Thoracoscopic surgery versus traditional open surgery for infants with congenital diaphragmatic eventration: A retrospective cohort study / 中国胸心血管外科临床杂志

@#Objective    To compare and analyze the treatment effect of thoracoscopic surgery and traditional open surgery on infants with congenital diaphragmatic eventration, and summarize the experience of thoracoscopic surgery in infants with congenital diaphragmatic eventration. Methods    We retrospectively analyzed the clinical data of 105 children with congenital diaphragmatic eventration who received operation in the Department of Cardiothoracic Surgery of Children’s Hospital of Chongqing Medical University from January 2010 to January 2019. The patients were divided into an open group and a thoracoscopic group according to the operation methods. There were 41 patients in the thoracoscopic group, including 30 males and 11 females, with an average of 13.42±11.08 months (range: 1 d to 3 years). There were 64 patients in the open group, including 44 males and 20 females, with an average age of 8.21±9.33 months (range: 15.0 d to 1.6 years). The operation time, intraoperative bleeding volume, postoperative mechanical ventilation time, hospital stay and other operation indexes as well as the mortality, recurrence rate and complication rate of the two groups were observed. Results    The operation indexes such as operation time, intraoperative bleeding volume, postoperative mechanical ventilation time, thoracic drainage time, CCU stay and hospital stay of the thoracoscopic group were better than those in the open group (P<0.05). There was no statistical difference between two groups in postoperative diaphgram muscles descent, postoperative feeding time or patients needing thoracic drainage (P>0.05). The incidence of postoperative complications in the thoracoscopic group (19.51%) was lower than that in the open group (23.44%, P>0.05), and the difference in mortality and recurrence rate between the two groups was not statistically significant (P>0.05). Conclusion    Both thoracoscopic diaphragmatic plication and traditional open surgery can effectively treat congenital diaphragmatic eventration, but compared with traditional open surgery, thoracoscopic diaphragmatic plication has the advantages of shorter operation time, less trauma, more rapid recovery and fewer complications, so it should be the first choice for children with congenital diaphragmatic eventration.

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)

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.

A “Near Miss" Congenital Eventration of the Right Hemidiaphragm in A Neonate: A Case Report

Focal eventration involving the posterior segment of the hemidiaphragm is a rare congenital anomaly. We report of a 10- day-old infant who presented with significant respiratory insufficiency and failure to show any responses to standard treatment. The diagnosis of focal eventration of the diaphragm was not anticipated until ultrasonographic examination revealed the defect. Diaphragmatic plication resulted in complete resolution of symptoms. A high level clinical awareness is crucial as a relatively simple surgical procedure could avert long term life-threatening complications.

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.

Presentación de un paciente con eventración diafragmática / Presentation of a Patient with Diaphragmatic Eventration

La eventración diafragmática constituye una rara anomalía congénita del diafragma que se presenta debido a una embriogénesis deficiente con atrofia de las fibras musculares del diafragma, transformación de conjuntiva y pérdida del tono muscular. Se presentó el caso de un paciente de 45 años de edad, mestizo, con antecedente de sufrir asma bronquial que acudió a Consulta de Cirugía del Centro Integral de Salud Salvador Allende, Caracas, en septiembre de 2011, refirió episodios de disnea y dolor torácico que se incrementaba con el ejercicio físico. Al examen clínico se detectaron ruidos hidroaéreos en hemitórax izquierdo lo que motivó la indicación de estudios radiográficos que evidenciaron la presencia de una eventración diafragmática de grandes proporciones; fue sometido a intervención quirúrgica con plicatura diafragmática con excelentes resultados.

The diaphragmatic eventration is a rare congenital anomaly of the diaphragm due to a deficient embryogenesis of the diaphragm, with atrophy of muscular fibers, conjunctive transformation and loss of the muscular tone. A 45- year-old male patient with history of asthma who came to Surgery Consultation of Salvador Allende Health Center, Caracas, in September 2011, with episodes of dyspnea and thoracic pain that was increasing with physical exercise. The examination revealed hydroaerial noises to the left hemithorax and radiographic exams were done, the diaphragmatic eventration diagnosis of big proportions was revealed. The surgical treatment was determined by diaphragmatic plication with excellent results.

