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1.
Ann Med Surg (Lond) ; 86(9): 5586-5589, 2024 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-39238984

RESUMEN

Introduction and importance: The pericardial hernia is a rarely encountered clinical condition characterized by the herniation of the abdominal viscera into the pericardial cavity. Trauma precedes the development of these hernias in most cases, yet iatrogenic intervention and congenital defects are other potential causes. Case presentation: A male aged 60 years, with no history of previous trauma, presented with epigastric pain and was diagnosed with a case of STEMI. After the treatment, the patient continued to deteriorate and developed repeated vomiting. Surgical consultation was counseled which recommended a plain erect abdominal radiograph that revealed air-fluid levels. During surgery, the surgeon noticed a strangulated jejunum protruding into the pericardium, and the case was diagnosed as a pericardial hernia. Clinical discussion: Pericardial hernias could be congenital or acquired and trauma is regarded as the commonest cause in adults. According to the history and clinical presentation the cause could be an old-forgotten trauma that has led to the small defect formation and protrusion of the jejunum. Trauma is regarded as the commonest cause; however, cases can pass unnoticed. The clinical presentation of this patient was initially related to acute coronary syndrome and was treated with primary coronary intervention, later a co-existing cause or probably the initiating cause was found to be a pericardial hernia. Conclusion: The diagnosis of the case makes a significant challenge that requires a high index of suspicion due to the rarity of the condition, variable clinical presentation, and the delayed development of symptoms.

2.
J Surg Case Rep ; 2024(9): rjae566, 2024 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-39239144

RESUMEN

Right sided congenital diaphragmatic hernia (CDH) associated with hepatopulmonary fusion (HPF) is a rare congenital anomaly in which the herniated liver is fused with lung parenchyma. We discuss the case of an infant with right-sided CDH and HPF found on index operation for repair of right-sided CDH. Due to the high incidence of vascular anomalies associated with HPF the decision was made to close the patient and get further imaging to characterize the HPF before returning to the operating room for definitive repair. We describe a novel and successful operative approach where the liver was left entirely fused to the lung and the liver was partially brought down from the chest and was plicated to the diaphragm form a seal between the parietal and pleural cavities to repair the CDH.

3.
Sleep Breath ; 2024 Sep 09.
Artículo en Inglés | MEDLINE | ID: mdl-39249608

RESUMEN

PURPOSE: Obstructive sleep apnea (OSA) is characterized by recurrent upper airway narrowing or collapse during sleep. Continuous positive airway pressure (CPAP) remains the preferred treatment in selected patients and masks' choice plays an important role for subsequent respiratory events' reduction. It is known that oronasal masks are not as effective at opening the upper airway compared to nasal ones. Thus, the objective of this study was to investigate differences in US-assessed diaphragmatic excursion (DE) using oronasal vs. nasal CPAP masks. METHODS: This observational study included 50 OSA patients presenting a moderate to severe apnea-hypopnea index and requiring CPAP treatment. All participants received US evaluations on diaphragm motion during their oronasal and nasal CPAP trial at equal positive end-expiratory pressure level. RESULTS: The difference of DE switching mask during CPAP was assessed by using the non-parametric Wilcoxon signed-rank test. A statistically significant increase in US- assessed DE was found when shifting from oronasal to nasal mask (p-value < 0.01). Linear regression models revealed that increased neck circumference and more severe AHI were associated with decreased DE when shifting to an oronasal mask. CONCLUSION: This study evaluated the acute impact on US-assessed DE after changing CPAP route from oronasal to nasal mask. Our results suggest that the nasal type should be the more suitable option for most patients with OSA, especially those with higher nasal circumference. Diaphragmatic motion throughout US may become a practical tool to help in the choice of the fittest mask in patients undergoing CPAP.

