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.

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.

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.

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.

[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.

[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.

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.

[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.

[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.

[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.

[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.

[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.

[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.