Parturient With Barnes Syndrome (Thoracolaryngopelvic Dysplasia) Undergoing Cesarean Delivery of a Neonate With Barnes Syndrome: A Case Report.

This case describes a parturient with Barnes syndrome, a rare disorder characterized by subglottic stenosis, thoracic dystrophy, and small pelvic inlet, who underwent cesarean delivery of a neonate diagnosed with Barnes syndrome. Live simulation training was performed by multidisciplinary team to prepare for the spinal anesthetic, personnel flow between 2 operating rooms, and management of various airway scenarios for the newborn. After delivery, the neonate underwent laryngoscopy-bronchoscopy with successful intubation in the operating room because of labored breathing. Airway evaluation revealed subglottic stenosis, tracheomalacia/bronchomalacia. Collaboration among perinatologists, obstetric/pediatric anesthesiologists, pediatric head and neck surgeons, and neonatologists was integral to perioperative management of both the mother and child.

Uso de la ecografía en la comprobación de la intubación traquealurgente en Pediatría: experiencia preliminar / Use of ultrasonograph in the verification of urgent tracheal intubation in pediatrics: preliminary experience

Introducción y objetivos. El método estándar para la confirmación de la intubación traqueal es la laringoscopia directa; siendo el método secundario más recomendado la capnografía. Por otro lado, existe un interés creciente en el uso de la ecografía como técnica alternativa y complementaria, con la ventaja añadida de permitir comprobar los movimientos respiratorios, sin embargo, su uso es aún limitado. Exponemos nuestra experiencia preliminar con el uso de la ecografía para este fin, describiendo e ilustrando la técnica en una pequeña serie de pacientes. Material y métodos. Se comprobó la intubación correcta en los planos longitudinal y transversal así como la ausencia de intubación bronquial selectiva mediante ecografía. Posteriormente un segundo investigador revisó y analizó las imágenes obtenidas para evaluar la concordancia entre ambos. Casos clínicos. Fueron incluidas 7 intubaciones en 5 pacientes, sin producirse en ningún caso intubación esofágica. La mediana del tiempo de comprobación fue 63,5 (28- 97,5) segundos. La posición del tubo fue considerada como correcta ecográficamente en 6 de los casos, según el signo del lung sliding y la motilidad diafragmática; sin embargo, por radiografía convencional sólo se consideró correcta en 5. En 27 de las 28 imágenes registradas hubo concordancia entre ambos investigadores. Comentarios. La ecografía parece ser un método alternativo útil y rápido para la confirmación de la intubación traqueal. En comparación con los métodos convencionales se trata de una técnica no invasiva, que permite descartar la intubación selectiva del bronquio derecho de forma rápida y podría contribuir a evitar la realización de radiografías (AU)

Introduction and objectives. Direct laringoscopy is the standard method to confirm proper endotracheal tube placement; capnography represents the second most recommended method. Nowadays, ultrasound is gaining interest as an alternative and complementary technique, which also allows the comprobation of respiratory movements. Unfortunately this use is still limited. This study aimed to show our experience with the use of ultrasound for this purpose, describing and illustrating the technique in a small series of patients. Material and methods. Proper intubation in longitudinal and transverse plane, as well as the absence of selective bronchial intubation was verified by ultrasound. Subsequently the obtained images were reviewed and analyzed by a second researcher to evaluate the correlation between them. Clinical cases. Seven intubations in five patients were included, none of them were esophagical. The average time to verify was 63.5 (28-97.5) seconds. Correct tube position was considered by ultrasound lung sliding and diaphragmatic motility in 6 cases, in contrast with 5 cases by conventional radiography. In 27 of 28 recorded images there was an agreement between both researchers. Comments. Ultrasound appears to be a useful and fast alternative technique to confirm tracheal intubation. Compared with conventional methods it is a noninvasive technique that allows to dismiss selective right bronchial intubation in a fast way and can contribute to avoid excessive use of radiography (AU)

Thoracoplasty for treatment of asphyxiating thoracic dysplasia in a newborn.

In our case report and literature review, we report about a female newborn with severe asphyxiating thoracic dysplasia of the spondylocostal dysostosis classification to whom an expandable thoracoplasty with metal implants offered survival and discharge at home from newborn to infancy.

Lateral thoracic expansion for Jeune syndrome: evidence of rib healing and new bone formation.

BACKGROUND: Lateral thoracic expansion is a procedure that has been described to enlarge the thoracic cage in patients with Jeune's asphyxiating thoracic dystrophy. The procedure involves separating ribs from their periosteum and plating them together in an expanded fashion with titanium struts. We have speculated that the ribs heal in this situation, despite the absence of surrounding periosteum, and that new rib formation occurs in the liberated periosteum. METHODS: Radiographic studies of patients who have undergone lateral thoracic expansion were reviewed for evidence of rib healing or periosteal new bone formation. RESULTS: This study presents radiologic evidence that rib healing actually occurs, as does periosteal ossification. CONCLUSIONS: Lateral thoracic expansion creates additional chest wall that is formed of autologous tissue, fully healed, and not ultimately dependent on titanium struts.

