A rare complication of pediatric liver transplantation: Post-transplant diaphragmatic hernia.

AIM: Acquired post-transplant diaphragmatic hernia (PTDH) is a rare complication of liver transplantation (LT) in children. We aimed to present our experience in PTDH, and a possible causative background is discussed. METHODS: Medical records of patients who had undergone diaphragmatic repair following LT between 2015 and 2023 were reviewed. Demographic information, details of primary diseases necessitating LT, transplantation techniques, and clinical findings associated with PTDH were evaluated. RESULTS: There were seven patients with PTDH. Median age at transplantation was 69 (range: 9-200) months. Five patients received a left lateral sector, one patient had a right lobe, and one had a left lobe graft. Time between LT and PTDH was 9 (2-123) months. One patient who was diagnosed in the postoperative 10th year was asymptomatic. Respiratory distress and abdominal pain were the main symptoms among all. All patients underwent laparotomy, and primary repair was performed in six patients, and one patient required mesh repair because of a large defect. Small intestine herniated in most cases. There were two complicated cases with perforation of the stomach and colonic volvulus. There is no recurrence or long-term complications for the median 60 (20-119) month follow-up period. CONCLUSION: PTDH is a rare but serious complication. Majority of symptomatic cases present within the first postoperative year, whereas some late-presenting cases may not be symptomatic. Inadvertent injury to the inferior phrenic vasculatures due to excessive use of cauterization for control of hemostasis may be a plausible explanation in those cases.

Anesthetic considerations in the perioperative management of the patient with Jarcho-Levin Syndrome.

Jarcho-Levin syndrome is an eponym used to describe a spectrum of small thoracic skeletal dysplasias with variable involvement of vertebrae and ribs. Initially considered lethal, it is currently compatible with life in its mildest forms. Bone alterations that lead to a restrictive respiratory pattern, recurrent respiratory infections and particular phenotype, can make perioperative anesthetic management difficult. The proper assessment of the airway is of special interest because it presents predictors of a difficult airway, as well as the prevention, early diagnosis and adequate treatment of respiratory failure. We present the case of a patient with Jarcho-Levin Syndrome who underwent vertebral distraction surgery, with its most notable implications in anesthetic management.

Long-Term Follow-up of Untreated Adult Patients with Spondylothoracic Dysostosis (Jarcho-Levin Syndrome).

BACKGROUND: Spondylothoracic dysostosis (STD), also known as Jarcho-Levin syndrome (JLS), is a rare autosomal recessive disorder affecting the formation of the spine, characterized by a complete bilateral fusion of the ribs at the costovertebral junction, producing a "crablike" appearance of the thorax. Despite being declared a core indication for a V-osteotomy vertical expandable prosthetic titanium rib (VEPTR) expansion thoracoplasty of the posterior thorax, the natural history of STD in untreated subjects remains poorly documented. In this study, we report radiographic and pulmonary function findings and Patient-Reported Outcomes Measurement Information System (PROMIS) and 24-Item Early Onset Scoliosis Questionnaire (EOSQ-24) scores for untreated adult subjects with STD to gain insights into the natural history. METHODS: We identified 11 skeletally mature, untreated subjects with STD. Findings on medical evaluation, demographics, radiographic parameters, pulmonary function, genetic testing results, PROMIS measures, and EOSQ-24 scores were assessed. RESULTS: Five male and 6 female subjects (mean age, 32.3 years [range, 15 to 70 years]) with a confirmed STD diagnosis based on radiographs and genetic testing were evaluated. Mean body mass index (BMI) was 24.4 kg/m 2 (range, 18 to 38.9 kg/m 2 ), and mean thoracic height was 16 cm (range, 12 to 17 cm). Pulmonary function tests (PFTs) showed a mean forced vital capacity (FVC) of 22% of predicted, mean forced expiratory volume in 1 second (FEV1) of 24% of predicted, and FEV1/FVC ratio of 107% of predicted. The mean PROMIS dyspnea score was 40 ± 8 points (range, 27.7 to 52.1 points). The mean total EOSQ-24 score was 77.3 ± 18 points (range, 43.9 to 93.2 points). CONCLUSIONS: Our study characterizes the natural history of STD in untreated subjects. We confirmed the expected restrictive pattern in pulmonary function, but interestingly, our subjects exhibited better EOSQ scores compared with those reported in neuromuscular populations. PFT results and thoracic height did not correspond to PROMIS and EOSQ scores, questioning the use of those parameters as a surgical indication. We therefore suggest that the STD diagnosis as an absolute indication for VEPTR expansion thoracoplasty surgery be reconsidered. LEVEL OF EVIDENCE: Therapeutic Level IV . See Instructions for Authors for a complete description of levels of evidence.

