Intestinal complications are common in patients with acquired diaphragmatic hernia after pediatric living donor liver transplantation.

BACKGROUND: Acquired diaphragmatic hernia (DH) following liver transplantation (LT) is usually considered a surgical emergency. Interplay of contributing elements determines its occurrence but, in children, LT with partial liver grafts seems to be the most important causative factor. METHODS: This retrospective study describes the clinical scenario and outcomes of 11 patients with acquired DH following LDLT. RESULTS: During the study period, 1109 primary pediatric LDLT were performed (0.8% DH). The median age and BW of the recipients with DH at transplantation were 17 months and 11.1 kg, respectively; 63.7% of the cases had a weight/age Z-score of less than -2 at transplantation. The median interval between transplantation and diagnosis of DH was 114 days (32-538 days). A total of 6 (54.5%) of the patients had bowel obstruction due to bowel migration into the hemithorax. Ten defects were right-sided. Three patients required enterectomy and enterorrhaphy. Two patients required a new bilioenteric anastomosis, and one of them had complete necrosis of the Roux-in-Y limb. The patient with left-side DH presented gastroesophageal perforation. CONCLUSION: Most defects necessitate primary closure as the first treatment, and recurrence is rare. The associated problems encountered, especially related to intestinal complications, can determine increased morbidity following DH repair. Early diagnosis and intervention are required for achieving better outcomes.

Vólvulo gástrico por hernia diafragmática crónica / Gastric volvulus due to chronic diaphragmatic hernia

Objetivo: Presentar un caso de vólvulo gástrico obstructivo. Caso clínico: Presentamos el caso de una paciente que inició con un episodio de obstrucción intestinal por una volvulación gástrica. El antecedente de otro episodio antiguo y la radiología de tórax alertaron de una posible hernia diafragmática crónica. La buena respuesta al tratamiento médico inicial permitió un estudio completo y una cirugía programada mediante abordaje laparoscópico.

Aim: To present a patient with gastric volvulus. Case report: A case of a female patient who started with an episode of intestinal obstruction due to gastric volvulus. The history of a former episode and chest radiology alerted us to a possible chronic diaphragmatic hernia. The patient's good response to initial medical treatment allowed a complete study and laparoscopic intervention.

Acute non-traumatic gastrothorax: presentation of a case with chest pain and atypical radiologic findings.

A previously well 71-year-old woman presented to the Emergency Department with acute-onset left-sided chest pain. She was haemodynamically stable with unremarkable systemic examination. Her electrocardiogram and troponin were within normal limits and her chest radiograph showed a raised left hemi-diaphragm. Two hours after admission, this woman became acutely breathless, and suffered a pulseless electrical activity cardiac arrest. After cardiopulmonary resuscitation, there was a return of spontaneous circulation and regained consciousness. A repeat clinical assessment revealed a new left-sided dullness to percussion with contralateral percussive resonance on respiratory examination. CXR revealed a left pan-hemi-thoracic opacity whilst better definition using CT-pulmonary angiography (CTPA) indicated an acute tension gastrothorax secondary to a large left-sided diaphragmatic hernia. Nasogastric (NG) tube insertion was used to decompress the stomach and the patient underwent uncomplicated emergency laparoscopic hernia reduction. She remained well at 1-year follow-up.

Una señora, previamente sana, de 71 años de edad acudió al departamento de emergencia con dolor agudo en el lado izquierdo del pecho. El examen físico no reveló hallazgos importantes y estaba hemodinámicamente estable. El electrocardiograma y la troponina estaban dentro de los límites normales, la radiografía de tórax mostró un diafragma elevado en el lado izquierdo. Dos horas después de ser admitida, esta señora empezó a respirar con dificultad y sufrió un paro cardíaco con actividad eléctrica sin pulso. Después de la reanimación cardiopulmonar, se evidenció el retorno de la circulación espontánea y la paciente recuperó la conciencia. Una segunda evaluación clínica del sistema respiratorio reveló a la percusión, disminución de la resonancia del lado izquierdo del tórax con hiperresonancia contralateral. La radiografía de tórax reveló una opacidad completa en el lado izquierdo del tórax y se obtuvo una mayor definición utilizando la angiografía pulmonar por tomografía computarizada, revelando un gastrotórax a tensión agudo causado por una hernia diafragmática. Se utilizó una sonda nasogástrica para descomprimir el estómago. La paciente se sometió a cirugía laparoscópica de emergencia para reducir la hernia sin complicaciones. Ella permaneció saludable durante un año de seguimiento.

