Diaphragmatic Eventration Research Articles

Thoracic versus abdominal approach to correct diaphragmatic eventration in children

BackgroundPlication of diaphragm (DP) for eventration (DE) can be done using thoracic or abdominal approaches. The purpose of our study was to compare outcomes between these approaches based on our experience and on systematic literature review. MethodsRetrospective records of children <16 years who underwent DP (single-center, 2004–2018) were recorded and analyzed. Systematic review and meta-analysis of related studies was undertaken. Data are reported as median (range). ResultsEighty-nine cases were identified in thoracic (Congenital = 5, Acquired = 84) and 13 (Congenital = 10, Acquired = 3) in abdominal group aged 5.88 (0.36–184.44) and 10.0 (0.12–181.8) months. Improvement in diaphragm level post-DP was significantly higher in abdominal [2(0–4)] than chest [1.5(0–5)] group (p = 0.04). On Cox regression analysis, there was a non-significant trend to a longer time to extubation in the chest group (Hazard ratio (HR) = 0.539[0.208–1.395], p = 0.203). Patients operated transthoracically left intensive care unit after a significantly longer time (HR = 0.339[0.119–0.966], p = 0.043). Patients operated transabdominally tended to be fed later, although this was not significant (HR = 1.801[0.762–4.253], p = 0.043). On Kaplan–Meier analysis, there was a non-significant trend to a lower rate of recurrence in the abdominal group (HR = 0.3196[0.061–1.675], p = 0.1876). In the meta-analysis including three published studies as well as our data (total n = 181, Thoracic = 139, Abdominal = 42), no difference was found in the incidence of recurrence amongst the 2 groups (RD = -0.04, 95%CI = -0.25, 0.18, p = 0.74). ConclusionThis is one of the largest reports on outcomes of children undergoing DP for DE. There is no significant difference in recurrence rate, even though all recurrences in our series (15.7%) were in the acquired cases operated using a thoracic approach. Type of StudyTreatment Retrospective Comparative Study. Level of EvidenceLevel III.

2763 A Case of Intermittent Organo-axial Gastric Volvulus

INTRODUCTION: Gastric volvulus is a rare clinical entity defined as twisting of the stomach along its long or short axis leading to gastric outlet obstruction (GOO). Commonly symptoms are acute and include retching, abdominal pain, and vomiting. Chronic intermittent manifestations of this entity constitute a diagnostic challenge as conclusive findings in imaging are only present during symptomatic periods and due to the intermittent nature of the disorder, a volvulus may spontaneously resolve before imaging studies are performed. Herein we illustrate a rare case of an intermittent organo-axial gastric volvulus that responded to conservative measures. CASE DESCRIPTION/METHODS: A 92-year-old female presented with intermittent epigastric pain and food intolerance of a few weeks duration. She reported dysphagia one year prior to presentation. Upper gastrointestinal (UGI) series showed gastric organo-axial rotation with no obstruction. Symptoms resolved at that time without any intervention. Physical examination revealed epigastric tenderness. Vitals and laboratory tests were unremarkable. UGI series showed a large para-esophageal hernia, an intrathoracic stomach with a gastric organo-axial rotation and partial obstruction at the level of gastroduodenal junction [Figures 1 and 2]. A contrast computed tomography (CT) scan of abdomen confirmed the same findings [Figure 3]. Endoscopic as well as surgical gastropexy were offered but the patient refused any intervention in view of her poor functional status. She was managed conservatively with intravenous fluids and bowel rest and was discharged 5 days later. DISCUSSION: Gastric volvulus was first described in 1866 as a torsion of the stomach around its short or long axis. Based on the axis of rotation, it was classified into organo-axial, mesentero-axial or combined. Organo-axial volvulus is common in adults, constitutes about 59% of cases and peaks after the 5th decade of life. It is mainly seen in the context of diaphragmatic abnormalities such as para-esophageal Hiatal hernias and diaphragmatic eventration. Diagnosis is challenging due to non-specific presenting symptoms. Contrast CT of abdomen and UGI series are gold standard diagnostic modalities. Endoscopic and surgical gastropexy is the mainstay of therapy. Heightened awareness is warranted in patients presenting with GOO in the setting of UGI tract anatomical abnormalities, since delayed diagnosis and management may lead to life-threatening complications and mortality rates up to 50%.

