal parameters and presence of left ventricular hypoplasia in utero were compared retrospectively to outcome and to presence of PH in 32 consecutive newborn infants with antenatally diagnosed CDH. Antenatal parameters included: gestational age at diagnosis, herniated organs, associated malformations and presence of polyhydramnios. Size of the cardiac ventricles, the aorta (Ao) and the pulmonary artery (PA) were obtained by fetal echocardiography, from which we calculated a cardioventricular index (left ventricle/right ventricle, LV/RV) and a cardiovascular index (Ao/PA). Delivery was planned in order to provide ventilatory and hemodynamic management. In case of death, PH was assessed according to the following criteria: the lung weight/body weight index and the radial alveolar count. For statistical comparisons, patients were separated into two groups: the hypoplasia group (H) and the non-hypoplasia group (NH). RESULTS: Thirty-two pregnancies were delivered. Twenty-six newborns died (81 %), 6 survived (19 %). When comparing non-survivors to survivors, predictors of poor outcome were: mean gestational age at diagnosis (23 vs 28 weeks, p = 0.002), intrathoracic stomach (20 vs 1 s, p = 0.01) and associated malformations (6 vs 0). Cardiac ventricular disproportion, expressed by the LV/RV ratio, appeared to correlate well with a poor outcome (0.63 in non-survivors vs 0.93 in survivors, p = 0.03) and with PH (0.63 in the H group vs 0.95 in the NH group, p = 0.03). CONCLUSIONS: Our study confirmed the factors for a poor prognosis associated with CDH previously described in the literature, but none with a consistent demonstration of accuracy. LV hypoplasia may be a more accurate predictor of outcome and of PH but it has to be assessed by prospective studies with larger samples. Further basic science and Doppler-flow studies may be helpful to understand the natural history and pathophysiology of LV hypoplasia in CDH.

Computation of eyeball growth by magnetic resonance imaging (26.11.93).

The computation of eyeball growth has a wide field of applications in orbital and ocular pathology. An anatomic and MRI study to compute eyeball growth has been done. The anatomic study was made to determine the best MRI parameters to calculate the ocular surface; then this surface was computed by MRI in the neuro-ocular plane in fetuses and children aged from 0 to 13 years. The curve of eyeball growth shows rapid growth in utero and until 18 months, followed by a phase of slower growth. This method allows measurement in two dimensions, which we find complementary to ultrasound in the antenatal search for certain congenital malformations.

Visceral neuropathies responsible for chronic intestinal pseudo-obstruction syndrome in pediatric practice: analysis of 26 cases.

Twenty-six children aged from 1 day to 15 years (18 boys and 8 girls) with visceral neuropathies are analyzed. Clinical symptomatology is dominated by abdominal distension, attacks of occlusion, abdominal pain, and malnutrition. Intestine bacterial overgrowth is frequent. From aspiration biopsies, surgical rectal biopsies, and, in some cases, ileal or ileocolic biopsies, histopathological studies revealed two patterns. One group had abnormalities of the myenteric plexus identified by conventional light microscopic studies, with two patterns: [myenteric plexus hyperplasia (9 patients), characterized by large ganglionic nodes, penetration into the mucosal zone, and altered argyrophilic neurons]. Clinically this pattern was observed in four patients with multiple endocrine neoplasia syndrome with risk of medullary thyroid carcinoma. The second pattern observed was characterized by glial cell hyperplasia (15 patients). Ganglion cells are present but are small and sparse, often infiltrated by collagen tissue; Schwann nerve fibers are hypertrophic. Eleven patients presented with neonatal intestinal obstruction. The second group is characterized by normal conventional light microscopic examination, but silver stains revealed important abnormalities of argyrophobic cells (one case) or argyrophilic cells (one case). In the two groups, most of the patients needed intestinal derivation and prolonged nutritional support with total parenteral nutrition.

Urinary bladder diverticula and the Ehlers-Danlos syndrome in children.

Two large bladder diverticula that induced severe micturition disturbances were surgically removed in a 5-year-old boy with Ehlers-Danlos syndrome (EDS). One year after surgery, a new, asymptomatic diverticulum developed. This case is discussed in the light of nine similar cases that have already been published. It is concluded that EDS diverticula form a distinct group characterized by postoperative recurrence. They are not due to vesicourethral obstruction but probably result from an anomaly of the vesical wall. They are responsible for micturition difficulties and urinary tract infections. Their unavoidable recurrence means that surgery should be restricted only to symptomatic patients.