Thoracoabdominal compartment syndrome complicating right-sided diaphragmatic eventration with co-existent unilateral renal agenesis

Diaphragmatic eventration (DE) associated with intestinal malrotation and renal agenesis is a rare entity. The authors report a case of a 69-year-old man who had symptoms of heart failure. He had a previous imaging diagnosis of right diaphragmatic eventration and dilated cardiomyopathy. He died on the second day after the hospital admission and had a post mortem examination that confirmed complete right diaphragmatic eventration, intestinal malrotation, left renal agenesis, dilated cardiomyopathy, and anteriorly rotated right kidney and had findings suggestive of a thoracoabdominal compartment syndrome. Thoracoabdominal compartment syndrome is described as transmission of abdominal pressure through a defective diaphragm causing compression of the hemithorax viscera and mediastinal shift with a hemodynamic alteration. The association of these anomalies is rare, and the possibility of this finding in a patient with eventration should always be considered.

Alternativas quirúrgicas en hernias y eventraciones diafragmáticas crónicas / Surgical alternatives for chronic diaphragmatic hernias and eventrations

Introducción: las eventraciones y las hernias diafragmáticas crónicas son dos afecciones que en ocasiones se confunden; tienen una frecuencia relativamente baja y su estrategia quirúrgica varía. Objetivo: mostrar la experiencia del Hospital Universitario Clínico Quirúrgico Comandante Manuel Fajardo en la atención de enfermos con hernias y eventraciones diafragmáticas. Métodos: se presentan 11 pacientes atendidos desde 1998 hasta 2015 por afecciones del diafragma como eventraciones y hernias diafragmáticas crónicas. Del total, 7 fueron operadas con anillos de hasta 10 cm y dos con más de 10 cm. Resultados: se operaron 9 pacientes con hernias diafragmáticas y 2 con eventraciones. Ocho hernias fueron del lado izquierdo y una derecha. En 8 de las hernias el contenido era multivisceral. La hernia derecha tenía un anillo de más de 10 cm y con el hígado en su contenido. Cinco fueron reparadas mediante superposición de colgajos y en cuatro se necesitó una malla protésica. Las eventraciones se presentaron en dos mujeres, ambas parciales, una derecha y otra izquierda, tratadas con plicatura del hemidiafragma en dos planos. Conclusiones: las hernias y las eventraciones diafragmáticas son entidades parecidas con particularidades y terapéutica diferentes. En las eventraciones, la plicatura del diafragma es la técnica de elección, por vía abierta o de preferencia por toracoscopia. En las hernias diafragmáticas, la reparación depende del tamaño del anillo, hasta 10 cm se prefiere la reparación con superposición de colgajos y en defectos mayores el uso de mallas protésicas, preferentemente por una toracotomía(AU)

Introduction: Chronic diaphragmatic hernias and eventrations are two conditions sometimes mistaken for each other. They have a relatively low frequency and their surgical strategy varies. Objective: To show the experience at Comandante Manuel Fajardo Clinical Surgical University Hospital in the caring for patients with diaphragmatic eventrations and hernias. Methods: 11 patients attended from 1998 to 2015 were presented for diaphragmatic conditions, such as hernias and chronic diaphragmatic eventrations. Of the total, 7 were operated with rings of up to 10 cm. Two were operated for rings of more than 10 cm. Results: 9 patients with diaphragmatic hernias and 2 with incisional hernias were operated. Eight hernias were on the left side. One hernia was on the right side. In 8 of the hernias, the content was multivisceral. The right hernia had a ring of more than 10 cm, and had the liver in its content. Five hernias were repaired by flaps. Four hernias required a prosthetic mesh. The eventrations were presented in two women, both partial: one was on the right and the other was on the left, and both were treated with plication of the hemidiaphragm in two planes. Conclusions: Diaphragmatic hernias and eventrations are similar entities with different characteristics and therapeutics. In eventrations, the plication of the diaphragm is the technique of choice, either openly or preferably by thoracoscopy. In diaphragmatic hernias, repair depends on the size of the ring, up to 10 cm, flap repair is preferred, and in larger defects, it is the use of prosthetic meshes, preferably by thoracotomy(AU)

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.