Distocia de hombros y eventración diafragmática / Shoulder dystocia and diaphragmatic hernia

Introducción: la distocia de hombros es un evento impredecible que puede ser leve o grave. En el período neonatal constituye el factor de riesgo más importante para lesión del plexo braquial y una causa excepcional de eventración diafragmática por injuria del nervio frénico. Presentación del caso: se presenta un recién nacido producto de parto distócico por distocia de hombros, que nace severamente deprimido y en su evolución requiere soporte ventilatorio prolongado. Conclusiones: el estudio fluoroscópico concluyó una eventración diafragmática derecha, permaneciendo con un síndrome de dificultad respiratoria de aproximadamente un mes de evolución y sin requerir tratamiento quirúrgico hasta el momento actual. Las complicaciones más frecuentes fueron la bronconeumonía y las atelectasias a repetición. Se realizó una revisión actualizada del tema destacándose diagnóstico etiológico. Se presentan fotos previo consentimiento familiar.

Introduction: shoulder dystocia is an unpredictable event that can be mild or severe. In the neonatal period is the most important risk factor for brachial plexus injury and an exceptional cause of diaphragmatic hernia by phrenic nerve injury. Case presentation: a newborn is presented after a shoulder dystocia delivery, born severely depressed and whose evolution requires prolonged ventilatory support. Conclusions: in the fluoroscopic study we concluded right diaphragmatic hernia, with respiratory distress syndrome approximately of one month of evolution and without requiring surgical treatment to date. The most frequent complications were repeated bronchopneumonia and atelectasis. An updated review of the literature highlighting etiologic diagnosis was made. Photos are presented prior parental consent.

A Case of Focal Eventration of Left Hemidiaphragm with Transthoracic Left Kidney Confused with a Traumatic Diaphragmatic Hernia

Focal eventration of the diaphragm with transthoracic kidney is a very rare condition. It is usually asymptomatic and often revealed as an incidental finding on imaging studies. We presented a case of previously undiagnosed focal eventration of left hemidiaphragm with transthoracic left kidney confused with traumatic diaphragmatic hernia. Differentiation of these two conditions is important as each were managed differently. A traumatic diaphragmatic hernia needs early surgical intervention whereas no treatment was required for focal diaphragmatic eventration in most cases. Diagnostic laparoscopy confirmed the findings in this case and the patient was managed conservatively.

A Case of Congenital Right Diaphragmatic Eventration / 대한주산의학회잡지

Congenital diaphragmatic eventration is defined as an abnormal elevation of the diaphragm resulting from developmental abnormality of muscle fibers during gestation. Differentiation between congenital diaphragmatic hernia (CDH) and eventration is very difficult but important for perinatal management and prognosis, because CDH is associated with higher perinatal and neonatal mortality. We describe a case that was initially diagnosed by prenatal sonography as a right CDH and later confirmed as a congenital diaphragmatic eventration after the delivery with a brief review of the literatures.

Acute Gastric Volvulus due to Diaphragmatic Eventration

We present the case report of a 72-year-old female in whom diaphragmatic eventration and secondary gastric volvulus developed 10 years after a left partial pneumonectomy for a bronchiectasis. Eventration of the diaphragm is defined as an abnormal elevation of an intact diaphragm. The abnormally wide subdiaphragmatic space provides the potential for a gastric volvulus, which results from the strong negative intrathoracic pulling force created by the potential subphrenic space and paradoxical movement of the diaphragm. Unless this strong negative force is first eliminated, gastropexy alone will lead to recurrence. Obliteration of the subphrenic space by colonic displacement is a simple and effective way of abolishing this negative subdiaphragmatic pulling force. Treatment of gastric volvulus requires immediate surgical repair to prevent subsequent necrosis and perforation, with surgical correction of the underlying anatomic abnormality being the treatment of choice for gastric volvulus. We experienced a case of gastric volvulus due to diaphragmatic eventration who was treated with colonic displacement.