Anaesthesia for infant with Jarcho Levin syndrome: case report / Anestesia em criança com síndrome de Jarcho-Levin: relato de caso

ABSTRACTJarcho Levin syndrome is a rare disorder. There are various vertebral and costal anomalies. Severe deformities and abnormal fusion of ribs and vertebrae cause respiratory insufficiency and pneumonia. We present anaesthesia in a patient with Jarcho Levin syndrome for vesicoureteral reflux.

RESUMOA síndrome de Jarcho-Levin é um distúrbio raro que apresenta várias anomalias vertebrais e costais. Deformidades graves e fusões anormais das costelas e vértebras causam insuficiência respiratória e pneumonia. Apresentamos um caso de anestesia em paciente com síndrome de Jarcho-Levin para refluxo vesicuretral.

Anaesthesia for infant with Jarcho Levin syndrome: case report.

Jarcho Levin syndrome is a rare disorder. There are various vertebral and costal anomalies. Severe deformities and abnormal fusion of ribs and vertebrae cause respiratory insufficiency and pneumonia. We present anaesthesia in a patient with Jarcho Levin syndrome for vesicoureteral reflux.

Vasopressin improves hemodynamic status in infants with congenital diaphragmatic hernia.

OBJECTIVE: To assess the ability of vasopressin to stabilize hemodynamics in infants with systemic hypotension secondary to congenital diaphragmatic hernia (CDH). STUDY DESIGN: A retrospective chart review was performed to identify 13 patients with CDH treated with vasopressin for refractory hypotension to assess the effect of vasopressin on pulmonary and systemic hemodynamics and gas exchange in this setting. Data collected included demographics, respiratory support, inotropic agents, pulmonary and systemic hemodynamics, urine output, and serum and urine sodium levels during vasopressin therapy. RESULTS: Vasopressin therapy increased mean arterial pressure and decreased pulmonary/systemic pressure ratio, heart rate, and fraction of inspired oxygen. In 6 of 13 patients, extracorporeal membrane oxygenation therapy was no longer indicated after treatment with vasopressin. Improvement in left ventricular function and oxygenation index after vasopressin initiation was associated with a decreased need for extracorporeal membrane oxygenation therapy. Prolonged vasopressin treatment was associated with hyponatremia, increased urine output, and increased urine sodium. CONCLUSIONS: Vasopressin stabilized systemic hemodynamics without adverse effects on pulmonary hemodynamics in a subset of infants with CDH. Our results suggest a potential role for vasopressin therapy in patients with CDH with catecholamine-resistant refractory hypotension.

Oclusión intestinal por hernia de Bochdalek / Intestinal occlusion caused by Bochdalek hernia

La hernia de Bochdalek es un defecto congénito de la región posterolateral del diafragma, usualmente del lado izquierdo, se presenta en 1 de cada 2 000-5 000 nacidos vivos, su diagnóstico en el momento del nacimiento es relativamente fácil, no así cuando este debe realizarse después del período neonatal, pues puede conllevar a evaluaciones erróneas desde el punto de vista clínico y radiológico. La presentación tardía de esta entidad no es frecuente, por lo que nos proponemos dar a conocer un caso de oclusión intestinal por hernia diafragmática de presentación tardía y revisar la literatura al respecto. Se presenta un lactante de 5 meses producto de un tercer embarazo, parto distócico por presentación pelviana, oligoamnios, Apgar 9-9, antecedentes de salud hasta el cuarto mes en que se le introduce leche de chiva y comienza con regurgitaciones bastante frecuentes. Se le realiza diagnóstico a los 5 meses de oclusión intestinal por hernia diafragmática congénita. Se interviene quirúrgicamente sin complicaciones posoperatorias, es dado de alta a los 7 días, y seguido por consulta externa, con evolución satisfactoria. La hernia diafragmática congénita, cuando se presenta después del periodo neonatal, puede diagnosticarse incidentalmente en un estudio radiológico, o por complicaciones secundarias al paso de las vísceras abdominales al tórax