Novel features of PIK3CA-Related Overgrowth Spectrum: Lesson from an aborted fetus presenting a de novo constitutional PIK3CA mutation

PIK3CA-Related Overgrowth Spectrum (PROS) encompass a group of disorders which are mainly characterized by segmental overgrowth of several tissues as well as venous and lymphatic malformations. It is caused by heterozygous, usually somatic mosaic, pathogenic variants in the PIK3CA gene. However, some patients presenting mainly isolated megalencephaly or “Cowden-like” features have been described harboring constitutional mutations of PIK3CA.Here, we report the case of a woman whose pregnancy was interrupted at 34 weeks of gestation after the detection of the following ultrasound abnormalities: left diaphragmatic hernia with intrathoracic stomach, right deviation of heart, intrathoracic double bubble sign, macrocephaly and polyhydramnios. Fetal autopsy contributed to better characterize the phenotype, showing megalencephaly, left diaphragmatic eventration, facial dysmorphism (hypertelorism, abnormal hair line implantation) and duplication of distal portion of the small bowel. Clinical exome sequencing identified a de novo constitutional variant c.1030G>A p.(Val344Met) in PIK3CA. Although this mutation has been previously described (as constitutional variant) in pediatric patients, our case represents the first detailed description of the prenatal features found in association with a constitutional PIK3CA mutation. Moreover, this case contributes to delineate novel features (diaphragmatic eventration and duplication of the distal part of the small bowel) which could be identified in association with PROS.

Congenital diaphragmatic eventration with absent left phrenic nerve in the fetal pig.

We encountered a fetal pig with eventration of the diaphragm and pulmonary hypoplasia accompanied by phrenic nerve agenesis. The fetal pig was female measuring 34cm in crown-rump length and about 1500g in body weight. The diaphragm was a complete continuous sheet, but comprised a translucent membrane with residual muscular tissue only at the dorsolateral area of the right leaf of the diaphragm. The left leaf protruded extraordinarily toward the thoracic cavity. The left phrenic nerve was completely absent, while there was a slight remnant of the right phrenic nerve that supplied the dorsolateral muscular area of the right leaf. Both lungs were small, and the number of smaller bronchioles arising from the bronchioles was decreased to about half of that of the normal lung. Additionally, the right and left subclavius muscles and nerves could not be identified. These findings imply that the diaphragm, the subclavius muscle and nerves innervating them comprise a developmental module, which would secondarily affect lung development. It is considered that the present case is analogous to the animal model of congenital eventration of the diaphragm in humans.

Dynamic magnetic resonance imaging in unilateral diaphragm eventration: knowledge improvement before and after plication.

The assessment before surgical plication for unilateral hemidiaphragm (HD) eventration is not clearly defined and no precise criteria exist to really understand which patient is operated with which results depending on the technique used. The goal of this study was to evaluate the place of dynamic magnetic resonance imaging (dMRI) before and after plication by developing measurement criteria. Between 2006 and 2017, 18 patients (group1: Gp1) were operated for eventrations, 15 left-sided (Gp1L) and 3 right-sided (Gp1R). All had preoperative and postoperative evaluations including dMRI and pulmonary function tests. Five healthy volunteer subjects (group2: Gp2) had the same imaging protocol. For each HD, we measured the respiratory excursion at three fixed points (S1, S2, S3) and the height of curvature on sagittal plane. We also searched for upward paradoxical diaphragm movements. Before surgery, no excursion (n=13) or extremely reduced excursion (n=5) was detected on the injured HD (IHD) in Gp1. Upward paradoxical movements were identified only in Gp1L (n=6). Compared with Gp2 subjects, the healthy HD for Gp1L patients had significantly reduced excursion values at three sites S1 (P=0.038), S2 (P=0.006), and S3 (P=0.004). After plication, the decreasing height of curvature confirmed a tightening of the IHD in all patients (median value from 100 to 39.5 mm in Gp1L and 92 to 74 mm in Gp1R, P=0.0001). All upward paradoxical movements disappeared. Healthy HD excursions in Gp1L normalised their values. All those imaging improvements were correlated with postoperative improvements of dyspnoea score (P<0.0001) and vital capacity (P=0.002). dMRI and the standardised grid we developed not only improve the knowledge of unilateral diaphragm eventration but also permit to evaluate the quality of its surgical repair. It also demonstrates that a dysfunction of the healthy HD contralateral to eventration is possible and reversible after plication of the IHD.

