[Inguinal hernia in children: easily incarcerated]. / Liesbreuk bij kinderen: snel beklemd.

Inguinal hernia in children is caused by an incomplete obliteration of the vaginal process during the embryological development. The vaginal process can thus become a hernia sac that often contains bowel and in girls, an ovary. The diagnosis of inguinal hernia is made by history and physical examination. According to current guidelines surgical repair should be performed without delay to avoid incarceration, which gives a high risk of complications, including testicular atrophy and ischemia of vital organs. However, patients are regularly not referred adequately. We present three cases of children who developed complications of a non-repaired inguinal hernia. Additionally, the data of all children with a congenital inguinal hernia, surgically treated from January 2018 until August 2019 show that out of 243 children 13.6% presented acutely with an incarcerated inguinal hernia. Another 6% received a wrong advice from their primary care doctor and was not referred to a (pediatric) surgeon.

Hypoxic/ischemic and infectious events have cumulative effects on the risk of cerebral palsy in very-low-birth-weight preterm infants.

BACKGROUND: Hypoxia/ischemia and inflammation are two major mechanisms for cerebral palsy (CP) in preterm infants. OBJECTIVE: To investigate whether hypoxia/ischemia- and infection-related events in the perinatal and neonatal periods had cumulative effects on CP risk in very-low-birth-weight (VLBW) premature infants. METHODS: From 1995 to 2005, 5,807 VLBW preterm infants admitted to Taiwan hospitals were enrolled. The cumulative effects of hypoxic/ischemic and infectious events during the perinatal and neonatal periods on CP risk at corrected age 24 months were analyzed. RESULTS: Of the 4,355 infants with 24-month follow-up, 457 (10.5%) had CP. The CP group had significantly higher incidences of hypoxia/ischemia-related events in the perinatal and neonatal periods, and sepsis in the neonatal period than the normal group. Three hypoxic/ischemic events, including birth cardiopulmonary resuscitation (OR 2.25; 95% CI 1.81-2.82), patent ductus arteriosus (PDA) ligation (2.94; 1.35-5.75) and chronic lung disease (3.14; 2.61-3.85) had the most significant contribution to CP. Relative to CP risk for infants with neither the three hypoxic/ischemic events nor sepsis, the CP odds increased 1.98-, 2.26- and 2.15-fold for infants with birth cardiopulmonary resuscitation, PDA ligation and chronic lung disease, respectively; while the combination with sepsis further increased the odds to 3.18-, 3.83- and 3.25-fold, respectively. Using the three hypoxic/ischemic events plus sepsis, CP rates were 10.0, 16.7, 26.7, 40.0 and 54.7% for infants with none, one, two, three and four events, respectively. CONCLUSIONS: Hypoxic/ischemic and infectious events across the perinatal and neonatal periods exerted cumulative effects on CP risk in VLBW premature infants.

Intrauterine gangrene of the lower extremity in the newborn: a report of two cases.

We describe 2 cases of intrauterine gangrene involving the lower extremity in 2 unrelated neonates. Both cases were complicated by prematurity, and 1 case was complicated by an intrauterine distal femur fracture and twin-twin transfusion syndrome. Both cases resulted in profound ischemic necrosis from the knee to the foot, requiring knee disarticulation. In both cases, a follow-up period of 7 years is now completed, and no further medical or surgical complications have arisen.

New targets for pharmacological intervention in the glutamatergic synapse.