Four-Dimensional CT of the Diaphragm in Children: Initial Experience

OBJECTIVE: To evaluate the technical feasibility of four-dimensional (4D) CT for the functional evaluation of the pediatric diaphragm. MATERIALS AND METHODS: In 22 consecutive children (median age 3.5 months, age range 3 days–3 years), 4D CT was performed to assess diaphragm motion. Diaphragm abnormalities were qualitatively evaluated and diaphragm motion was quantitatively measured on 4D CT. Lung density changes between peak inspiration and expiration were measured in the basal lung parenchyma. The diaphragm motions and lung density changes measured on 4D CT were compared between various diaphragm conditions. In 11 of the 22 children, chest sonography was available for comparison. RESULTS: Four-dimensional CT demonstrated normal diaphragm (n = 8), paralysis (n = 10), eventration (n = 3), and diffusely decreased motion (n = 1). Chest sonography demonstrated normal diaphragm (n = 2), paralysis (n = 6), eventration (n = 2), and right pleural effusion (n = 1). The sonographic findings were concordant with the 4D CT findings in 90.9% (10/11) of the patients. In diaphragm paralysis, the affected diaphragm motion was significantly decreased compared with the contralateral normal diaphragm motion (−1.1 ± 2.2 mm vs. 7.6 ± 3.8 mm, p = 0.005). The normal diaphragms showed significantly greater motion than the paralyzed diaphragms (4.5 ± 2.1 mm vs. −1.1 ± 2.2 mm, p < 0.0001), while the normal diaphragm motion was significantly smaller than the motion of the contralateral normal diaphragm in paralysis (4.5 ± 2.1 mm vs. 7.6 ± 3.8 mm, p = 0.01). Basal lung density change of the affected side was significantly smaller than that of the contralateral side in diaphragm paralysis (89 ± 73 Hounsfield units [HU] vs. 180 ± 71 HU, p = 0.03), while no significant differences were found between the normal diaphragms and the paralyzed diaphragms (136 ± 66 HU vs. 89 ± 73 HU, p = 0.1) or between the normal diaphragms and the contralateral normal diaphragms in paralysis (136 ± 66 HU vs. 180 ± 71 HU, p = 0.1). CONCLUSION: The functional evaluation of the pediatric diaphragm is feasible with 4D CT in select children.

Reparación protésica en una paciente con hernia incisional catastrófica / Prosthetic repair in a patient with catastrophic incisional hernia

Se presenta el caso clínico de una paciente de 66 años de edad, blanca, con antecedentes de hipertensión arterial, diabetes mellitus de tipo II y sobrepeso, quien fue operada en el Hospital General Universitario Dr Juan Bruno Zayas Alfonso de Santiago de Cuba por presentar una eventración gigante en la pared abdominal anterior, que no le permitía deambular sin apoyo. Se realizó la reparación protésica que resultó eficaz y segura, con 13 años sin recurrencia

The case report of a 66 years white patient with a history of hypertension, type II diabetes mellitus and overweight is presented, who was operated on at Dr Juan Bruno Zayas Alfonso University General Hospital in Santiago de Cuba due to a considerable eventration in the front abdominal wall that didn't allow her wandering around without support. The prosthetic repair which was effective and safe, was carried out with no recurrence during 13 years

Early Experience of Pediatric Thoracoscopic Surgery Performed by a Pediatric Surgeon / 소아외과