DOZ047.89: Thoracoscopic (TS) management of early complications after esophageal atresia (EA) correction: single center experience

Abstract Background Early complications and their surgical management after esophageal atresia (EA) correction aren’t largely described in the literature. Anastomotic stricture and anastomotic leakage are the most commonly described. Those, along with iatrogenic chylothorax, are safely managed with a conservative approach. Surgery is indicated for nonresponding cases and often carries major invasiveness. This study reports thoracoscopy use for EA complications at our center in a 2-year period (2017–2018). Case Reports Case 1, birth weight (BW) 1550 gr, presented respiratory difficulties 6 days after open surgery: chest X-ray revealed right diaphragmatic eventration probably due to phrenic nerve damage; Case 2 (BW 3420 gr) presented anastomotic dehiscence with salivary traces in drainage tube, 9 days after thoracoscopic (TS) repair with azygos vein sparing; Case 3 (BW 2530 gr) developed right chylothorax 13 days after right thoracotomy following primary TS left approach for dextrocardia (5 days after oral feeding was started) resistant to Octreotide treatment. All cases were successfully treated with TS technique. Case 1 underwent TS right diaphragmatic plication after an initial conservative approach. In case 2 we performed TS anastomosis revision with bovine pericardium patch apposition. Case 3 underwent right TS cruentation and glueing of the right costophrenic recess (6 weeks after surgery) for chylothorax persistence despite 22 days of Octreotide therapy. No intra- or postoperative complication occurred. A TS approach for EA repair is replacing open surgery, which is nowadays limited to selected neonates. The open technique seems to increase the risk of postoperative early complications, probably because thoracoscopy allows optimal anatomy visualization and minimal tissue handling. It is also postulated that TS azygos sparing may be a preventive factor against anastomotic leakage, even though it can be technically demanding. Furthermore, thoracoscopy can reduce invasiveness in the case of a diagnostic maneuver such as left anatomy exploration. Conclusions Early surgical complications after EA repair are rare, and can be safely treated with a TS approach, regardless of the primary surgical technique. If properly managed, they have good prognosis. Benefits of TS EA repair are widely known, and early reintervention for complications with this technique seems to be safe and justified in centers where neonatal MIS is the –first-choice approach.

A strategy for pulmonary resection after contralateral diaphragm plication: a surgical case report

BackgroundPulmonary carcinoma patients with low pulmonary function cannot be treated surgically because of the high risk of complications. Diaphragmatic eventration is a disease characterized by diaphragmatic paralysis and dyspnea. Here, we report a surgical case of multiple pulmonary carcinomas with contralateral diaphragmatic eventration.Case presentationThe patient was a 75-year-old woman with multiple metachronous right lung carcinomas complicated by left diaphragmatic eventration. When she was 70 years old, a right upper lobectomy and right S6b wedge resection were performed for double lung carcinomas. Five years later, two new lung tumors in her right lower lobe and left diaphragmatic eventration were identified, but resection was thought to be impossible because of her low pulmonary function. We performed video-assisted thoracoscopic surgery (VATS) plication with carbon dioxide (CO2) insufflation for the left diaphragmatic eventration, and her pulmonary function improved. Subsequently, we performed a right S6 wedge resection and right S9 segmentectomy for the double lung tumors with no complications. The tumors were diagnosed as double primary carcinomas.ConclusionsOur case presented with low pulmonary function and right multiple lung carcinomas with left diaphragmatic eventration. VATS plication for the left diaphragmatic eventration achieved improvement in her pulmonary function, and right pulmonary resection for the lung carcinomas was performed. VATS plication can expand the choice of treatments in such cases.