Bochdalek hernia is a congenital defect of the posterolateral region of the diaphragm, usually located on the left side. It appears in one per 2000 to 5000 live births; the diagnosis at birth is relatively easy but after the neonatal period, it can lead to erroneous assessments from the clinical and radiological viewpoints. The late-presenting hernia is not frequent, so we intended to present a case of intestinal occlusion caused by late-presenting diaphragmatic hernia and to make a literature review on this topic. Here is the case of a 5 months-old infant born from a third pregnancy, which was dystotic as a result of pelvic presentation and with oligohydramnios. He had an Apgar score of 9.9 and a history of health problems until his fourth month of life when he began to be fed with goat's milk and it appeared pretty frequent regurgitation. He was diagnosed at 5 months of life with intestinal occlusion due to congenital diaphragmatic hernia. He was operated on, no complications were observed and then he was discharged from the hospital after 7 days and followed-up at outpatient service, with satisfactory recovery. The congenital diaphragmatic hernia that occurs after the neonatal period can be incidentally diagnosed in a radiological study or because of secondary complications when abdominal viscerae passed into the thorax

Hernia diafragmática congénita atascada / Stuck congenital diaphragmatic hernia

La hernia diafragmática congénita constituye uno de los retos pendientes dentro de las enfermedades quirúrgicas del recién nacido. Se presenta un caso muy inusual, en el cual se diagnosticó una hernia diafragmática derecha atascada, con compromiso de gran parte del intestino. Se describen los medios diagnósticos utilizados, el procedimiento quirúrgico realizado y las complicaciones presentadas. La hernia diafragmática congénita atascada es una complicación potencialmente letal, por el amplio compromiso vascular que produce en los órganos abdominales y que conlleva a la necrosis de estos si no se diagnostica precozmente. Por ello, debe ser considerada en el diagnóstico diferencial de la insuficiencia respiratoria en los recién nacidos y lactantes pequeños

Congenital diaphragmatic hernia is one of the pending challenges in dealing with the surgical diseases of the newborn. This was a very unusual case of a right stuck diaphragmatic hernia that affected a large part of the intestine. The used diagnostic means, the surgical procedure and the observed complications were described. Stuck congenital diaphragmatic hernia is a potentially lethal complication because of the wide vascular effect on the abdominal organs, leading to their necrosis if this problem is not early diagnosed. Therefore, it must be taken into account in the differential diagnosis of the respiratory failure in newborns and small infants

Hernia diafragmática complicada con rotura de víscera hueca dentro de la cavidad torácica / Complicated diaphragmatic hernia with hollow viscus rupture in thoracic cavity

Introduction: diaphragmatic hernias (DH) are a rare condition in adult patients. Most of the cases are asymptomatic findings in imaging studies. Rarely do they present as complicated cases. Clinical cases: we report two clinical cases of complicated DH, one of Bochdalek and another with history of a penetrating thoracic trauma two years before, both with hollow viscus rupture in the thoracic cavity.

Introducción: las hernias diafragmáticas (HD) son una patología relativamente poco frecuente en adultos. La gran mayoría son hallazgos asintomáticos en exámenes de imágenes, y en ocasiones debutan con complicaciones de éstas. Casos clínicos: se presentan dos casos clínicos de HD, una de Bochdalek y otra con antecedente de trauma penetrante torácico hace 2 años, ambas complicadas con rotura de víscera hueca en la cavidad torácica.