[Diaphragmatic hernia in a female alpaca (Vicugna pacos)]. / Zwerchfellhernie bei einer Alpakastute (Vicugna pacos).

A 3-year-old pregnant female alpaca was noticed due to increased lying and decreased feed intake after shearing. Twelve days after shearing, she aborted an almost completely developed cria. After another two days, the alpaca was recumbent and subsequently died during transport to the clinic. A dissection of the carcass revealed a diaphragmatic hernia. Diaphragmatic hernias have been described in many different species, diagnosis can be made by radiography or ultrasound. Depending on the extent, surgical therapy may be successful.

Repair of diaphragmatic hernias: Retrospective analysis of 70 cases.

BACKGROUND: Congenital and traumatic diaphragmatic hernias (DH) can lead to respiratory and gastrointestinal complications that can be the cause of serious morbidity and mortality. In this study, we aimed to share our experience with the surgical repair of complicated or non-complicated DH. METHODS: Patients who were operated on under emergency or elective conditions with the diagnosis of DH between 2009 and 2023 were analyzed retrospectively. Demographic characteristics, histories, symptoms, etiology of DH, computed tomography find-ings, surgical techniques, and postoperative outcomes of the patients were recorded. RESULTS: The mean age of the cases was 51.5±18.5, and 29 were female and 41 were male. Hernia etiology was found to be con-genital (40%), traumatic (32.8%), spontaneous (14.3%), and iatrogenic (12.8%), respectively. The mean diameter of the defects was 7.3±2.76 cm (range: 3-15 cm), and 84% of the defects were on the left side. Sixty percent of the cases were treated by laparoscopic surgery and 11.4% by laparotomy. The conversion rate from laparoscopic to open was 24.3%. Dual mesh was used in 48% of the pa-tients, and primary suturing was applied in 34%. The postoperative mortality rate was 7.1%. CONCLUSION: DH is an important cause of morbidity and mortality due to abdominal organ strangulation and pulmonary and cardiac complications. When a DH is diagnosed, laparoscopic or open surgery is the treatment that should be preferred.

Incidence and outcomes of post-hepatectomy diaphragmatic hernia: a systematic review.

BACKGROUND: Post-hepatectomy diaphragmatic hernia is the second most common cause of acquired diaphragmatic hernia. This study aims to review the literature on this complication's incidence, treatment and prognosis. METHODS: Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, we systematically searched PubMed for all studies related to acquired diaphragmatic hernias after hepatectomy. RESULTS: We included 28 studies in our final analysis, comprising 11,368 hepatectomies. The incidence of post-hepatectomy diaphragmatic hernia was 0.75% (n = 86). The most frequent type of hepatectomy performed was right hepatectomy (79%, n = 68), and the indications for liver resection were a liver donation for living donor transplantation (n = 40), malignant liver tumors (n = 13), and benign tumors (n = 11). The mean onset between liver resection and the diagnosis of diaphragmatic hernia was 25.7 months (range, 1-72 months), and the hernia was located on the right diaphragm in 77 patients (89.5%). Pain was the most common presenting symptom (n = 52, 60.4%), while six patients were asymptomatic (6.9%). Primary repair by direct suture was the most frequently performed technique (88.3%, n = 76). Six patients experienced recurrence (6.9%), and three died before diaphragmatic hernia repair (3.5%). CONCLUSION: Diaphragmatic hernia is a rare complication occurring mainly after right liver resection. Repair should be performed once detected, given the not-negligible associated mortality in the emergency setting.