[Rupture of a renal artery aneurysm during delivery]. / Ruptur eines Nierenarterienaneurysmas unter der Geburt.

INTRODUCTION: Renal artery aneurysms represent a rare entity with an incidence of 0.3 - 1.3% in angiographies. Rupture of such aneurysms are rare. During pregnancy there is an increased risk of rupture with a high mortality for mother and child. We report the first case of rupture of a left renal artery aneurysm during delivery with survival of mother and child. CASE REPORT: Due to arrest of labor during a home birth, the patient was admitted to the hospital, where an immediate forceps extraction on account of fetal asphyxia was performed. Immediately after delivery a hypovolemic shock developed. Ultrasound examination showed a huge subsplenic hematoma. Emergency laparotomy revealed a ruptured renal artery aneurysm. The renal artery was ligated on the assumption of a lower pole vessel. Despite hemodynamic stability a drop in hemoglobin occurred the following day. Angiography showed no perfusion of the left kidney. A left nephrectomy was performed without complications. The further course was uneventful. Mother and child survived. CONCLUSIONS: Rupture of aneurysms of the splenic and renal artery during later stages of pregnancy, during delivery or after childbirth are possible etiologies in cases of unexplained shock and abdominal pain. An emergency angiography in such cases helps to reveal the underlying cause and renders possible a targeted operative approach.

Chest reconstruction in asphyxiating thoracic dystrophy.

Asphyxiating thoracic dystrophy is a rare, complex malformation with a broad spectrum of clinical expression. Surgery is indicated only in severe cases in which failure to intervene will result in progressive pulmonary damage and eventual death. Conventional surgical techniques for expanding the thoracic cage diameter by sternotomy and the insertion of a metal prosthesis for anterior chest wall stability usually provide these patients with the time needed for thoracic cage growth. However, some of the most severe cases may require a two-stage approach. Hence, management should be directed toward resolving immediate ventilatory problems and minimizing secondary damage to the lungs caused by prolonged ventilatory support.

Lateral thoracic expansion for Jeune's asphyxiating dystrophy: a new approach.

A new procedure is described for thoracic expansion in Jeune's asphyxiating dystrophy. The chest wall is enlarged by division of ribs and underlying tissue in a staggered fashion so that either rib or periosteum covers the lung. New bone formation has been demonstrated so that a viable enlargement has been obtained. The clinical result is excellent to date.

[Uterine rupture without predisposing factors after a single vaginal PgE2 administration in prolonged pregnancy]. / Uterusruptur ohne prädisponierende Faktoren nach einmaliger vaginaler PgE2-Applikation bei Terminüberschreitung.

The case of a 38-year old 3/1 gravida with prolonged pregnancy is discussed. Labour was induced with a prostaglandin (PgE2-) vaginal tablet 4 days after an oxytocin stress test had failed. After rapid labour development, imminent fetal asphyxia suddenly occurred, leading to an emergency cesarean section. A rupture of the left uterus wall rupture with laceration of uterine vessels was demonstrated. This is the first case report of a uterus rupture that happened in prolonged pregnancy without predisposing risk factors after a single PgE2 dose that was correctly placed into the posterior fornix.

Early and delayed SPECT images of extracerebral fluid collection in infants using 123I-N-isopropyl-p-iodoamphetamine.

Cerebral blood flow in seven infants with extracerebral fluid collections was investigated using single photon emission computed tomography (SPECT) with 123I-N-isopropyl-p-iodoamphetamine. Early and delayed SPECT imaging was carried out. Areas of hypoperfusion were observed in five cases. The watershed zone of the major cerebral arteries or the territory of the anterior cerebral arteries were common areas of low perfusion. The hypoperfusion area was redistributed in two cases with intracranial hypertension. Subduroperitoneal shunts produced improvement of clinical symptoms in these cases. Hypoperfusion without redistribution was observed in three patients. In these areas, permanent tissue damage caused by a primary disease existed. Normal circulation patterns were observed in two patients. They showed normal development and follow-up CT revealed a decrease in the size of the extracerebral fluid collection. Measurement of regional cerebral blood flow may be helpful in considering surgical indications and in following up extracerebral fluid collection in infants.

[Partial tracheal rupture in a newborn infant. A rare traumatic birth complication or intubation sequela?]. / Tracheateilabriss bei einem Neugeborenen. Seltene geburtstraumatische Komplikation oder Intubationsfolge?

The neonate was born with the help of vacuum extraction because of difficult delivery of the shoulders. After birth respiratory distress developed and endotracheal intubation and artificial ventilation became necessary. When the tube was changed in the intensive care unit the infant's clinical status suddenly deteriorated. A rupture of the trachea was diagnosed immediately followed by surgery. The child survived without neurological sequelae. The chronological sequence of symptoms after birth suggests that the rupture of the trachea primarily developed during delivery and was secondarily enlarged by repeated endotracheal intubation.

[Cesarean section of the second twin. Is this unusual mode of delivery justifiable?]. / Sectio am II. Zwilling. Ist dieser ungewöhnliche Geburtsmodus vertretbar?