Survival of newborn infants with severe respiratory failure before and after establishing an extracorporeal membrane oxygenation program.

BACKGROUND: Severe hypoxic respiratory failure is a leading cause of neonatal mortality in Chile. Extracorporeal membrane oxygenation improves survival in neonates with hypoxic respiratory failure. OBJECTIVE: To determine the impact of the establishment of a Neonatal Extracorporeal Membrane Oxygenation Program on the outcome of newborns with severe hypoxic respiratory failure in a developing country. DESIGN/PATIENTS: Data of newborns (birthweight > 2,000 g and gestational age ≥ 35 wk) with hypoxic respiratory failure and oxygenation index greater than 25 were compared before and after extracorporeal membrane oxygenation was available. Extracorporeal membrane oxygenation was initiated in infants with refractory hypoxic respiratory failure who failed to respond to inhaled nitric oxide/high-frequency oscillatory ventilation. MAIN RESULTS: Data from 259 infants were analyzed; 100 born in the pre-extracorporeal membrane oxygenation period and 159 born after the extracorporeal membrane oxygenation program was established. Patients were similar in terms of risk factors for death for both periods except for a higher oxygenation index and a greater proportion of outborn infants during the extracorporeal membrane oxygenation period. Survival significantly increased from 72% before extracorporeal membrane oxygenation to 89% during the extracorporeal membrane oxygenation period (p < 0.01). During the extracorporeal membrane oxygenation period, 98 of 159 patients (62%) with hypoxic respiratory failure were rescued using inhaled nitric oxide/high-frequency oscillatory ventilation, whereas 61 (38%) did not improve; 52 of these 61 neonates were placed on extracorporeal membrane oxygenation. Extracorporeal membrane oxygenation survival rate to discharge was 85%. After adjusting for potential confounders, the severity of the pretreatment oxygenation index, a late arrival to the referral center, the presence of a pneumothorax, and the diagnosis of a diaphragmatic hernia were significantly associated with the need for extracorporeal membrane oxygenation or death. CONCLUSIONS: The establishment of an extracorporeal membrane oxygenation program was associated with a significant increase in the survival of newborns more than or equal to 35 weeks old with severe hypoxic respiratory failure.

Oclusión intestinal por hernia diafragmática derecha encarcelada / Intestinal occlusion for incarcerate left diaphragmatic hernia

La hernia de Morgagni es un defecto congénito de la cara anteromedial del diafragma, que se presenta de forma infrecuente en el adulto. En la mayor parte de los casos su diagnóstico es un hallazgo incidental, y por lo general, los pacientes se encuentran asintomáticos. Se diagnostican radiológicamente y posteriormente se indica la cirugía por el riesgo de estrangulación. Se presentó un caso clínico de una paciente de 78 años, con previo diagnóstico de hernia diafragmática derecha mediante técnicas imagenológicas, que comenzó con manifestaciones de cuadro oclusivo. Fue operada de urgencia por vía transabdominal, se hizo la reducción del saco herniario donde se encontraba epiplón, colon transverso y parte del estómago, y se reparó el defecto diafragmático. El propósito de este trabajo fue reportar un caso reciente de oclusión intestinal causada por hernia de Morgagni encarcelada, su diagnóstico y abordaje quirúrgico.

The Morgagni hernia is a congenital defect of the diaphragm anteromedial side, infrequently present in adults. In most of the cases its diagnosis is an incidental finding, and in general the patients are asymptomatic. They are diagnosed radiologically, and once diagnosed the surgery is indicated to avoid the risk of strangulation. We presented the clinical case of a 78-years-old female patient with a previous diagnosis of left diaphragmatic hernia obtained using imaging techniques, who began with manifestations of an occlusive picture. She was immediately operated by transabdominal way, reducing the hernia sac containing mesentery, transversal colon and part of the stomach, and the diaphragmatic defect was repaired. The purpose of this work was reporting a recent case of intestinal occlusion caused by incarcerated Morgagni hernia, its diagnosis and surgical management.