Excitotoxicity is thought to be a major mechanism in many human disease states such as ischemia, trauma, epilepsy and chronic neurodegenerative disorders. Briefly, synaptic overactivity leads to the excessive release of glutamate that activates postsynaptic cell membrane receptors, which upon activation open their associated ion channel pore to produce ion influx. To date, although molecular basis of glutamate toxicity remain uncertain, there is general agreement that N-methyl-d-aspartate (NMDA) subtype of ionotropic glutamate receptors plays a key role in mediating at least some aspects of glutamate neurotoxicity. On this view, research has focused in the discovery of new compounds able to either reduce glutamate release or activation of postsynaptic NMDA receptors. Although NMDA receptor antagonists prevent excitotoxicity in cellular and animal models, these drugs have limited usefulness clinically. Side effects such as psychosis, nausea, vomiting, memory impairment, and neuronal cell death accompany complete NMDA receptor blockade, dramatizing the crucial role of the NMDA receptor in normal neuronal processes. Recently, however, well-tolerated compounds such as memantine has been shown to be able to block excitotoxic cell death in a clinically tolerated manner. Understanding the biochemical properties of the multitude of NMDA receptor subtypes offers the possibility of developing more effective and clinically useful drugs. The increasing knowledge of the structure and function of this postsynaptic NMDA complex may improve the identification of specific molecular targets whose pharmacological or genetic manipulation might lead to innovative therapies for brain disorders.

[Selective intraarterial axillar artery thrombolysis in a neonate: angiographic and thermographic serial time course]. / Selektive intraarterielle Lysetherapie bei neonatalem Arteria-axillaris-Verschluss: Angiographische und thermographische Verlaufskontrolle.

Arterial thrombotic vascular occlusions in the neonatal period are rare. Intraarterial thrombolytic therapy in infants and children is unchanged rarely used but can be very effective. Thrombolytic therapy in adults is well established and a summary of clinical and pharmacologic aspects exists of different thrombolytic agents. Recombinant-Tissue-Plasminogen-Activator (rTPA) is increasingly used in thrombolytic therapy in infants and children in the last years. We report thrombolysis of axillar arterial thrombosis with rTPA in a premature infant. Additional thermographic images were performed, which were very helpful in the clinical monitoring of the newborn.

Successful surgical management of a neonate with a saddle pulmonary embolus.

A female neonate presented with evidence of a massive ventilation-perfusion mismatch. She was subsequently found to have a saddle pulmonary embolus. The infant successfully underwent surgical pulmonary embolectomy.

[An ischemic pulmonary malformation of the surviving fetus occurring after selective embryo reduction in bichorionic pregnancy. Report of one case and literature review]. / Une anomalie pulmonaire ischémique inhabituelle du foetus survivant après interruption sélective sur grossesse bichoriale. A propos d'un cas et revue de la littérature.

We describe, to our knowledge, the first case of a pulmonary malformation called acinar dysplasia occurring at a surviving fetus after selective embryo reduction in a bichorionic pregnancy. The chronological and histological observations suggest that this anomaly may be linked with a feticide achieved at 13 week's gestation. Literature review concerning selective embryo reduction shows rare cases of vascular connections in bichorionic pregnancies especially during the first half of gestation, that can explain in part the apparition of survivor's anomalies.

Congenital ischemic forearm necrosis associated with a compound presentation.

An infant born in a compound presentation had ischemic forearm necrosis requiring amputation. Bilateral renal vein thrombosis predating labor and delivery was also diagnosed. The possibility of congenital ischemic necrosis in an extremity should not affect the obstetrical management of compound presentation, but if this complication occurs a search for an underlying coagulopathy is important.

Intrauterine vascular deficiency of the upper limb.

We report six cases of intrauterine vascular deficiency of the upper limb, presenting over a 12-year period, with established areas of necrosis at the time of birth. Three of the six mothers were diabetic. An urgent examination of the neonate should be done to exclude occult systemic thomboses, as early anticoagulation or thrombolytic therapy may be indicated. The majority of cases were managed conservatively with dressings and splintage of the limb; exceptionally surgical intervention was required. Long-term sequelae included flexion contractures and shortening of the forearm bones.

[Severe protracted intrauterine impaired perfusion--a cause of enteral motility disorder in the premature infant]. / Die schwere protrahierte intrauterine Perfusionsstörung--eine Ursache der enteralen Motilitätsstörung des Frühgeborenen.