Within a ten-year period 1983-1992 eleven Caesarean sections were performed on the second twin after the first twin had been born vaginally. The reasons leading to this rather unusual mode of delivery are described here. Predominantly, multiple risk factors were present. One first twin and one second twin died in the early post natal period. In a comparative study of the literature, it is shown, that this, disputed mode of delivery has increased during the last twenty years and that, under special circumstances, it is a justifiable method of management of gemini deliveries.

[Emergency Cesarean section--basic data]. / Der Notfall-Kaiserschnitt-Basisdaten.

Performing a computerized multicenter-study of 66 clinics in the state of North-Rhine-Westfalia concerning the management of emergency caesarean-section (ECS), the resulting basic data were calculated as follows: In 207 cases of ECS studied, the time period needed from the decision for operation on to the beginning of surgery (DFOS) is on the average 20.4 +/- 12.3 minutes (Median 17 min., 90th Percentile 37 min.). The mere duration of surgery until delivery of the baby averaged to 4.4 +/- 2.1 min. (Median 4.0 min., 90th Percentile 7 min.). In an emergency case, on the average 24.8 +/- 12.9 minutes (Median 22 min., 90th Centile 43 min.) pass by from the decision for the operation until delivery. The median actual pH-value in umbilical artery blood of these neonates was 7.169 +/- 0.160 (N = 168). 25.0% of these neonates had pH-values below 7.1, 16.0% below 7.0 and 3.0% below 6.900. 20.1% of these babies had an Apgar-score of less than or equal to 3 after 1 min., 4 newborns received a score of 0, but recovered, whereas 4 other demised. The unpurified perinatal mortality amounted to 53.1/1000, the purified one to 48.3/1000. From these data we conclude for the time being, that 1. efforts have to be made to shorten the time factors (especially the first one (DFOS)), although there is still no evidence, that severe neonatal acidosis is significantly associated with undue long time factors in ECS; 2. further thorough analysis is need to evaluate the medical profile and the organizational background of these highest risk-deliveries.

[Delivery of quadruplets after in vitro fertilization: perinatologic and ethical aspects]. / Entbindung von Vierlingen nach In-vitro-Fertilisierung: Perinatologische und ethische Aspekte.

Progress in modern reproductive medicine has brought much success to infertile couples. A side effect of IVF procedure are multiple pregnancies with clinical, ethical, perinatal and social problems. We report about the first case of quadruplets after IVF born in Austria and try to illustrate the perinatal, social and ethical problems. The 34 year old woman was treated in our sterility outpatient clinic because of secondary sterility. In 1988 finally she underwent IVF procedure. Four oocytes were retrieved by means of vaginal follicle puncture. 4 embryos were transferred, and after 3 weeks 4 viable embryos were seen at an ultrasound investigation. Due to placental insufficiency a cesarean section had to be performed in the 31st week of gestation. The weights of the premature infants were 1280 g, 1340 g, 1260 g and 1250 g. Two years after birth two infants show neurologic defects and need permanent care.

[Deliveries in patients with more than 1 cesarean section]. / Entbindungen bei Patientinnen mit mehr als einem Kaiserschnitt.

The mode of delivery in 49 women with two or more previous cesarean sections was examined. In 33 cases (67%) a primary re-cesarean section was performed, in 12 of 16 patients (75%) a vaginal birth was possible. Emphasis was given to the causes leading to the latest cesarean section to the maternal and infant conditions and complications and the relevant factors from earlier cesarean sections. The results were discussed by comparison to related publications. Some fundamental considerations about the mode of delivery in cases of repeated cesarean sections were presented. The justifications for a repeated primary cesarean section based on the previous record to two or more cesarean sections alone seems to be no longer given.

[Clinical aspects and problems in thoracic dystrophy]. / Zur Klinik und Problematik der Thoraxdystrophie.

We report about a child with Jeune-syndrome in whom surgical enlargement of the thorax at the age of 4 months was performed. In the beginning there was a significant improvement of the respiratory situation. Due to resorption of the interposed ribs there was again a shrinking of the thorax and a deterioration in the child's condition. A reoperation at the age of 8 months couldn't improve the situation anymore, because at that time the child had already additional problems such as pulmonary hypertension and broncho-pulmonary dysplasia. We overview the literature and line out our opinion about the indication for surgical enlargement of the thorax in children with Jeune syndrome.

[Tracheal agenesis: which surgical measures are sensible?]. / Trachealagenesie: Welche chirurgischen Massnahmen sind sinnvoll?

Tracheal agenesis is a rare congenital malformation. Up to now no surgical concept exists for a definitive correction. In this paper we describe our experiences with an own case and discuss the purpose and justification of temporary surgical measures.

[Diagnosis and therapy of choanal atresia in the new born (author's transl)]. / Zur Diagnose und Behandlung der Choanalatresie beim Neugeborenen

Report on 8 cases of congenital atresia or severe stenosis of the choanae. In 2 cases early transnasal surgery followed by canulation was necessary. One child died of pleuropneumonia and sepsis following asphyxia on the 10th day, when mouth-breathing had already been established.