Coexistence of diaphragm eventration and multiple organ anomalies diagnosed after sigmoid volvulus

Doğumsal diyafram evantrasyonu ve otozomal dominant polikistik böbrek diğer organ ve sistem tutulumlarının da eşlik edebildiği hastalıklardır. Daha önceden bilinen herhangi bir hastalığı, geçirilmiş travma ya da cerrahisi olmayan 84 yaşında erkek hastanın 2 gündür solunum sıkıntısı, gaz gaita yapamama, karın ağrısı, karında şişlik, bulantı-kusma şikayetleri varmış. Son birkaç saat içinde karın ağrısında şiddetlenme ve ani solunum yetmezliği gelişmesi üzerine başvurdu. Bilgisayarlı tomografide sigmoid volvulus, içi boş organ perforasyonu, dekstrokardi, diyafram evantrasyonu, karaciğer ve böbreklerde multipl kistler saptandı. Hastaya sigmoid rezeksiyon ve kolostomi açılması işlemi uygulandı. Ameliyat sonrası erken dönemde genel durumu düzelen hasta 4. günde sorunsuz şekilde taburcu edildi. Birden fazla sistemi ya da organı tutan patolojiler hastanın kliniğinde beklenenden daha hızlı ve gürültülü bozulmaya sebep olabilmektedir. Böyle hastaların bir bütün olarak değerlendirilmesi tedavinin daha doğru planlanmasını, böylece hastada morbidite ve mortaliteyi artırabilecek gereksiz işlemlerin önüne geçilmesini sağlayacaktır.

Congenital Cystic Diaphragm with Diaphragmatic Eventration in a Fetus: A Case Presentation

Introduction: Congenital diaphragmatic eventration (CDE) is defined as the abnormal elevation of the diaphragm, due to incomplete muscularization of the diaphragm with a thin membranous sheet replacing normal diaphragmatic muscle. Case report: We report a prenatal case with a diaphragmatic mesothelial cyst combined with CDE. Conclusion: A large cystic mass between the thoracic wall and the liver in early pregnancy is highly suggestive of cystic diaphragm.

Thoracoscopic Diaphragm Plication with Pledgets in an 8 kg Infant After Congenital Heart Surgery

Abstract Introduction: Diaphragm eventration from a phrenic nerve injury after congenital heart surgery is a well-recognized complication. Treatment of the eventration is diaphragm plication if the...

Azygos lobe and partial eventration of diaphragm

An azygos lobe is an accessory lobe of the lung that may occasionally be confused with a pathological process such as a bulla, lung abscess, or neoplasm. Eventration of the diaphragm is generally regarded as a condition in which the left or the right leaf of the diaphragm has ascended abnormally high into the chest. In this case, both eventration of diaphragm and azygos lobe presented in the same patient on the same side.

Ultrasound-guided perioperative management of 28-month-old patient with congenital diaphragmatic eventration

Diaphragmatic eventration is a rare anomaly. When patients with this condition undergo general anesthesia, anesthetic management should be performed with particular care owing to the risk of diaphragmatic rupture. Such a rupture can be perioperatively diagnosed using multiple tools including lung ultrasonography. This case report describes the anesthetic management of a male infant with osteochondroma in the distal ulna, presenting with diaphragmatic eventration on the right side.

Eventration of Diaphragm with Gastric Volvulus: Lesson Learned.

Eventration of Diaphragm with Gastric Volvulus: Lesson Learned.

An undiagnosed eventration of diaphragm in a pregnant lady–out of the blue problem

We report a rare case of 34-year-old female with left diaphragmatic eventration presenting during adulthood. The primigravida patient came for emergency cesarean section who presented with breathlessness during perioperative period. Patient had no previous history of similar complaints, or any history of trauma. Physical examination showed normal findings in the patient during preanesthesia checkup. With best of our knowledge this is the first in pregnant patient in Maharashtra in whom this problem was identified.