4.
Radiol Case Rep ; 19(11): 5044-5049, 2024 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-39253049

RESUMEN

Congenital diaphragmatic hernia (CDH) is a developmental disorder in which the diaphragm, the muscle that separates the chest from the abdomen, does not close during prenatal development, allowing abdominal organs to herniate into the chest cavity. It occurs mainly on the left side (80%-85% of cases). CDH is often identified during prenatal assessment. However, instances of late-presenting CDH beyond infancy are exceedingly uncommon, contributing to frequent misdiagnosis and delayed therapeutic intervention. We present a case of a 10-month-old female with an uneventful antenatal and perinatal history who presented with respiratory distress and multiple episodes of vomiting. Her vital signs were stable upon arrival, but she was sent to the PICU due to hypoactivity, reduced oral intake, and agitation. After an urgent CT scan, a herniation of the small and large bowel loops into the right hemithorax was discovered, along with a defect in the right hemidiaphragm. This resulted in a pleural effusion on the right side, a partially collapsed left lung, and a mediastinal shift to the left. The diaphragmatic hernia was corrected through a lateral thoracotomy at the sixth rib with multiple interrupted sutures, and a chest tube was then inserted into the pleural space above the diaphragm following a smooth reduction of the bowl. This case highlights the importance of early diagnosis, appropriate clinical investigation, and treatment. A good prognosis can be anticipated by promptly discovering and examining the condition.

5.
Eur J Obstet Gynecol Reprod Biol ; 302: 134-140, 2024 Sep 05.
Artículo en Inglés | MEDLINE | ID: mdl-39265199

RESUMEN

OBJECTIVES: This study aims to ascertain the prevalence of appendiceal endometriosis (AppE) in patients diagnosed with diaphragmatic endometriosis (DiaE), compare it with the prevalence in patients without DiaE, and delineate the anatomical distribution of endometriotic lesions within these cohorts. STUDY DESIGN: Comparison of the characteristics of patients with AppE and DiaE with the characteristics of patients with abdominal endometriosis without diaphragmatic involvement, in a prospective cohort study. SETTING: Tertiary referral center; endometriosis center. PATIENTS: A cohort of 1765 patients with histologically confirmed endometriosis INTERVENTIONS: Evaluation of correlations between demographic, clinical, and surgical variables of AppE patients with DiaE and without DiaE. We performed appendectomies selectively, in the presence of gross abnormalities of the appendix, such as endometriotic implants, edema, tortuosity, and discoloration of the organ. MEASUREMENTS: Patients' characteristics were evaluated using basic descriptive statistics (chi-square test or Fisher's exact test). A logistic regression analysis was performed to evaluate the relationship (hazard ratio) between patient characteristics and the presence of DiaE and AppE. MAIN RESULTS: Within a cohort of 1765 patients with histologically confirmed endometriosis, 31 were identified with AppE (1.8 %), and 83 with DiaE (4.7 %). The prevalence of DiaE was significantly elevated at 30.1 % (25/83), among patients with AppE compared to those without AppE, who showed a DiaE prevalence of 7.2 % (6/83). The calculated odds ratio for DiaE given the presence of AppE was 5.5, 95 % CI 2.1-14.4, p = 0.0004, and risk ratio was 4.2, 95 % CI 1.8-9.6, p = 0.0008, indicating a profound association. Surgical interventions did not lead to significant perioperative or postoperative complications. In the group with DiaE, the left ovary was affected in 96 % of cases (24/25), p < 0.05, the right ureter in 80 % of cases (20/25), p < 0.01 (in 19/25 only the serosa was affected, due to external compression of an endometriotic nodule of the parametrium). Concurrent AppE and right diaphragm was found in 92 % of cases (23/25 patients), p < 0.001. The concurrent presence of DiaE and AppE was often associated with severe endometriosis, rASRM IV 72 % OR = 3, 95 % CI (1.216-7.872). CONCLUSION: The investigation delineates a marked association between AppE and DiaE, with an odds ratio of 5.5 and risk ratio of 4.2, suggesting a markedly increased likelihood of DiaE in patients with AppE. These statistics significantly substantiate the notion that AppE can serve as a predictive marker for DiaE, underscoring the necessity for a meticulous intraoperative assessment of diaphragmatic regions in patients diagnosed with AppE. The absence of a significant correlation between the depth of DiaE infiltration and the presence of AppE implies that the detection of AppE should prompt a thorough search for DiaE, regardless of the perceived severity of the endometriosis or preoperative results of diaphragmatic MRI.