PURPOSE: Minimally invasive surgery (MIS) in abdomen and thorax has been widely accepted for pediatric diseases. Thoracoscopic surgery has the advantage of less pain, better cosmetic outcomes and less musculoskeletal sequelae in comparison to open surgery. We would like to share our initial experience with thoracoscopic surgery performed by one pediatric surgeon. METHODS: We performed a retrospective review of patients who underwent thoracoscopic surgery by one pediatric surgeon between April 2010 and August 2017 in Department of Pediatric Surgery, Seoul National University Children's Hospital. RESULTS: There were totally 18 cases; 8 cases for esophageal atresia, 3 cases for congenital diaphragm hernia, 2 cases for diaphragm eventration, 2 cases for esophageal duplication cyst, 2 cases for pleural mass and 1 case for esophageal bronchus. At the operation, median age was 9.5 months (range, 0-259 months) and median body weight was 9.4 kg (range, 1.9-49.4 kg). Median operative time was 157.5 minutes (range, 45-335 minutes). There was no case of open conversion and 2 cases of minor leakage at anastomosis site in case of esophageal atresia. Median follow-up month was 5 months (range, 0-87 months). During follow-up, 4 cases of esophageal atresia showed anastomosis site narrowing and average 2.5 times (range, 1-5 times) of esophageal balloon dilatation was done. CONCLUSION: We performed thoracoscopic surgery in case of esophageal, diaphragm disease and pleural mass. Thoracoscopic surgery can be an effective and feasible option of treatment for well-selected pediatric patients of intra-thoracic disease including esophagus, diaphragm and mediastinum disease.

Atypical Neonatal Marfan Syndrome with p.Glu1073Lys Mutation of FBN1: the First Case in Korea

Neonatal Marfan syndrome (nMFS) is considered to be on the most severe end of the spectrum of type I fibrillinopathies. The common features of nMFS include ascending aortic dilatation, severe mitral and/or tricuspid valve insufficiency, ectopia lentis, arachnodactyly, joint contractures, crumpled ear, loose skin, and pulmonary emphysema.We describe a newborn male diagnosed with nMFS. He presented several atypical features, such as diaphragmatic eventration, severe hydronephrosis with hydroureter, and dilated cisterna magna. Molecular analysis revealed a missense mutation at nucleotide 3217 (c.3217G>A) in exon 26 of the fibrillin-1 (FBN1) gene, resulting in the substitution of a glutamate for a lysine at codon 1073 (E1073K) in the 12th calcium binding epidermal growth factor-like domain of the FBN1 protein. Here we report a rare case of Nmfs with several combined atypical features, such as diaphragmatic eventration, severe hydronephrosis with hydroureter, and dilated cisterna magna. Our report is the first atypical nMFS case with p.Glu1073Lys mutation of FBN1 in Korea and may help clinicians with the diagnosis and follow-up of atypical nMFS.

Is polysomnographic examination necessary for subjects with diaphragm pathologies?

OBJECTIVES: While respiratory distress is accepted as the only indication for diaphragmatic plication surgery, sleep disorders have been underestimated. In this study, we aimed to detect the sleep disorders that accompany diaphragm pathologies. Specifically, the association of obstructive sleep apnea syndrome with diaphragm eventration and diaphragm paralysis was evaluated. METHODS: This study was performed in Süreyyapasa Chest Diseases and Thoracic Surgery Training and Research Hospital between 2014-2016. All patients had symptoms of obstructive sleep apnea (snoring and/or cessation of breath during sleep and/or daytime sleepiness) and underwent diaphragmatic plication via video-assisted mini-thoracotomy. Additionally, all patients underwent pre- and postoperative full-night polysomnography. Pre- and postoperative clinical findings, polysomnography results, Epworth sleepiness scale scores and pulmonary function test results were compared. RESULTS: Twelve patients (7 males) with a mean age of 48 (range, 27-60) years and a mean body mass index of 25 (range, 20-30) kg/m2 were included in the study. Preoperative polysomnography showed obstructive sleep apnea syndrome in 9 of the 12 patients (75%), while 3 of the patients (25%) were regarded as normal. Postoperatively, patient complaints, apnea hypopnea indices, Epworth sleepiness scale scores and pulmonary function test results all demonstrated remarkable improvement. CONCLUSION: All patients suffering from diaphragm pathologies with symptoms should undergo polysomnography, and patients diagnosed with obstructive sleep apnea syndrome should be operated on. In this way, long-term comorbidities of sleep disorders may be prevented.