Laparoscopic-assisted thoracoscopic repair of latent traumatic diaphragmatic hernia: A case report.

Surgical approaches for traumatic diaphragmatic hernia include transabdominal, transthoracic, and thoracoabdominal. Selection of the optimal approach depends on the timing and organ damage, often minimally invasive approaches with laparoscopy or thoracoscopy are performed. A 47-year-old man with blunt chest trauma was diagnosed with left traumatic diaphragmatic hernia 1 month after the trauma. The prolapsed omentum was detached from the chest wall and around the hernia orifice and returned to the abdominal cavity by coordinated thoracoscopic and laparoscopic manipulations. The 4 × 2 cm herniation in the diaphragm was sutured closed from the thoracic side while preventing re-prolapse of the omentum and abdominal organs from the abdominal side. A combined thoracoscopic and laparoscopic approach can be effective in confirming organ damage, repositioning of prolapsed organs, and safe repair of the diaphragm in latent traumatic diaphragmatic hernia.

Right-Side Acquired Diaphragmatic Hernia in an Adult 15 Years After Living Donor Liver Transplant.

Cases of adult liver transplant recipients with a postoperative right-side acquired diaphragmatic hernia are extremely rare. In this report, we describe an adult case of right-side acquired diaphragmatic hernia 15 years after living donor liver transplant. A 27-year-old woman was diagnosed with pancreatic insulinoma with multiple metastases in the liver. To treat the liver failure, she underwent left lobe living donor liver transplant and distal pancreatectomy with splenectomy 3 years after the transcatheter arterial chemoembolization. As a result of the liver abscesses that reached the diaphragm, the delicate diaphragm was injured, which required repair during the transplant surgery. At the age of 46 years, she developed a cough and intermittent abdominal pain. One month later, she went to another hospital's emergency room with complaints of epigastric pain. The computed tomography scan revealed colon and small intestine prolapse into the right thoracic cavity. She was referred to our hospital and underwent surgery the next day. Two adjacent right diaphragm defects were successfully sutured with nonabsorbable sutures. The patient was discharged on postoperative day 11.

[Acquired diaphragmatic hernia in a child with a related liver transplant]. / Priobretennaya diafragmal'naya gryzha u rebenka s rodstvennoi transplantatsiei pecheni.

Acquired diaphragmatic hernia in children is rare. Even less often, this disease occurs after liver transplantation for biliary atresia. In our case, diaphragmatic hernia was acquired, since the patient underwent repeated chest X-ray examinations including CT prior to liver transplantation. There were no signs of hernia. Clinical manifestations of diaphragmatic hernia were absent throughout 9 months after liver transplantation and manifested acutely with combination of symptoms of respiratory failure and intestinal obstruction. Surgical treatment was performed after emergency consultation with attending doctor.

[Hepatic Hernia through a Diaphragmatic Defect Mimicking Pleural Tumor:Report of a Case].

Hepatic heria in adult without history of trauma or surgery is rare, and is sometimes operated under the diagnosis of lung or diaphragmatic tumor. Here, we report a case of hepatic hernia which had been preoperatively suspected of pleural tumor and surgically treated. At surgery, multiple lesions mimicking ectopic endometriosis were found on the diaphragma and hepatic profrusion was found from one of lesions.

Diaphragmatic hernia after radiofrequency ablation of liver metastases: a case report and review of the literature.

