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.
Diaphragmatic Eventration ; Hernia, Diaphragmatic ; Laparoscopy

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.
Child ; Diaphragm ; Diaphragmatic Eventration ; Four-Dimensional Computed Tomography ; Humans ; Lung ; Paralysis ; Pleural Effusion ; Thorax ; Ultrasonography

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.
Arachnodactyly ; Calcium ; Cisterna Magna ; Codon ; Contracture ; Diagnosis ; Diaphragmatic Eventration ; Dilatation ; Ear ; Ectopia Lentis ; Exons ; Follow-Up Studies ; Glutamic Acid ; Humans ; Hydronephrosis ; Infant, Newborn ; Joints ; Korea* ; Lysine ; Male ; Marfan Syndrome* ; Mutation, Missense ; Skin ; Tricuspid Valve Insufficiency

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.
Abdomen ; Body Weight ; Bronchi ; Diaphragm ; Diaphragmatic Eventration ; Dilatation ; Esophageal Atresia ; Esophagus ; Follow-Up Studies ; Hernia ; Humans ; Mediastinum ; Minimally Invasive Surgical Procedures ; Operative Time ; Pediatrics ; Retrospective Studies ; Seoul ; Thoracoscopy* ; Thorax

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.
Busan ; Carbon Dioxide* ; Carbon* ; Chest Tubes ; Diaphragm ; Diaphragmatic Eventration ; Drainage ; Dyspnea ; Fatigue ; Follow-Up Studies ; Forced Expiratory Volume ; Hospital Mortality ; Humans ; Insufflation ; Length of Stay ; Medical Records ; Methods ; One-Lung Ventilation ; Operative Time ; Recurrence ; Respiratory Muscles ; Retrospective Studies ; Spirometry ; Thoracic Surgery, Video-Assisted ; Thoracoscopy ; Transcutaneous Electric Nerve Stimulation ; Ventilation ; Vital Capacity

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.
Child* ; Cough ; Diaphragm* ; Diaphragmatic Eventration* ; Early Diagnosis ; Follow-Up Studies ; Humans ; Infant* ; Length of Stay ; Pneumonia ; Polypropylenes ; Polytetrafluoroethylene ; Recurrence ; Respiratory Sounds ; Thoracotomy

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.
Abdomen ; Child ; Delayed Diagnosis ; Dextrocardia ; Diaphragmatic Eventration ; Failure to Thrive ; Hernia, Diaphragmatic* ; Humans ; Infant ; Infant, Newborn* ; Parturition ; Pneumoperitoneum ; Pneumothorax

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.
Birth Weight ; Diaphragmatic Eventration ; Female ; Gestational Age ; Heart Diseases ; Hernia ; Hernia, Diaphragmatic ; Hernia, Hiatal ; Humans ; Infant ; Infant, Newborn ; Male ; Medical Records ; Retrospective Studies

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.
Diaphragm ; Diaphragmatic Eventration ; Hernia, Diaphragmatic ; Humans ; Infant ; Infant Mortality ; Muscles ; Pregnancy ; Prognosis

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.
Aged ; Bronchiectasis ; Colon ; Diaphragm ; Diaphragmatic Eventration ; Displacement (Psychology) ; Female ; Gastropexy ; Humans ; Necrosis ; Pneumonectomy ; Recurrence ; Stomach Volvulus