A Case Report of Diaphragmatic Incisional Hernia Developed 18 Years after Plication of Diaphragmatic Eventration in Infancy

A Case Report of Diaphragmatic Incisional Hernia Developed 18 Years after Plication of Diaphragmatic Eventration in Infancy

Laparoscopic Plication for Left Anterior Diaphragmatic Eventration in a Pediatric Patient: A Case Report

Reported here is the first documented laparoscopic repair of a rare partial diaphragmatic eventration in a pediatric patient. The case involves a three-year-old female who had recurrent cough. While a Morgagni hernia was the initial impression, an eventration of the left anterior diaphragm was instead found on laparoscopy. Repair was aided by using transcutaneous traction, with plication achieved by intracorporeal sutures. The patient recovered uneventfully and follow-up x-ray after six months demonstrated an intact repair. Analogous diaphragmatic anomalies may have divergent clinical manifestations. Bochdalek hernias, while often associated with severe respiratory distress in affected neonates, may also not manifest overtly in other patients. Likewise, different anomalies may have similar clinical presentations. Patients with Morgagni hernias or eventrations, while often asymptomatic, can also present with severe pulmonary and gastrointestinal impairments. The diagnostic uncertainty is highlighted in the present case, wherein a child preoperatively diagnosed to have a Morgagni hernia was found intra-operatively to have a rare left anterior diaphragmatic eventration. 5 The laparoscopic approach adopted not only facilitated diagnosis, but was adaptable enough to enable intracorporeal repair for an altered indication. Key words: diaphragmatic eventration, diaphragmatic hernia, laparoscopy

Abstract 319: Return of Spontaneous Circulation is Not Affected by Gastric Distension

Introduction: Gastric distension is a common complication after CPR and is caused by excess gastric inflation. Consequently, regurgitation of gastric contents and diaphragmatic eventration with increased intrathoracic pressure can occur, which may lead to anoxia and ventricular diastolic dysfunction. We measured gastric volume of out-of-hospital cardiac arrest (OHCA) patients using computed tomography (CT) scan images, and examined the effect of gastric distension on the return of spontaneous circulation (ROSC). Hypothesis: Gastric distension from over-inflation is associated with decreased incidence of ROSC. Methods: We conducted a retrospective observational study with OHCA patients transported to Tsuyama Chuo Hospital from 2014 - 2017. We included cases with total body CT scan examined after ROSC or death. We excluded inter-facility transport and insertion of gastric tube before CT. We measured the total gastric volume using 3D imaging software (FUJIN Anatomia, AZE, Tokyo). Primary outcome: Comparison of gastric volume between patients with and without ROSC. Secondary outcome: Factors associated with increased gastric volume. Mann Whitney U-test was used for the primary outcome. Multivariable regression analysis was used for the secondary outcome. Results: 446 cases were included in the study. Median age was 79 [IQR: 68 - 86], 55% was male, 4% with initial rhythm VF/VT, 45% was estimated cardiac origin, 37% was witnessed arrest, 27% had ROSC. The total gastric volume was 400ml [192 - 793]. There was no difference in gastric volume between ROSC group and no-ROSC group (418 [232 - 733] vs 389 [183 - 824], p = 0.46). Airway management maneuver on arrival did not affect gastric volume (bag-valve-mask β: -99; 95% CI: -250 - 52, laryngeal tube β: -85; 95% CI: -228 - 59, endotracheal intubation: reference). Longer transport time was associated with increased the gastric volume (β: 5.6; 95% CI: 1.0 - 10) as was a non-cardiac origin of arrest (β: 121; 95% CI: 34 - 208). Conclusion: Abdominal distention was not associated with ROSC. Type of initial airway management did not significantly affect gastric volume. Factors associated with increased gastric volume at emergency department arrival were longer transportation time and non-cardiac origin of arrest.