Abordaje fisioterapéutico en paciente con eventración compleja / Physiotherapeutic approach in patient with complex eventration

Los pacientes con hernias complejas, se caracterizan por presentar cambios sistémicos y locales. Las complicaciones respiratorias pueden ser mínimas o podrían ser evitadas con la utilización de un protocolo de atención de fisioterapia respiratoria. Se presenta el caso clínico de una paciente de 53 años con diagnóstico de eventración compleja, Obesidad Mórbida y Diabetes Mellitus tipo II. Recibe el tratamiento de fisioterapia respiratoria en las etapas pre y post operatorias, mediante la evaluación clínica kinésica se pudo comprobar la pronta mejoría, pudiendo lograr su máximo grado de dependencia funcional. Resulta interesante medir dicho avance, dado que existen escasas publicaciones sobre este tema.

Patients with complex hernias, are characterized by systemic and local changes. Respiratory complications can be minimal or could be avoided with the use of a protocol of physiotherapy care. Presented the clinical case of a 53 year old patient with diagnosis of complex eventration, morbid obesity and Diabetes Mellitus type II. Receiving physiotherapy treatment in stages pre and post operative, through evaluation clinical kinaesthetic failed to check the prompt improvement, and can achieve its maximum degree of functional dependence. It is interesting to measure such progress, given that there are little publishing on this topic.

Eventración diafragmática en pediatría: revisión de un caso y revisión de la literatura / Diaphagmatic eventration in children: a case report and review of the literature

Diaphragmatic eventration is an abnormal elevation of one of the hemidiaphragms, with deviation of the mediastinum to the contralateral side. It is usually asymptomatic. It can be either congenital or acquired. If it is asymptomatic, the patient should be kept under observation. The resolution must be surgical if the ascent of the diaphragm is pointed, if there is any symptom or recurrent pneumonia. This article describes the case of a patient with congenital diaphragmatic eventration. The difficulties the patient presented in the diagnosis are reviewed.

La eventración diafragmática es la elevación anormal de uno de los hemidiafragmas, con desviación del mediastino hacia el lado contralateral, generalmente asintomática. Se clasifica en congénita y adquirida, si es asintomática el paciente se debe mantener en observación, la resolución debe ser quirúrgica si el ascenso diafragmático es acentuado, presenta síntomas o neumonía recurrente. En este artículo se revisa el caso de un paciente con eventración diafragmática congénita, que presentó dificultades en el diagnóstico.

Surgical Outcomes of Pneumatic Compression Using Carbon Dioxide Gas in Thoracoscopic Diaphragmatic Plication

BACKGROUND: Surgical correction needs to be considered when diaphragm eventration leads to impaired ventilation and respiratory muscle fatigue. Plication to sufficiently tense the diaphragm by VATS is not as easy to achieve as plication by open surgery. We used pneumatic compression with carbon dioxide (CO2) gas in thoracoscopic diaphragmatic plication and evaluated feasibility and efficacy. METHODS: Eleven patients underwent thoracoscopic diaphragmatic plication between January 2008 and December 2013 in Pusan National University Hospital. Medical records were retrospectively reviewed, and compared between the group using CO₂ gas and group without using CO2 gas, for operative time, plication technique, duration of hospital stay, postoperative chest tube drainage, pulmonary spirometry, dyspnea score pre- and postoperation, and postoperative recurrence. RESULTS: The improvement of forced expiratory volume at 1 second in the group using CO₂ gas and the group not using CO₂ gas was 22.46±11.27 and 21.08±5.39 (p=0.84). The improvement of forced vital capacity 3 months after surgery was 16.74±10.18 (with CO₂) and 15.6±0.89 (without CO₂) (p=0.03). During follow-up (17±17 months), there was no dehiscence in plication site and relapse. No complications or hospital mortalities occurred. CONCLUSION: Thoracoscopic plication under single lung ventilation using CO₂ insufflation could be an effective, safe option to flatten the diaphragm.