6.
J Pediatr Surg ; : 161766, 2024 Aug 22.
Artículo en Inglés | MEDLINE | ID: mdl-39271309

RESUMEN

BACKGROUND: Institutions lack consensus on the management of patients with congenital diaphragmatic hernia (CDH) who are repaired on extracorporeal membrane oxygenation (ECMO). Our study aimed to evaluate risk factors associated with bleeding complications in patients with CDH repaired on ECMO. METHODS: A single-institution retrospective review evaluated all patients with CDH who underwent on-ECMO repair between January 2005 and December 2023. A significant bleeding complication post-repair was defined as bleeding necessitating re-operation. The association between preoperative factors and bleeding complications was evaluated. RESULTS: Forty-six patients were included. Bleeding complications developed in 11/46 (24%) patients. Birthweight (2.5 vs. 3.2 kg, p = 0.02), platelet count <100/mm3 (64% vs. 29%, p = 0.04), elevated blood urea nitrogen (BUN; 24.5 vs. 17.5 mg/dL, p = 0.05), and older age at repair (8 vs. 5 days, p = 0.04) were associated with bleeding. In univariate analysis, patients with platelets under 100/mm3 were more likely to develop a bleeding complication (OR = 4.4, p = 0.04). Patients who experienced a significant bleeding event experienced increased ECMO days (12 vs. 7 days, p < 0.01), ventilator days (31 vs. 18 days, p < 0.05), and lower survival to discharge (36% vs. 74%, p = 0.03). CONCLUSION: Among CDH patients undergoing repair on ECMO, those with lower birth weight, platelet counts under 100/mm3, elevated BUN, and older age at repair had an increased risk of a significant bleeding complication, resulting in more ECMO and ventilator days and higher mortality. Patients undergoing on-ECMO repair should have platelet count transfused to greater than 100/mm3. Patients at high risk for bleeding may benefit from early repair on ECMO. LEVEL OF EVIDENCE: Level III.

7.
Ann Surg Oncol ; 2024 Sep 16.
Artículo en Inglés | MEDLINE | ID: mdl-39283579

RESUMEN

BACKGROUND: Inspiratory muscle training (IMT) has preventive effects against postoperative pulmonary complications (PPCs) after upper abdominal surgery. However, its impact on diaphragmatic function has not been evaluated. This study investigated the effect of preoperative IMT on diaphragmatic excursion (DE) and prevention of PPCs for patients with esophageal cancer. METHODS: This study was an unblinded, parallel, randomized controlled trial. Patients with thoracic or abdominal esophageal cancer scheduled for esophagectomy were randomized into the incentive spirometry (IS) or IMT group. During preoperative neoadjuvant chemotherapy, IS or IMT intervention was performed. The inspiratory resistance of the IMT group was consistently set at 50% maximal inspiratory pressure. The primary outcome was the amount of change in DE evaluated with ultrasonography, and the secondary outcome was the incidence of Clavien-Dindo grade II or higher PPCs. RESULTS: This study recruited 42 patients. Among these patients 21 were randomized into the IS or IMT group, and 2 patients dropped out from the study. Finally, 40 patients were included in this analysis. The DE of the IMT group increased significantly after the intervention. The IMT group had significantly larger DE changes than the IS group. Of the 39 patients analyzed for postoperative outcome, 5 experienced grade II PPCs. The IMT group had a lower incidence of PPCs than the IS group. CONCLUSIONS: Patients with thoracic and abdominal esophageal cancer scheduled for surgery who had preoperative IMT have increased DE, which may have an important role in prevention of PPCs.