Outcomes of congenital diaphragmatic hernia in the modern era of management.

OBJECTIVE: To identify clinical factors associated with pulmonary hypertension (PH) and mortality in patients with congenital diaphragmatic hernia (CDH). STUDY DESIGN: A prospective cohort of neonates with a diaphragm defect identified at 1 of 7 collaborating medical centers was studied. Echocardiograms were performed at 1 month and 3 months of age and analyzed at a central core by 2 cardiologists independently. Degree of PH and survival were tested for association with clinical variables using Fischer exact test, χ(2), and regression analysis. RESULTS: Two hundred twenty patients met inclusion criteria. Worse PH measured at 1 month of life was associated with higher mortality. Other factors associated with mortality were need for extracorporeal membrane oxygenation, patients inborn at the treating center, and patients with a prenatal diagnosis of CDH. Interestingly, patients with right sided CDH did not have worse outcomes. CONCLUSIONS: Severity of PH is associated with mortality in CDH. Other factors associated with mortality were birth weight, gestational age at birth, inborn status, and need for extracorporeal membrane oxygenation.

Clinical characteristics and outcomes of patients with cardiac defects and congenital diaphragmatic hernia.

OBJECTIVE: To evaluate the impact of associated heart defects on outcomes to discharge, and identify factors affecting survival of all infants born with congenital diaphragmatic hernia (CDH) in last decade using Congenital Diaphragmatic Hernia Study Group data. STUDY DESIGN: This was a retrospective review of all infants with CDH enrolled in Congenital Diaphragmatic Hernia Study Group database from January 2000 to December 2010. The study cohort was divided into 3 groups (GRP): GRP 1, CDH with major heart defects; GRP 2, CDH with minor heart defects; and GRP 3, CDH with no reported heart defects. RESULTS: The 4268 enrolled infants included 345 (8%) in GRP 1, 412 (10%) in GRP 2, and 3511 (82%) in GRP 3. Survival was significantly lower in GRP 1 compared with GRP 2 and GRP 3 (36% vs 73%). In GRP 1, the most common defects were left heart obstructive lesions (34%). Survival was lowest in infants with transposition of great arteries (0%) and single ventricle physiology (16%). There was no change in survival rate for any group between 2000-2005 and 2006-2010. In GRP 1, factors that predicted lower survival were birth weight <2.5 kg, associated noncardiac anomalies, single ventricle physiology, no sildenafil therapy, no CDH repair, and no cardiac repair. CONCLUSION: Survival is significantly lower in patients with CDH and major heart defects compared with patients with minor or no heart defects. Outcomes of newborns with CDH and major heart defects have not improved over the last decade.

Effect of nitrofen in the final stages of development of the diaphragm muscle in rats / Efeito do nitrofen na fase final do desenvolvimento da musculatura do diafragma em ratos

PURPOSE: To evaluate the expression of myosin in muscle fibers of the diaphragm in experimental congenital diaphragmatic hernia (CDH). METHODS: Fetuses of pregnant rats were divided into four groups: External Control (EC), composed of non-manipulated rats; Nitrofen, composed of pregnant rats that received 100 mg of nitrofen (2,4-dichloro-4'nitrodiphenyl ether) diluted in olive oil on gestational day (GD) 9.5, whose fetuses developed CDH (N+) or not (N-), and Olive Oil Placebo (OO), composed of pregnant rats that received the oil on the same GD. The fetuses were collected on GD 18.5, 19.5, 20.5 and 21.5 (term = 22 days). We obtained body weight (BW) and photographed the diaphragm area (DA), hernia area (HA) and subsequent calculated the HA/DA ratio in N+ group. Samples of Diaphragm muscle were processed for histological staining with H/E and immunohistochemistry (IHQ) for myosin.} RESULTS: The fetuses of N- and N+ groups had decreased BW and DA compared to EC and OO groups (p <0.001). HA was decreased on GD 18.5 compared to 21.5 (p <0.001) and the HA/DA ratio showed no difference. IHQ showed decreased expression of myosin in nitrofen groups. CONCLUSION: CDH induced by nitrofen model contributes to the understanding of muscularization in the formation of the diaphragm where the myosin expression is decreased.(AU)