Open Transthoracic Plication of the Diaphragm for Unilateral Diaphragmatic Eventration in Infants and Children

BACKGROUND: To evaluate our experience of early surgical plication for diaphragmatic eventration (DE) in infancy and childhood. METHODS: This study evaluated infants and children with symptomatic DE who underwent plication through an open transthoracic approach in our childhood development department between January 2005 and December 2012. Surgical plication was performed in several rows using polypropylene U-stitches with Teflon pledgets. RESULTS: The study included 12 infants and children (7 boys and 5 girls) with symptomatic DE (9 congenital and 3 acquired). Reported symptoms included respiratory distress (91.7%), wheezing (75%), cough (66.7%), and recurrent pneumonia (50%). Preoperative mechanical ventilatory support was required in 41.7% of the patients. The mean length of hospital stay was 6.3+/-2.5 days. The mean follow-up period was 24.3+/-14.5 months. Preoperative symptoms were immediately relieved after surgery in 83.3% of patients and persisted in 16.7% of patients one year after surgery. All patients survived to the end of the two-year follow-up and none had recurrence of DE. CONCLUSION: Early diagnosis and surgical plication of the diaphragm for symptomatic congenital or acquired diaphragmatic eventration offers a good clinical outcome with no recurrence.

Two Cases of Congenital Diaphragmatic Hernia Manifestated with Atypical Symptoms in Newborn Infants / 대한주산의학회잡지

Most of the congenital diaphragmatic hernia (CDH) cases are diagnosed at prenatal period or immediately after birth with severe respiratory symptom. The classic triad, which is respiratory distress, apparent dextrocardia and a scaphoid abdomen, is usually seen in this period. Several case reports have described older infants and children with a wide spectrum of symptoms of CDH, whereas extremely few cases were reported in neonatal period except classic triad such as straungulation of the bowel. These atypical manifestations can lead physician to delayed diagnosis. We report two cases of CDH newborns. First case was diagnosed with pneumoperitoneum following tension pneumothorax, transient diaphragm eventration on 5 days after birth. The other case was diagnosed with failure to thrive and mediastinal mass on 30 days after birth. These cases suggest physicians to consider CDH in late newborn period with pneumoperitoneum following tension pneumothorax, transient diaphragm eventration, failure to thrive, and mediastinal mass.

Eventracion congenita de diafragma, a proposito de un caso / Congenital eventration of diaphragm, a purpose of a case

La eventración diafragmática es una anomalía poco frecuente, que se caracteriza por una elevación anormal del músculo diafragma, pudiendo ser de causa congénita o adquirida. El diafragma afectado presenta movilidad paradójica, con afectación respiratoria de intensidad variable; se ha considerado como causa del defecto una falla en el proceso de muscularización del diafragma embrionario. Se presenta el caso de una recién nacida, sin antecedentes obstétricos de importancia, que presentó síntomas respiratorios leves desde el nacimiento; en este caso se realizó el diagnóstico por imágenes con una radiografía y tomografía de tórax. La resolución quirúrgica fue mediante técnica de plicatura diafragmática.

The diaphragmatic eventration is a rare anomaly, which is characterized by an abnormal elevation of the diaphragm muscle, can be congenital or acquired. The affected diaphragm displays paradoxical mobility, with respiratory insufficiency of variable intensity; it has been considered as a cause of the defect of a failure in the process of muscularization of the embryonic diaphragm. This is the case of a newly born, without obstetric history of importance, who presented mild respiratory symptoms from birth; in this case, the diagnosis was performed with an X-ray and CT scan of the chest. The surgical resolution was through a technique of diaphragmatic plication.

Evaluación por imágenes del diafragma en el niño / Image evaluation of diaphragm in children

The anatomy, embriology andfunctions of the neonate 's diaphragm, as well as its anatomical and functional oddities were reviewed. And, in a deeper way, imaging studies were also reviewed; these have an important role in functional and anatomic evaluation of the diaphragm, each one with its own advantages and limitations. Chest X-rays allow an anatomic two dimensional evaluation of the diaphragm and constitutes the first approach in the study of diaphragm pathology in children; hence, the normal anatomy and the most common pathological signs are reviewed. Digestive tube studies using contrast media still are the best choice for diagnosis of hiatal hernia and of herniation through the foramen of Morgagni, where the colon is ascended. Ultrasound use is highlighted for the evaluation of diaphragmatic motility, as well as some of its advantages over fluoroscopy, which is and has been the method of choice in the diagnosis of diaphragmatic paralysis in children. Multiplanar images are the most complete method for the anatomic evaluation of the diaphragm, since they show its spatial orientation and allow the detailed evaluation of those pahologies where the anatomy is altered, such as diaphragmatic hernias, trauma and tumors.