8.
Biomed Rep ; 21(5): 157, 2024 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-39268399

RESUMEN

Adult congenital localized diaphragmatic eventration (DE) is an uncommon condition that, due to the absence of notable symptoms, is often ignored and is consequently not frequently treated following diagnosis. Furthermore, the progression of localized DE into complete DE is rare. The recent coronavirus disease 2019 pandemic has placed a strain on global health care systems, including those in China, and exacerbated several underlying medical complications and diseases. This has resulted in a rapid progression of underlying diseases and even death in cases of diabetes or pre-existing respiratory diseases and other chronic conditions. The present study describes the case of a patient with congenital localized DE that progressed to complete DE following severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. The patient had a medical history of localized DE with persistent cough, chest tightness and abdominal distension following infection with SARS-CoV-2. Chest X-ray and computed tomography examinations confirmed the diagnosis of complete DE on the left side. Therefore, the patient underwent thoracic surgery for left-sided diaphragmatic plication. At the 6-month follow-up visit, the patient had recovered well and did not show any complications. Overall, the present study demonstrated that SARS-CoV-2 infection exacerbated the progression of left-sided congenital localized DE, which had no obvious symptoms, to complete DE accompanied by severe symptoms.

9.
Arthritis Res Ther ; 26(1): 164, 2024 Sep 18.
Artículo en Inglés | MEDLINE | ID: mdl-39294670

RESUMEN

Systemic autoimmune rheumatic diseases (SARDs) related pulmonary disease is highly prevalent, with variable clinical presentation and behavior, and thus is associated with poor outcomes and negatively impacts quality of life. Chest high resolution computed tomography (HRCT) is still considered a fundamental imaging tool in the screening, diagnosis, and follow-up of pulmonary disease in patients with SARDs. However, radiation exposure, economic burden, as well as lack of point-of-care CT equipment limits its application in some clinical situation. Ultrasound has found a place in numerous aspects of the rheumatic diseases, including the vasculature, skin, muscle, joints, kidneys and in screening for malignancies. Likewise it has found increasing use in the lungs. In the past two decades, lung ultrasound has started to be used for pulmonary parenchymal diseases such as pneumonia, pulmonary edema, lung fibrosis, pneumothorax, and pleural lesions, although the lung parenchymal was once considered off-limits to ultrasound. Lung ultrasound B-lines and irregularities of the pleural line are now regarded two important sonographic artefacts related to diffuse parenchymal lung disease and they could reflect the lesion extent and severity. However, its role in the management of SARDs related pulmonary involvement has not been fully investigated. This review article will focus on the potential applications of lung ultrasound in different pulmonary scenarios related with SARDs, such as interstitial lung disease, diffuse alveolar hemorrhage, diaphragmatic involvement, and pulmonary infection, in order to explore its value in clinical daily practice.


Asunto(s)
Enfermedades Autoinmunes , Enfermedades Pulmonares , Pulmón , Enfermedades Reumáticas , Ultrasonografía , Humanos , Enfermedades Reumáticas/diagnóstico por imagen , Ultrasonografía/métodos , Enfermedades Autoinmunes/diagnóstico por imagen , Enfermedades Pulmonares/diagnóstico por imagen , Pulmón/diagnóstico por imagen
10.
HCA Healthc J Med ; 5(4): 483-488, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-39290486

RESUMEN

Introduction: A diaphragmatic hernia (DH) is a defect within a part of the diaphragm that allows intra-abdominal contents to enter the thoracic cavity. Diaphragmatic hernias can be congenital or acquired later in life. The most common congenital DH is the Bochdalek hernia (posterolateral hernia), but the most commonly acquired DH is due to traumatic injury. These hernias are rare in adults and are typically diagnosed incidentally. Surgical repair is the standard of care; however, data regarding the surgical approach is scarce. We report a case of a rare right posterolateral DH in an adult female patient who presented with acute abdominal pain. Case Presentation: A 69-year-old female presented with recurrent epigastric pain that had acutely worsened, nausea, vomiting, and food intolerance. A computed tomography (CT) scan demonstrated a right posterolateral DH containing the hepatic flexure of the colon. The patient was taken urgently to surgery due to concern for strangulation. Reduction of the hernia was attempted laparoscopically but was converted to an open procedure with a subcostal incision due to poor visualization. This approach revealed adequate exposure of the defect and subsequent reduction of the herniated abdominal contents. The defect was easily closed without tension or the use of mesh. The patient was discharged on postoperative day 3. Conclusion: Chronic DH can have severe life-threatening sequelae when left untreated. This case demonstrates the importance of thorough history-taking and raises awareness of missed diaphragmatic injuries in trauma situations. Since patients who present with a symptomatic DH often need urgent repair, it is important for surgeons working in the acute care setting to understand the surgical options available and when mesh placement may benefit the situation. Our case outlines a successful primary defect repair, without mesh, of a right-sided DH in which a minimally invasive technique was attempted but converted to laparotomy for patient safety.