A diaphragmatic hernia is the protrusion of abdominal tissues into the thoracic cavity secondary to a defect in the diaphragm. Reviewing the literature, we found only 44 references to diaphragmatic hernia secondary to percutaneous radiofrequency treatment. The vast majority of these cases were secondary to the treatment of hepatocellular carcinoma in segments V and VIII. Nevertheless, to date, this is the first reported case of diaphragmatic hernia after radiofrequency ablation of a liver metastasis from colorectal cancer. Complications secondary to diaphragmatic hernias are very diverse. The principal risk factor for complications is the contents of the hernia; when small bowel or colon segments protrude in the thoracic cavity, they can become incarcerated. Asymptomatic cases have also been reported in which the diaphragmatic hernia was discovered during follow-up. The pathophysiological mechanism is not totally clear, but it is thought that these diaphragmatic hernias might be caused by locoregional thermal damage. Given that most communications correspond to asymptomatic and/or treated cases, it is likely that the incidence is underestimated. However, due to the advent of percutaneous treatments, this complication might be reported more often in the future. Most cases are treated with primary herniorrhaphy, done with a laparoscopic or open approach at the surgeon's discretion; no evidence supports the use of one approach over the other. Nevertheless, it seems clear that surgery is the only definitive treatment, as well as the treatment of choice if complications develop. However, in asymptomatic patients in whom a diaphragmatic hernia is discovered in follow-up imaging studies, management should probably be guided by the patient's overall condition, taking into account the potential risks of complications (contents, diameter of the opening into the thoracic cavity …).

Hernia diafragmática congénita de diagnóstico tardío / Late presenting congenital diaphragmatic hernia

La hernia diafragmática congénita es un defecto del desarrollo diafragmático que suele diagnosticarse prenatalmente o en el periodo neonatal. Aunque su presentación tardía se asocia a mejor pronóstico, el diagnóstico supone en ocasiones un reto debido a su curso inespecífico. Resulta interesante conocer su existencia ante cuadros clínicos de difícil diagnóstico (AU)

Congenital diaphragmatic hernia is a diaphragmatic developmental defect that usually presents prenatally or in the neonatal period. Although its late presentation is associated with a better prognosis, the diagnosis is sometimes a challenge due to its non-specific course. It is interesting to know its existence in clinical presentations that are difficult to diagnose. (AU)

Diaphragmatic hernia after radiofrequency ablation of liver tumor case report and literature review.

INTRODUCTION: Radiofrequency ablation is an effective management modality for irresectable primary and secondary liver tumors. Some serious complications have been reported including diaphragmatic hernia. Diaphragmatic hernia is the protrusion of abdominal viscera into the thoracic cavity through a diaphragmatic defect and usually classified into congenital and acquired. After RFA, diaphragmatic hernia is a rarely-reported complication. CASE REPORT: A 62-year-old male patient, known to have liver cirrhosis on top of hepatitis C virus, presented to the emergency department with generalized abdominal pain and vomiting four months after having a RFA procedure for a liver tumor in segment VIII. Computed tomography showed diaphragmatic hernia with strangulated terminal ileum in the chest. Emergency laparotomy was performed with resection of an ileal segment and creation of double barrel ileostomy. The patient was discharged in a good condition after tolerating oral intake. CONCLUSION: Radiofrequency ablation is an effective modality for management of the primary and secondary liver tumors. Despite its safety, some complication may happen owing to its thermal effect and the associated patients general condition. Many techniques have been described to decrease its thermal injury. Diaphragmatic hernia is a rare complication after RFA. Its clinical presentation may be confusing and it may occur as early as one month after RFA. Its diagnosis depends mainly on computed tomography. Emergency surgical management is the standard approach.

Pneumopericardium after peritoneopericardial diaphragmatic hernia repair in a dog.

A one-year-old neutered, Goldendoodle presented for evaluation. One week prior to presentation, the surgical closure of a peritoneopericardial diaphragmatic hernia was performed via ventral midline celiotomy. Since the procedure, the dog had been lethargic and anorexic at home. Thoracic radiographs revealed a pneumopericardium. Pericardiocentesis was performed which evacuated approximately 100 mL of air from the pericardial sac. The dog recovered well and began eating in the hospital and resumed normal activity. This is the first reported case of a pneumopericardium post-peritoneopericardial diaphragmatic hernia repair requiring pericardiocentesis. Clinicians should be aware of this rare complication in patients with post-operative peritoneopericardial diaphragmatic hernia.