Eventración diafragmática derecha: reporte de un caso: unidad de cuidados intensivos neonatales, hospital pediátrico Doctor Roberto Gilbert Elizalde / Right diaphragmatic eventration: case report: neonatal intensive care unit, pediatric hospital Doctor Roberto Gilbert Elizalde

Se reporta el caso de un neonato femenino de 26 días evaluado y diagnosticado con una patología diafragmática, observándose 7 días antes del ingreso manifestaciones clínicas como tos, obstrucción nasal, rechazo a la succión y signos variables de dificultad respiratoria. En la radiografía de tórax se evidencia vísceras en el tórax. La valoración cardiológica fue normal. Se interviene quirúrgicamente y se encuentra eventración diafragmática. En su estancia hospitalaria presenta otras complicaciones digestivas, por lo que es sometido a una nueva cirugía, encontrándose malformaciones a nivel de tubo digestivo. La evolución clínica es favorable hasta el alta.

A 26 days female neonate case is reported. Evaluation and diagnosis was made: diaphragmatic pathology. Seven days before admission were present some signs like cough, nasal blockage, rejection to suction, and changeable signs of respiratory discomfort. In the chest X-ray we can see abdominal viscera in the thorax. Cardiologic evaluation was normal. Surgical treatment was applied and a big diaphragmatic eventration was found. In her stay at the Hospital the patient presented other digestive complications, which caused another surgical intervention, this time we found malformations in the digestive tract. Clinical evolution was favorable until discharge.

Chronic Recurrent Volvulus of the Colonic Splenic Flexure Associated with the Eventration of Left Diaphragm / 대한소화기학회지

The eventration of diaphragm is usually found incidentally on chest X-ray or sometimes presented as acute gastric volvulus. However, colonic volvulus on splenic flexure area complicated by diaphragmatic eventration is extremely rare. A 25 year old man complained of upper abdominal pain for three days. He had a history of brain injury during infant period, and had epilepsy and mental retardation. Plain chest X-ray showed left diaphragmatic eventration and marked dilatation of colon on splenic flexure area which had not been changed for last three years. Barium enema showed bird beak appearance on distal colon near the splenic flexure. Colonoscopic reduction failed. After decompression with rectal and nasogastric tubes, colonic volvulus was relieved. To prevent the recurrence of volvulus, we performed segmental resection of left colon including splenic flexure area and repaired the left diaphragmatic eventration. After the operation, the patient had no further recurrent episode of volvulus although ileus persisted.

Left Diaphragmatic Eventration Diagnosed as Congenital Diaphragmatic Hernia by Prenatal Sonography / 대한소아소화기영양학회지

Congenital diaphragmatic eventration is the abnormal elevation of the diaphragm into the thoracic cavity. Sometimes, it is not easy to differentiate congenital diaphragmatic eventration from diaphragmatic hernia by either prenatal sonography or postnatal chest radiography. However, differential diagnosis of both diseases is practical because of different prognosis and surgical approaches. Careful interpretation of postnatal serial chest X-rays is mandatory to differentiate between both diseases. We report two neonates with congenital diaphragmatic eventration of left diaphragm that initially misdiagnosed as diaphragmatic hernia by prenatal sonography and postnatal chest radiography.

A Case of Right Diaphragmatic Eventration Initially Diagnosed as Congenital Diaphragmatic Hernia / 대한주산의학회잡지

Diaphragmatic eventration is an abnormal elevation of an intact diaphragm into the thoracic cavity as a result of paralysis, aplasia or atrophy of muscular fibers, which accounts for 5~10% of all diaphragmatic disorders. Congenital eventration result from a incomplete muscularization of the pleuroperitoneal membranes at 8~10 weeks' menstrual age, the cause of this failure is not known. Although some patients are asymptomatic and find out incidentally, significant compression of the affected chest contents can result in severe respiratory distress. The differentiation between congenital diaphragmatic eventration and congenital diaphragmatic hernia by sonography may be difficult, but important because of a significant differences in postnatal management and prognosis. We experienced a case of right congenital diaphragmatic eventration with severe respiratory distress during immediate postnatal period, who was initially diagnosed as congenital diaphragmatic hernia and performed plication at 18 hours after birth. So we report this case with review of literatures.