11.
J Pediatr ; : 114286, 2024 Sep 02.
Artículo en Inglés | MEDLINE | ID: mdl-39233115

RESUMEN

OBJECTIVE: To describe our experience utilizing epoprostenol for pulmonary hypertension (PH) in infants with congenital diaphragmatic hernia (CDH) requiring extracorporeal life support (ECLS). STUDY DESIGN: We retrospectively reviewed infants diagnosed with CDH who required ECLS at our institution from 2013-2023. Data collected included demographics, disease characteristics, medication administration patterns, and hospital outcomes. We first compared infants who received intravenous epoprostenol and those who did not. Among infants who received epoprostenol, we compared survivors and non-survivors. Chi-square/Fisher's exact and Mann-Whitney tests were used, with significance p<0.05. RESULTS: Fifty-seven infants were included; 40 (70.2%) received epoprostenol. Infants receiving epoprostenol had lower observed/expected total fetal lung volume (O/E TFLV) on MRI (20 vs. 26.2%, p=0.042) as well as higher prenatal frequency of liver-up (90 vs 64.7%, p=0.023) and "severe" classification (67.5 vs 35.3%, p=0.007). Survival with and without epoprostenol was comparable (60% vs. 64%, p=0.23). Of those receiving epoprostenol, both survivors and non-survivors had similar prenatal indicators of disease severity. Most (80%) of hernia defects were classified as Type C/D and 68% were repaired <72 hours after ECLS cannulation. The median age at initiation of epoprostenol was day of life 6 (IQR: 4, 7) in survivors and 8 (IQR: 7, 16) in non-survivors (p=0.012). Survivors had shorter ECLS duration (11 vs 20 days, p=0.049). Of non-survivors, refractory PH was the cause of death for 13 infants (81%). CONCLUSION: In infants with CDH requiring ECLS, addition of epoprostenol appears promising and earlier initiation may affect survival.

12.
BMC Med Imaging ; 24(1): 217, 2024 Aug 15.
Artículo en Inglés | MEDLINE | ID: mdl-39148010

RESUMEN

BACKGROUND: The ratio (E/Ea) of mitral Doppler inflow velocity to annular tissue Doppler wave velocity by transthoracic echocardiography and diaphragmatic excursion (DE) by diaphragm ultrasound have been confirmed to predict extubation outcomes. However, few studies focused on the predicting value of E/Ea and DE at different positions during a spontaneous breathing trial (SBT), as well as the effects of △E/Ea and △DE (changes in E/Ea and DE during a SBT). METHODS: This study was a reanalysis of the data of 60 difficult-to-wean patients in a previous study published in 2017. All eligible participants were organized into respiratory failure (RF) group and extubation success (ES) group within 48 h after extubation, or re-intubation (RI) group and non-intubation (NI) group within 1 week after extubation. The risk factors for respiratory failure and re-intubation including E/Ea and △E/Ea, DE and △DE at different positions were analyzed by multivariate logistic regression, respectively. The receiver operating characteristic (ROC) curves of E/Ea (septal, lateral, average) and DE (right, left, average) were compared with each other, respectively. RESULTS: Of the 60 patients, 29 cases developed respiratory failure within 48 h, and 14 of those cases required re-intubation within 1 week. Multivariate logistic regression showed that E/Ea were all associated with respiratory failure, while only DE (right) and DE (average) after SBT were related to re-intubation. There were no statistic differences among the ROC curves of E/Ea at different positions, nor between the ROC curves of DE. No statistical differences were shown in △E/Ea between RF and ES groups, while △DE (average) was remarkably higher in NI group than that in RI group. However, multivariate logistic regression analysis showed that △DE (average) was not associated with re-intubation. CONCLUSIONS: E/Ea at different positions during a SBT could predict postextubation respiratory failure with no statistical differences among them. Likewise, only DE (right) and DE (average) after SBT might predict re-intubation with no statistical differences between each other.