The importance of measurement of blood flow in the fetal and uteroplacental circulations for the assessment of fetal wellbeing has been undisputed since some years. The present study is designed to prove if any relationship exists between severe hemodynamic disturbance in fetal as well as uteroplacental vessels and the occurrence of postnatal impairment of intestinal motility. The progress of 130 children, born in the University Women's Hospital Leipzig between 1991-1993 and with birth weights below 1500 g, has been analyzed. Doppler ultrasound examinations for detection of impairment in fetal and uteroplacental circulation were performed in all cases during pregnancy. A severe impairment of blood flow in the above mentioned circulations was defined by the presence of pathological pulsatility or resistance indices in both fetal and uteroplacental vessels as well as absent end diastolic flow in the umbilical artery and signs of centralization in the fetus. A severe hemodynamic impairment was found in 27 children and 26 of these were classified as severe hypotrophic after birth. The progress of these children was compared with this of other hypotrophic and euthrophic premature babies who had not revealed hemodynamic abnormalities. The incidence of disturbed postnatal intestinal motility (delayed meconium excretion, abdominal distention, retrograde peristalsis, subileus) was significantly higher in hypotrophic neonates with hemodynamic abnormalities in the course of pregnancy. Four of these newborns underwent surgery and surgical findings did not correlate with enterocolitis. The resumption of oral food intake for neonates who had hemodynamic impairments during pregnancy was delayed compared with the control groups.(ABSTRACT TRUNCATED AT 250 WORDS)

[Neonatal testicular ischemic necrosis without torsion, associated with antithrombin III deficit]. / Nécrose ischémique testiculaire néonatale sans torsion, associée á un déficit en antithrombine III.

There have been few reports of neonatal ischemic necrosis of the testis without torsion of the spermatic cord, which may be caused either by compression in utero or by transient torsion of the spermatic cord resolving spontaneously before surgery. The patient reported herein developed an inflammatory swelling of the right scrotum and an ecchymotic plaque over the left thigh at four days of age. Ischemic necrosis of the right testis without torsion was found upon surgery. When the patient was ten days of age, he developed an inflammatory swelling in the left scrotum; ischemic necrosis of the left testis without torsion was again found upon surgery. Thrombosis of the spermatic vessels was suspected. Postoperatively, ecchymotic and necrotic skin lesions developed, followed by pulmonary embolism and cerebral thrombosis. Outcome was fatal. Hematologic tests in the neonate and his parents established the diagnosis of inherited antithrombin III deficiency. Ischemic necrosis of the testes was thus probably due to hypercoagulability. No similar cases have been reported to date.

Peripheral ischaemia and gangrene presenting at birth.

The aetiology of non-iatrogenic causes of peripheral ischaemia and gangrene presenting either at birth or within a few hours of delivery is unknown in the majority of 56 confirmed cases. In this review of 47 cases occurring since 1941 the aetiology was clear in only 6, four due to compression by the encircling umbilical cord. There was no clear association with gestational age, birth weight, maternal age or type of delivery. Seven were infants of poorly controlled diabetic mothers and these may constitute a subgroup due to altered haemostatic mechanisms. Pregnancy hypertension was an association in 7 cases, oligohydramnios in 6. There is only limited support for birth trauma, sepsis, and thrombo-emboli from the ductus arteriosus as causes. There is indirect evidence that thrombo-emboli can migrate from the placental bed to the fetus. In recent years death from this condition has been rare with surgical thrombectomy increasingly successful in late presenting cases. When gangrene is established at birth surgical amputation, autoamputation, or some loss of function is usual. Peripheral ischaemic insults presenting at birth may be part of a wider spectrum of disorders, both prenatal and perinatal, attributable to occlusive vascular disruption.

[Congenital ischemic onychodystrophy (Iso-Kikuchi syndrome) and chronic lupus erythematosus]. / Kongenitale ischämische Onychodystrophie (Iso-Kikuchi-Syndrom) und chronischer Lupus erythematodes.

We report a patient with nail and bone disorders of the index and middle fingers (Iso and Kikuchi syndrome) associated with chronic discoid lupus erythematosus. Angiographic studies showed filiform arteries of the fingers and slow blood circulation. Since a vascular pathogenic mechanisms is probable, the designation "Congenital ischemic onychodystrophy" seems more suitable. The association with chronic discoid lupus erythematosus has not previously been reported.