Change of Diaphragmatic Level and Movement Following Division of Phrenic Nerve / 대한흉부외과학회지

BACKGROUND: Diaphragm is innervated by phrenic nerve and lower intercostal nerves. For patients with avulsion injury of brachial plexus, an in situ graft of phrenic nerve is frequently used to neurotize a branch of the brachial plexus. We studied short-term and mid-term changes of diaphragmatic level and movement in patients with dissection of phrenic nerve for neurotization. MATERIAL AND METHOD: Thirteen patients with division of either-side phrenic nerve for neurotization of musculocutaneous nerve were included in this study. With endoscopic surgical procedure, the intrathoracic phrenic nerve was entirely dissected and divided just above the diaphragm. The dissected phrenic nerve was taken out through thoracic inlet and neck wound and then anastomosed to the musculocutaneous nerve through a subcutaneous tunnel. With chest films and fluoroscopy, levels and movements of diaphragm were measured before and after operation. RESULT: There was no specific technical difficulty or even minor postoperative complications following endoscopic division of phrenic nerve. After division of phrenic nerve, diaphragm was soon elevated about 1.7 intercostal spaces compared with the preoperative level, but it did not show paradoxical motion in fluoroscopy. More than 1.5 months later, diaphragm returned downward close to the preoperative level (average level difference was 0.9 intercostal spaces; p=NS). Movement of diaphragm was not significantly decreased compared with the preoperative one. CONCLUSION: After division of phrenic nerve, the affected diaphragm did not show a significant decrease in movement, and the elevated diaphragm returned downward with time. However, the decreased lung volumes in the last spirometry suggest the decreased inspiratory force following partial paralysis of diaphragm.

A case of mesenteroaxial type of acute gastric volvulus associated with diaphragmatic eventration / 대한내과학회지

Acute gastric volvulus is extremely rare emergency surgical condition by abnormal rotation of stomach. It presents a puzzling picture which makes early diagnosis difficult, yet surgical interference must be accomplished early if life is to be saved. Gastric volvulus can be classified anatomically as organoaxial or mesenteroaxial. The symptoms triad of gastric volvulus are severe nausea with a paradoxical inability to vomiting, localized epigastric pain and impossibility of introducing a gastric tube. The diagnosis of it may be suspected on plain radiographic examination of the abdomen and symptoms, it is confirmed by specific findings on the upper gastrointestinal series. We experienced a case of mesenteroaxial type of acute gastric volvulus associated with diaphragmatic eventration. We treated this patient with reduction of volvulus, repair of diaphragmatic eventration, gastrojejunostomy and gastropexy. The authors report this case with a brief review of recent literatures.

A Central Diaphragmatic Eventration with Accessory Hepatic Lobe Causing Cardiac Compression / 대한흉부외과학회지

A case of congenital diaphragmatic eventration on the right and central tendinous portion with accessory hepatic lobe causing direct compression of the right heart is presented. We have performed the video assisted thoracoscopic plication of the right hemidiaphragm and eliminated the mass effect of the accessory hepatic lobe.

Spontaneous Rupture of Diaphragm in an 8 Years-old Girl with Nephrotic Syndrome

Diaphragmatic eventration and spontaneous rupture of the diaphragm are not common. Authors experienced a case of spontaneous rupture of the congenital diaphragmatic eventration. An 8 year-old girl with right congenital diaphragmatic eventration and nephrotic syndrome, visited emergency room because of severe abdominal pain and vomiting. She had intermittent abdominal pain for 1 year. Plain chest X-ray and ultrasonography showed entrapped bowels in the right thoracic cavity. She underwent exploratory laparotomy. Reduction of the herniated abdominal viscera, trimming of the ruptured diaphragm and plication of the diaphragm were done. Postoperative course was uneventful. In the English literature there is only one reported case of spontaneous rupture of eventrated diaphragmatic.

Organo-axial volvulus of the stomach with diaphragmatic eventration

Gastric volvulus occurs when the stomach rotates about its longitudinal axis (organo-axial volvulus), or about an axis joining the lesser and greater curvatures (mesentero-axial volvulus). Primary gastric volvulus, making up one third of cases, occurs when the stabilizing ligaments are too lax as a result of congenital or acquired causes. Secondary gastric volvulus, making up the remainder of cases, occurs in association with a paraesophageal hernia or other congenital or acquired diaphragmatic defects. While gastric volvulus may occur acutely, especially in children, it may not be clinically apparent and discovered incidentally. The authors present a case of chronic organo-axial volvulus of the stomach secondary to left hemidiaphragmatic eventration with a review of the relevant literature.