Asunto(s)
Extubación Traqueal , Diafragma , Insuficiencia Respiratoria , Desconexión del Ventilador , Humanos , Masculino , Diafragma/diagnóstico por imagen , Diafragma/fisiopatología , Femenino , Estudios Retrospectivos , Insuficiencia Respiratoria/diagnóstico por imagen , Insuficiencia Respiratoria/fisiopatología , Anciano , Desconexión del Ventilador/métodos , Persona de Mediana Edad , Curva ROC , Ecocardiografía/métodos , Corazón/diagnóstico por imagen , Factores de Riesgo
13.
Asian J Endosc Surg ; 17(4): e13372, 2024 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-39143668

RESUMEN

We report the case of a 32-year-old man who developed a giant diaphragmatic hernia following the removal of a left ventricular assist device 4 years prior due to improved cardiac function. Chest radiography revealed an intrathoracic prolapse of the gastrointestinal tract. The patient was diagnosed with a diaphragmatic hernia and a laparoscopy-assisted repair was performed. A 12 × 8 cm hernia was found intraoperatively on the left diaphragm, and a large portion of the gastrointestinal tract had prolapsed into the thoracic cavity. We attempted to repair the ventromedial defect using mesh; however, it was found to be insufficient. Therefore, we used a left rectus abdominis myocutaneous flap to fill the defect and sutured it to the mesh. A myocutaneous flap could be a useful strategy in cases where complete closure with mesh is difficult.


Asunto(s)
Remoción de Dispositivos , Corazón Auxiliar , Herniorrafia , Laparoscopía , Colgajo Miocutáneo , Mallas Quirúrgicas , Humanos , Masculino , Adulto , Herniorrafia/métodos , Colgajo Miocutáneo/trasplante , Hernia Diafragmática/cirugía , Hernia Diafragmática/etiología
14.
Pediatr Surg Int ; 40(1): 240, 2024 Aug 22.
Artículo en Inglés | MEDLINE | ID: mdl-39172194

RESUMEN

PURPOSE: This study aimed to clarify surgical complications associated with open surgery for congenital diaphragmatic hernia (CDH). METHODS: We performed an exploratory data analysis of the clinical characteristics of surgical complications of neonates with CDH who underwent laparotomy or thoracotomy between 2006 and 2021. Data of these patients were obtained from the database of the Japanese CDH Study Group. RESULTS: Among 1,111 neonates with left or right CDH, 852 underwent open surgery (laparotomy or thoracotomy). Of these 852 neonates, 51 had the following surgical complications: organ injury (n = 48; 6% of open surgeries); circulatory failure caused by changes in the organ location (n = 2); and skin burns (n = 1). Injured organs included the spleen (n = 30; 62% of organ injuries), liver (n = 7), lungs (n = 4), intestine (n = 4), adrenal gland (n = 2), and thoracic wall (n = 2). Fourteen of the patients who experienced organ injury required a blood transfusion (2% of open surgeries). The adjusted odds ratio of splenic injury for patients with non-direct closure of the diaphragm was 2.2 (95% confidence interval, 1.1-4.9). CONCLUSION: Of the patients who underwent open surgery for CDH, 2% experienced organ injury that required a blood transfusion. Non-direct closure of the diaphragmatic defect was a risk factor for splenic injury.