A Rare Case of Partial Diaphragmatic Eventration with Hepatic Herniation

A 43 year old Female with past history of hypertension, diabetes mellitus presented to the Emergency room with a chief complaint of left flank pain. Her physical examination was notable for left sided flank tenderness with no abdominal rigidity or guarding. Routine blood work showed normal complete blood count, with normal comprehensive metabolic profile. A chest xray showed a soft tissue mass at the right lung base. Further contrast Computed Tomography(CT) of the chest and abdomen showed an incidental soft tissue mass at right lung base that measured 4.4 cm in diameter representing a focal eventration of the right hemi-diaphragm with liver tissue extending through the eventration.(Fig 1,2).2343_A Figure 1. Arrow depicting hepatic herniation2343_B Figure 2. Coronal CT showing hepatic mass extending through diaphragamDiaphragmatic eventration was first recognized by Jean Louis Petit in 1774 and the term was coined by Beclard in 1916. It is defined anatomically by thinning of the diaphragmatic tissue, characterized by the cephalic displacement of all or a part of an intact diaphragm. While complete eventration in more common on left, partial eventration is seen majorly on the right side. Congenital diaphragmatic eventration has been frequently cited as common in infancy, requiring urgent care and observation but it is rarely seen in adults. Typically this entity is frequently asymptomatic but can rarely manifest as progressive dyspnea, chest pain, abdominal discomfort, nausea, vomiting, heartburn and recurrent pneumonia. Partial eventration are usually detected incidentally on chest x-ray as a smooth homogeneous mass in continuation with the diaphragm.Ultrasound may reveal more anatomical detail about the diaphragmatic integrity, specifically eventration content or other diaphragmatic pathologies.If a contrast CT scan is performed, hepatic or omental vascular enhancements are seen as hyper-dense areas. Other modalities such as fluoroscopy with sniff testing and Magnetic resonance imaging are done if the diagnosis is still in doubt. Once diagnosed asymptomatic patients are usually managed conservatively and diaphragmatic plication is done in symptomatic patient. Our patient's left sided pain was musculoskeletal and not related to the diaphragmatic eventration. Her pain resolved with analgesics and she has remained asymptomatic on follow up visits. In summary we present a rare case of partial diaphragmatic eventration with hepatic herniation. Our case highlights conservative management in asymptomatic patients with diaphragmatic eventration.

An Unusual Presentation of an Adult with Diffuse Abdominal Pain Related to Congenital Diaphragmatic Hepatic Eventration

This case report identifies a uncommon presentation of a congenital diaphragmatic hepatic eventration in a middle aged female adult with an acute onset of diffuse abdominal pain. This case highlights a significant congenital event which is not commonly found as an adult. However, this case is interesting, as many clinicians may be puzzled over the radiologic presence, leading to numerable diagnostic studies and unnecessary consultations. A 43 year old Hispanic female with history of hypertension, diabetes and dyslipidemia presented to ER with complaint of diffuse abdominal pain, all quadrants of abdomen with no guarding or rigidity, normal bowel sounds. Routine labs showed no leukocytosis, normal metabolic panel except an elevated Cr of 1.7. CT Abdomen was done without any contrast in light of acute kidney injury, which showed a soft tissue mass at right lung base measuring 4.4 cm in diameter. Thought to be part of the hepatic dome pushing the diaphragm to the lung base versus, albeit less likely, a pulmonary mass, a CT with contrast was recommended. The patient was given symptomatic treatment in ER and was discharged with outpatient follow up. After her kidney function returned back to baseline CT abdomen with contrast showed focal eventration of the right hemidiaphragm with liver tissue extending through the eventration and no other suspicious mass. The patient was seen in GI clinic with impression of likely congenital anomaly. The patient was completely asymptomatic at that time and was conservatively managed with regular follow up. Congenital diaphragmatic eventration has been frequently cited in literature as a common condition in infancy, requiring urgent care and observation. However is a rare anomaly found in an adult. Eventation of the diaphragm is defined anatomically by thinning of the diaphragmatic tissue, characterized by the cephalic displacement an intact diaphragm. This entity is frequently asymptomatic and initially detected by chest x-ray. Adults can occasionally complain of progressive dyspnea, chest pain, abdominal discomfort, nausea/vomiting, heartburn. A differential must still be considered, including; diaphragmatic hernia, paraesophageal hernia, hiatus hernia, bronchogenic cyst, teratoma, cardiac sarcoma, lymphosarcoma, schwannoma, or metastatic tumor. Asymptomatic patients are usually managed with surveillance, and if persistently symptomatic managed with plication of the diaphragm with thoracotomy.2340_A Figure 1. Chest CT with IV Contrast- Sagittal view with hepatic eventration extending into thoracic cavity.2340_B Figure 2. Chest CT with IV Contrast- Coronal view with hepatic eventration extending into thoracic cavity.

Причины рецидива релаксации диафрагмы после кардиохирургических операций у детей

Причины рецидива релаксации диафрагмы после кардиохирургических операций у детей