OBJETIVO: Avaliar a expressão da miosina na muscularização do diafragma na hérnia diafragmática congênita (CDH) experimental. MÉTODOS: Fetos de ratas foram divididos em quatro grupos: Controle Externo (EC), composto de ratas não manipuladas; Nitrofen, composto de ratas que receberam 100 mg de nitrofen (2,4-dicloro-4'nitrodifenil éter) diluído no azeite no dia de gestação (GD) 9.5, cujos fetos desenvolveram CDH (N+) ou não (N-) e Placebo óleo de oliva (OO), composto de ratas que ingeriram apenas óleo no mesmo GD. Os fetos foram coletados com 18,5, 19,5, 20,5 e 21,5 GD (termo = 22 dias). Foi obtido o peso corporal (BW) e tiradas fotografias da área do diafragma (DA), da hérnia (HA) e calculada a relação HA/DA no grupo N+. Amostras de diafragmas foram processadas histologicamente para coloração com H/E e imunohistoquímica. RESULTADOS: Os fetos dos grupos N- e N+ tiveram BW e DA diminuídos em relação aos grupos EC e OO (p<0.001). Só houve diferença na HA entre os GD 18.5 e 21.5 (p<0.001) e a relação HA/DA não mostrou diferença entre os grupos. A imunohistoquímica mostrou menor expressão de miosina nos grupos que receberam nitrofen. CONCLUSÃO: O modelo de CDH induzida por nitrofen contribui para entender a muscularização na formação do diafragma onde a expressão da miosina está diminuída.(AU)

Vertical expandable prosthetic titanium rib as treatment of thoracic insufficiency syndrome in spondylocostal dysplasia.

BACKGROUND: Spondylocostal dysplasia (SCD) constitutes a heterogeneous patient group with multiple vertebral formations and segmentation defects of the entire spine, with asymmetric rib malformations. Respiratory failure has been reported in spondylocostal dysplasia secondary to thoracic insufficiency syndrome. The vertical expandable prosthetic titanium rib (VEPTR) reconstructs the chest wall to address the thoracic insufficiency seen in this patient population. The purpose of this study is to evaluate spinal deformity correction and respiratory function outcomes in a spondylocostal dysplasia population treated with VEPTR. METHODS: A cohort of 20 patients with spondylocostal dysplasia and 2-year follow-up were evaluated from a multicenter IDE study of 214 patients who had surgery with the VEPTR device. Data collected included gender, nonskeletal malformations, age at surgery, number of procedures, estimated blood loss, length of stay, and surgical time. Clinical and radiographic parameters were collected, and respiratory function was assessed. RESULTS: In 14 of 20 patients (70%), spinal deformity was controlled evidenced by a decrease of the initial Cobb coronal angle at last follow-up. Fourteen patients (70%) maintained their oxygen level throughout treatment. At preoperative and last evaluation, assisted ventilation rating (AVR) scores showed that 5 patients improved their level of ventilation and 14 patients maintained their AVR level at room air. One patient decreased his level from supplemental oxygen to night ventilation. Mean thoracic spinal length (growth) by year was 0.82 cm. No mortality occurred in this group of patients. CONCLUSIONS: VEPTR implantation in SCD allows continued thoracic spine growth while controlling progressive spine deformity. The improved AVR ratings after surgery suggest a beneficial effect on the natural history of TIS in this population. Mortality and complication rate seem acceptable in this high-risk population of SCD patients. LEVEL OF EVIDENCE: Therapeutic study, Level IV, (case series, no comparison group).