Bilateral congenital eventration of diaphragm: keep in mind, the other side

Abstract

Eventration of diaphragm is an uncommon disorder in which diaphragmatic muscle is replaced by fibroelastic tissue, either partially or completely. Bilateral eventration is even rarer. We present a case of bilateral eventration of diaphragm in newborn with a fibroelastic sac on left side and diaphragmatic eventration with good muscular lips on right side. The right-sided diaphragmatic eventration was not evident initially, but manifested after surgical repair of the left-sided eventration.

Congenital Diaphragmatic Eventration in the Neonatal Period: Systematic Review of the Literature and Report of a Rare Case Presenting with Gastrointestinal Disorders

“…CDE as an anomaly of the diaphragm was initially described by Jean Louis Petit in 1774 as “a special form of thoracic hernia” during a postmortem examination, but the term diaphragmatic eventration appeared in the literature for the first time by Beclard and Cruveilhier in 1829 and became generally accepted instead of definitions such as diaphragmatic relaxation, phrenic insufficiency, and hypertrophied diaphragm [ 11 , 15 , 16 , 17 ]. In 1947, Bisgard reported the difference between CDH and CDE, and according to this report, CDE is “an abnormally high, or elevated position of one leaf of the diaphragm as a result of paralysis, aplasia, or atrophy of varying degree of the muscle fibers.…”

“…Embryologically, the diaphragm is derived from muscular components and three more elements: the septum transversum, the pleuroperitoneal membranes, and the mesentery of the esophagus. Eventration of the diaphragm can be congenital or acquired [ 11 , 15 ]. CDE is present at birth as the result of inadequate muscularization of the fused pleuropericardial membrane that builds the diaphragm [ 22 ].…”

“…Eventration of the diaphragm may occur as a secondary diaphragmatic paralysis (absence or injury of the phrenic nerve) due to birth trauma, congenital infection (polio, cytomegalovirus, and rubella), tumor infiltration of the phrenic nerve, and operative injury during cervical spine surgery. In infants, the most common cause of acquired eventration is birth trauma, attributed in most cases [ 15 , 27 , 29 , 30 ] to difficult vaginal delivery with shoulder dystocia that may lead to a stretch injury of the C3–C5 roots and affect the origin of the phrenic nerve and brachial plexus (Erb–Duchenne or Klumpke’s palsy). Clavicle fractures may be present at the same hemithorax with the eventration of the hemidiaphragm.…”

“…Clinical presentation of eventration of the diaphragm varies in severity, and the patients often appear to be asymptomatic. In most of the reported cases in the literature, clinical presentation occurred mainly with respiratory distress (tachypnoea, dyspnea, decreased breath sounds, paradoxical movement of the chest), cyanosis, cardiac symptoms, and gastrointestinal symptoms such as vomiting, anorexia, nausea, bowel sounds into the thoracic cavity, scaphoid abdomen, and abdominal pain [ 6 , 11 , 13 , 15 ]. During the neonatal period, respiratory symptoms dominate, and this finding is in line with our review results.…”

“…Nevertheless, the differential diagnosis between CDH and CDE remains difficult since radiographic findings, such as mediastinal shift, the appearance of stomach or bowel in the thorax with no bowel gas in the abdomen, are the same for both entities in most cases. Ultrasound can be used as a diagnostic method by demonstrating minimal or paradoxical movement of the diaphragm [ 11 , 15 , 33 ]. Finally, in most cases, the diagnosis is confirmed intraoperatively, as it happened in our case; the diaphragm was found to be not well developed and atrophic, although intact.…”