The pharmacological treatment of newborn diaphragmatic hernia — update 1987

Before the mid 1970s, neonates with diaphragmatic hernias (CDH) had a survival rate of 40%. Since then increased interest has been focused on the pulmonary artery and its hypertension (the persistent fetal circulation) and the pharmacologic treatment of this pathophysiology. Our initial pharmacologic experience left us with much information and even more unanswered questions. While other series were encouraging, they also showed a survival rate of about 50%. The following problems are associated with pulmonary vasodilator therapy: (1) there is no specific pulmonary vasodilator and the response is often unpredictable; (2) the pulmonary arterioles in these babies are abnormal and appear in many instances anatomically incapable of responding; (3) cardiac output may be affected secondary to inotropic or chronotropic side effects of vasodilators; and (4) the effect of a vasodilator on the pulmonary vasculature under hypoxic conditions is unknown. Moreover, we have yet to identify at birth which CDH babies require this treatment, and we must find improved pharmacologic agents that will more specifically dilate the constricted pulmonary vascular bed with minimal effect on the systemic circulation. Although some attempts at pharmacological manipulation of the pulmonary vascular bed continue in most centers, including ours, there are many other innovations in the perioperative management which require further evaluation, among them extracorporeal membrane oxygenation, high-frequency oscillation and possibly lung transplantation in the future. Offprint requests to: S. H. Ein     

Hepatotórax crônico devido à ruptura do diafragma direito: um desafio anestésico em um caso raro

Background

Diaphragmatic rupture is an uncommon condition, with 90% of ruptures occurring on the left side. However, its incidence on the right side is increasing along with the increase in traffic accidents. Liver herniation may become progressive causing severe atelectasis of the right lung, resulting in impaired respiratory status and hemodynamic changes.

Morgagni hernia repair in children over two decades: Institutional experience,systematic review,and meta-analysis of 296 patients

Background/Purpose

Morgagni diaphragmatic hernia (MH) is rare. We report our experience based on routine patch use in MH repair to curb recurrence. A systematic review and meta-analysis were performed to study the recurrence and complications associated with minimally invasive surgery and the use of patch.

Non-operative management of right side thoracoabdominal penetrating injuries—The value of testing chest tube effluent for bile

Introduction

While mandatory surgery for all thoracoabdominal penetrating injuries is advocated by some, the high rate of unnecessary operations challenges this approach. However, the consequences of intrathoracic bile remains poorly investigated. We sought to evaluate the outcome of patients who underwent non-operative management of right side thoracoabdominal (RST) penetrating trauma, and the levels of bilirubin obtained from those patients’ chest tube effluent.

Patients and methods

We managed non-operatively all stable patients with a single RST penetrating injury. Chest tube effluent samples were obtained six times within (4-8 h; 12-16 h; 20-24 h; 28-32 h; 36-40 h; 48 h and 72 h) of admission for bilirubin measurement and blood for complete blood count, bilirubin, alanine (ALT) and aspartate aminotransferases (AST) assays. For comparison we studied patients with single left thoracic penetrating injury.

Results

Forty-two patients with RST injuries were included. All had liver and lung injuries confirmed by CT scans. Only one patient failed non-operative management. Chest tube bilirubin peaked at 48 h post-trauma (mean 3.3 ± 4.1 mg/dL) and was always higher than both serum bilirubin (p < 0.05) and chest tube effluent from control group (27 patients with left side thoracic trauma). Serum ALT and AST were higher in RST injury patients (p < 0.05). One RST injury patient died of line sepsis.

Conclusion

Non-operative management of RST penetrating trauma appears to be safe. Bile originating from the liver injury reaches the right thoracic cavity but does not reflect the severity of that injury. The highest concentration was found in the patient failing non-operative management. The presence of intrathoracic bile in selected patients who sustain RST penetrating trauma, with liver injury, does not preclude non-operative management. Our study suggests that monitoring chest tube effluent bilirubin may provide helpful information when managing a patient non-operatively.     