Revisamos la embriología, anatomía y funciones del diafragma, sus particularidades anatómicas y funcionales en los neonatos y, en forma más profunda, los estudios por imágenes, que en la actualidad tienen un importante rol en su evaluación anatómica y funcional, cada uno de ellos con sus ventajas y limitaciones. La radiografía de tóraxpermite una evaluación anatómica en dos planos del diafragma y constituye la primera aproximación en el estudio de la patología del diafragma en los niños, por lo que se describe la anatomía normal y los signos de las patologías más frecuentes. Los estudios contrastados del tubo digestivo siguen siendo de elección para el estudio de las hernias hiatales y hernias de Morgagni en donde está ascendido el colon. Destacamos el uso del ultrasonido en la evaluación de la motilidad diafragmática, y algunas de sus ventajas sobre la fluoroscopia, que es y ha sido el método de elección en el diagnóstico de la parálisis diafragmática en los niños. Las imágenes multiplanares son un método más completo en la evaluación anatómica del diafragma, muestran mejor su orientación espacial y permiten la evaluación detallada de las patologías donde la anatomía está alterada, como hernias diafragmáticas congénitas, trauma y tumores.

Rare association of eventration of left hemidiaphragm with ipsilateral thyroid agenesis.

Eventration is a well-known congenital malformation of the diaphragm, usually asymptomatic and diagnosed incidentally on chest radiography. It is sometimes associated with a number of other congenital syndromes and anomalies. We report a rare case of eventration of left hemidiaphragm associated with gastric volvulus, ipsilateral thyroid agenesis and microphthalmia.

Surgical repair of congenital diaphragmatic eventration in a septugerian lady / Reparo cirúrgico de eventração diafragmática congênita em paciente septuagenária

Congenital diaphragmatic eventration in an elderly is a rare anomaly. We describe a case of congenital diaphragmatic eventration causing respiratory insufficiency in a 72 year lady who underwent successful surgical repair.

A eventração diafragmática congênita em um idoso é uma anomalia rara. Descrevemos um caso de eventração diafragmática congênita que causava insuficiência respiratória em uma idosa de 72 anos, que passou, com sucesso, por uma cirurgia de reparo.

Clinical Study of Congenital Diaphragmatic Diseases in Neonates and Infants

Congenital diaphragmatic disease is one of the common major congenital anomalies, and its mortality remained still high despite recent medical advances. The aim of this study is to examine the clinical characteristics of congenital diaphragmatic diseases. A total of 39 patients with congenital diaphragmatic disease that underwent surgery from January, 1997 to December, 2009 at Pusan National University Hospital were included in this study. Medical records were retrospectively reviewed. The male to female ratio was 30:9. Six out of 39 cases died (NS) before surgery, 17 patients had Bochdalek's hernia (BH), 11 patients hiatus hernia (HH), 4 diaphragmatic eventration (DE), and 1 Morgagni hernia (MH). There were no differences in mean birth weight and mean gestational age. NS (83.3%). BH (35.3%) was diagnosed more frequently than other diseases in the prenatal period. Three patients (17.6%) of BH expired due to pulmonary hypoplasia and 1 patient had co-existing congenital heart disease. BH was diagnosed more frequently in the prenatal stage and had a higher motality rate than other conditions. Therefore, BH needs to be concentrated more than other anomalies.

Eventroplastia laparoscópica / Laparoscopic eventroplasty

Eventration is the protusion or the way out of the abdominal content through an opening in the abdominal wall. This content can be an omentum or viscera allocated in the subcutaneous tissue in a sac of fibrous tissue and rests of peritoneum. The technique for laparoscopic route is effective to treat eventrations, even in obese patients and with previous recidivals. With this procedure decreases the incidence of postoperative complications. The author describes both the anatomical technique, and later on the laparoscopic technique.