Asunto(s)
Hernias Diafragmáticas Congénitas , Complicaciones Intraoperatorias , Humanos , Hernias Diafragmáticas Congénitas/cirugía , Japón/epidemiología , Masculino , Femenino , Recién Nacido , Complicaciones Intraoperatorias/epidemiología , Laparotomía/métodos , Toracotomía/métodos , Toracotomía/efectos adversos , Estudios Retrospectivos
15.
Cureus ; 16(7): e63754, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-39099961

RESUMEN

Morgagni hernia (MH), also known as a retrosternal or parasternal hernia, is a rare type of congenital diaphragmatic hernia (CDH) characterized by a defect in the anterior diaphragm. Patients with late-diagnosed MH typically present with vague gastrointestinal or respiratory symptoms. In some instances, MH is incidentally identified through chest X-rays performed for other reasons, such as foreign body ingestion, as illustrated in our presented case. We present a case of a delayed congenital diaphragmatic hernia of the Morgagni type in a two-year-old boy with a history of foreign body ingestion and severe abdominal pain. Diagnostic imaging, including chest radiograph and computed tomography (CT) scan, confirmed the diaphragmatic defect. Surgical repair, performed laparoscopically, resulted in an uncomplicated postoperative course and a favorable long-term outcome.

16.
J Pediatr Genet ; 13(3): 237-244, 2024 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-39086451

RESUMEN

Although 40 years have passed since the first case of DiGeorge's syndrome was described, and the knowledge about this disorder has steadily increased since that time, 22q11.2 deletion syndrome (DS) remains a challenging diagnosis because its clinical presentation varies widely. We describe an infant with 22q11.2 DS who presented with annular pancreas, anorectal malformation, Morgagni-type congenital diaphragmatic hernia, and ventricular septal defect. This constellation of anomalies has never been described in DiGeorge's syndrome. Here, we provide a case presentation and a thorough review of the literature.

17.
Front Pediatr ; 12: 1389062, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-39086625

RESUMEN

Objective: To explore the relationship between Oxygenation Index (OI) and Oxygen Saturation Index (OSI) among infants with Congenital Diaphragmatic Hernia (CDH), both within the first 24 h after birth and in extended observations in those who survived until their surgical intervention. Methods: Seven- years retrospective review of CDH cases at a single Level III neonatal intensive care unit. The correlations of various combinations of OI-OSI pairs were assessed using the Spearman's rho Correlation Coefficient. Additionally, during the initial 24 h, the correlations between admission (first), best (lowest), highest, and mean OI and OSI values were determined. The predictive ability of the first 24 h oxygen and oxygen saturation indices for mortality and other adverse outcomes were assessed using the Area Under the Curve (AUC) analysis. Results: Thirty-seven infants with CDH were included in the analysis. A strong correlation was observed between all pairs of OI/OSI (2,289) (Spearman's rho = 0.843), matched pairs of Postductal OI/OSI (1,232 pairs) (Spearman's rho = 0.835) and the unmatched pairs of Postductal OI and Preductal OSI (1,057 pairs) (Spearman's rho = 0.852). Using the regression equations for all pairs, matched and unmatched OI/OSI pairs, we deduced that for clinically pertinent OI thresholds of 10, 15, 20 and 40, the corresponding OSI values were 5, 8, 11, and 23, respectively. Furthermore, in the first 24 h, strong correlations were evident between OI/OSI: at admission (Spearman's rho = 0.783), best OI/OSI (Spearman's rho = 0.848), and highest OI/OSI (Spearman's rho = 0.921). The most robust correlation was observed between the mean OI/OSI with a Spearman's rho of 0.928. First (AUC = 0.849), best (AUC = 0.927), highest (AUC = 0.942) and mean day 1 OI (AUC = 0.946) were all predictive of mortality. Similarly, first (AUC = 1.00), best (AUC = 0.989), highest (AUC = 1.00) and the mean OSI in day 1 (AUC = 0.978) were all predictive of mortality. All of the OIs and OSIs in day 1 except for the admission OSI (AUC = 0.683) were predictive of pulmonary hypertension. Additionally, all of OI and OSI indices in the first 24-hour except for the best day 1 OI (AUC = 0.674) were predictive of the need for rescue HFOV. Conclusion: There were a strong correlation between the OI and OSI in infants with CDH. Oxygenation indices and OSI in the first 24 h were predictive of mortality and other adverse outcomes in infants with CDH.