Strangulated diaphragmatic hernia presenting clinically as pericarditis

A case of strangulation of the transverse colon in a traumatic left diaphragmatic hernia manifesting as pericarditis is reported. This is unusual because pericardial signs in traumatic diaphragmatic hernia have been previously described in association with direct pericardial injury. This is the only such case where electrocardiographic changes of pericarditis were seen without direct pericardial trauma. The possibility of internal herniation through a traumatic diaphragmatic hernia must be considered in patients with chest symptoms and a compatible history.     

Holoprosencephaly in Mexico: first reported autopsy case by Dr. Agustín Arellano

INTRODUCTION: Holoprosencephaly with cyclocephaly is an early disturbance of organogenesis and has been classified as a severe brain malformation starting in 1755 by Eller in Germany, then in 1822 by Etienne Geoffroy de Saint-Hilaire in France, and finally in 1828 by Tiedemann in Germany. In 1839, Dr. Arellano published in Mexico a necropsy case of holoprosencephaly. This was the fourth publication worldwide on this kind of pathological alteration. Furthermore, in reference to diaphragmatic herniation, Arellano's paper is the fourth world report, having appeared 9 years before Bochdalek's publication. We have not found any other report that appeared before 1839 in the Americas on this particular malformation, and we consider that Arellano's paper was the first of its kind on the American continent. CONCLUSION: As is well known, the publications of this Mexican medical researcher were, for his time, at the level of those of the most developed countries. It is also important to know that the medical journal where Arellano's work was published, the "Periódico de la Academia de Medicina de Mégico(sic)," founded and directed by Dr. Manuel Carpio in 1836, is the direct forerunner of the present Gaceta Médica de México, the oldest currently published journal in the Americas.     

Diaphragmatic rupture: a frequently missed injury in blunt thoracoabdominal trauma patients

In the US and Western Europe, trauma is the fourth most common cause of death and the leading cause of death in the population less than 45 years of age [Mullinix and Foley, J Comput Assist Tomogr 28(Suppl 1):S20–S27, 2004]. Diaphragmatic injuries occur in 0.8 to 8% of patients after blunt trauma (Gray H, The muscles of the thorax. Anatomy of the human body. Lea & Febiger, Philadelphia, 1918) and may be a predictor of severity of injury in the blunt trauma patient [Worthy et al., Radiology 194(3):885–888, 1995]. The clinical diagnosis of diaphragmatic rupture (DR) is difficult and is missed in anywhere from 7 to 66% of patients [Cantwell, Radiology 238(2):752–753, 2006]. The accurate diagnosis and prognosis of this pathology depend on a complete knowledge of the clinical and radiological presentation. Computed tomography is the imaging modality of choice in the assessment of patients with clinical or radiographic findings suggestive of DR.     

Neonatal-onset nemaline myopathy mimicking congenital diaphragmatic hernia

In a newborn with severe respiratory failure and abnormal elevation of the right diaphragm, congenital diaphragmatic hernia with sac was diagnosed during surgery. However, microscopic examination of the sac showed atrophic striated muscle cells, indicating eventration instead of hernia. After several extubation failures, the final diagnosis of nemaline myopathy was made by skeletal muscle biopsy. In diaphragmatic defects with sac, diaphragm microscopic analysis should be recommended in order to discriminate between hernia and eventration. Congenital myopathies may underlie such diaphragmatic defects and should be promptly recognized, given their prognostic implications.     

Left congenital diaphragmatic hernia, absent pericardium, and liver heterotopia: a case report and review

A case of a left congenital diaphragmatic hernia (CDH), absent pericardium, and a hernial sac containing ectopic liver tissue is presented. This triplicate of defects is exceedingly rare with only 2 previous reports in the literature dating back to 1936. This case supports the evidence that CDH is the result of failure of the closure of the pleuroperitoneal canals by the growth of the posthepatic mesenchymal plate. Moreover, the role of the phrenic nerve as a mediator for both CDH and absent pericardium is discussed.