18.
Front Surg ; 11: 1369255, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-39086919

RESUMEN

Introduction: Blunt diaphragmatic rupture (BTDR) is a rare condition that can occur in children following high-energy blunt thoracoabdominal trauma. In less than 1% of the cases, pericardial rupture can coexist with a BTDR. A coexistence of BTDR and pericardial rupture can result in displacement of the heart and is associated with high mortality. Clinical presentation is non-specific and requires a high index of suspicion for early management. Case presentation: A 4-year-old child presented to the emergency unit of our hospital following high-energy trauma with severe respiratory distress. Initially, a left-side chest tube was inserted, but it resulted in no clinical improvement. A chest x-ray showed a collapse of the left lung with a herniation of bowel loops into the left hemithorax. An exploratory laparotomy was done, which revealed a 10 cm × 4 cm defect in the left hemidiaphragm with a medial extension involving the pericardium. The fundus of the stomach and left lobe of the liver were displaced into the pericardial space, pushing the cardiac apex posteriorly to the right side. Concomitantly, the transverse colon and small bowel were displaced into the left pleural space. After the reduction of the herniated abdominal viscera back into the peritoneal cavity, the pericardial sac was repaired by employing an interrupted resorbable suture, while the diaphragmatic defect was repaired by using a horizontal mattress. No other injuries were identified and the abdomen was closed in layers. Conclusion: BTDR with pericardial rupture is an elusive condition with a high mortality rate that necessitates a high index of clinical suspicion. Early surgical repair of the defect with a reduction of herniated organs can reduce morbidity and mortality.

19.
World J Clin Cases ; 12(22): 5253-5257, 2024 Aug 06.
Artículo en Inglés | MEDLINE | ID: mdl-39109022

RESUMEN

BACKGROUND: Diaphragmatic paralysis is typically associated with phrenic nerve injury. Neonatal diaphragmatic paralysis diagnosis is easily missed because its manifestations are variable and usually nonspecific. CASE SUMMARY: We report a 39-week-old newborn delivered via vaginal forceps who presented with tachypnea but without showing other birth-trauma-related manifestations. The infant was initially diagnosed with pneumonia. However, the newborn still exhibited tachypnea despite effective antibiotic treatment. Chest radiography revealed right diaphragmatic elevation. M-mode ultrasonography revealed decreased movement of the right diaphragm. The infant was subsequently diagnosed with diaphragmatic paralysis. After 4 weeks, tachypnea improved. Upon re-examination using M-mode ultrasonography, the difference in bilateral diaphragmatic muscle movement was smaller than before. CONCLUSION: Appropriate use of M-mode ultrasound to quantify diaphragmatic excursions could facilitate timely diagnosis and provide objective evaluation.

20.
Cureus ; 16(7): e63990, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-39109103

RESUMEN

Shrinking lung syndrome (SLS) is a rare pulmonary complication primarily associated with autoimmune diseases such as systemic lupus erythematosus (SLE). A 38-year-old female recently diagnosed with SLE on hydroxychloroquine, prednisone, and methotrexate presented with a one-week history of progressive shortness of breath, non-productive cough, and pleuritic chest pain. She was afebrile with adequate oxygen saturation. Examination revealed a few fine crackles in the lung fields. Laboratory results showed pancytopenia. Initial treatment included broad-spectrum antibiotics and intravenous methylprednisolone for a suspected lupus flare. Cultures and tests for infections, including tuberculosis, were negative. Imaging revealed bilateral airspace disease with no pulmonary embolism. Autoimmune workup showed high antinuclear antibodies, positive anticardiolipin antibody, ribonucleoprotein, and anti-Smith antibody. Diagnosed with SLS, she was started on a tapering dose of methylprednisolone and hydroxychloroquine, along with rituximab, leading to significant improvement. Pulmonary function tests (PFTs) showed a restrictive pattern. SLS, with a very low prevalence in SLE, can also occur in systemic sclerosis, Sjogren's syndrome, and rheumatoid arthritis. Typical symptoms include dyspnea, pleuritic chest pain, and cough. Diagnosis involves chest radiography showing an elevated diaphragm and restrictive PFT pattern. Treatment often includes corticosteroids such as methylprednisolone and immunosuppressive agents. Rituximab has shown improvement in cases unresponsive to conventional therapy.

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