A case of diaphragmatic hernia incarceration after a heart transplant operation

We report a case of a diaphragmatic hernia after a heart transplant operation. A 43-year-old woman, who underwent orthotropic heart transplantation for hypertrophic cadiomyopathy two year earlier, presented with vomiting and epigastric pain. A computed tomography scan showed that the stomach and transverse colon were dislocated in the left thoracic cavity. We diagnosed left diaphragmatic hernia incarceration and performed laparoscopic repair of the diaphragmatic hernia. A 12 × 8 cm diaphragmatic defect was found intraoperatively on the ventrolateral aspect of the left diaphragm, and the stomach with volvulus had herniated into the thorax through the defect. The hernia was considered to be iatrogenic. The diaphragmatic defect was large, and the diaphragm was thinning. We closed the defect by mesh repair. Laparoscopic mesh repair of the diaphragmatic hernia could be performed safely and with minimal invasiveness.     

Bilateral Ulnar Agenesis: Case Report and Review of the Literature

Bilateral ulnar agenesis is a rare abnormality. A total of 36 cases are analyzed: 35 of these are documented in the literature and 1 stillborn male is presented in this study. Most patients had one of the three conditions: Al-Awadi/Raas-Rothschild syndrome, syndrome of ulnar aplasia with split hand/split foot deformity, or the Brachmann—de Lange syndrome. Fifty percent of all cases with bilateral ulnar agenesis were associated with lower limb defects and these cases, for the most part, also belonged to the aforementioned syndromes. Nonskeletal, internal organ malformations were identified in 34% of all patients. Nine patients presented with isolated bilateral ulnar agenesis. The Al-Awadi/Raas-Rothschild syndrome and the split hand/split foot deformity are heritable disorders. There was no evidence for genetic etiology in most of the other cases. Bilateral ulnar agenesis in our fetus was part of the Brachmann—de Lange syndrome with associated cardiac defect, diaphragmatic hernia, and umbilical artery agenesis.     

创伤性膈疝并发多器官功能障碍的诊治

目的　探讨急性创伤性膈疝的发病机理 ,临床特征和处理方式。方法　回顾性分析2 6例急性创伤性膈疝的临床资料。结果　患者均为多发伤 ,有不同程度的呼吸困难 ,均行手术探查 ,膈肌修补。 2例术中死亡。 14例术后出现多器官功能障碍 ,其中 3例术后死于多器官衰竭。本组 2 1例存活。结论　外伤性膈疝早期诊断 ,及时手术 ,防治并发症可提高抢救成功率。     

Blood gas and ventilatory parameters in predicting survival in congenital diaphragmatic hernia

Blood gas analysis can be used to reliably predict outcome in infants with congenital diaphragmatic hernia (CDH) both before and after surgical repair, providing these values are indexed to some measurement of alveolar ventilation. Until recently there has been difficulty in interpreting some of the published data because of differing sampling sites and the fact that ventilatory parameters, which have major influences on all blood gas values in this anomaly, were not included. Application of this information enables us to identify infants with similar degrees of severity of CDH in order to evaluate the efficacy of novel forms of therapy and to determine whether they represent a genuine advance in management where more conventional forms of treatment have failed.     

Monosomy and trisomy of 15q24→qter in a family with a translocation t(6;15)(P25;q24)

A child with multiple anomalies, including growth retardation, a left-sided diaphragmatic hernia with lung hypoplasia, and cerebral malformations is described. Cytogenetic investigation demonstrated a deletion of the distal part of one chromosome 15, del(15)(q24qter), an aberration not previously described. Family studies revealed that the mother had a balanced translocation, t(6;15)(p25;q24). Two of her subsequent pregnancies resulted in abortions after prenatal diagnosis: one fetus was trisomic for 15q24→qter, while the other had monosomy 15q24→qter and a left-sided diaphragmatic hernia similar to the first child.     

"Balanced" thoracic drainage is the method of choice to control intrathoracic pressure following repair of diaphragmatic hernia

Respiratory failure from pulmonary hypoplasia continues to be the major cause of death in newborn infants with diaphragmatic hernia. Recent investigations have suggested that postnatally induced pulmonary injury can result from excessive positive or negative intrathoracic pressure and contribute to the respiratory deterioration. Therefore, the method of thoracic drainage on the side of the diaphragmatic hernia is critical in controlling and maintaining normal intrathoracic pressure in both intrathoracic spaces. No chest tube or an ipsilateral chest tube connected to water seal, can result in either excessive negative or positive intrathoracic pressure and, therefore, both methods should be avoided. Recently, we employed a "balanced" intrathoracic drainage system which maintains the ipsilateral intrathoracic pressure within the normal physiologic range of +2 to -8 cm H2O regardless of the degree of pulmonary hypoplasia, presence of an ipsilateral pulmonary air leak, straining by the infant, or mechanical ventilation. This system is simple, requires no suction apparatus, and is easily assembled with equipment readily available within the hospital. This technique has been utilized in 18 newborn infants with diaphragmatic hernia and pulmonary hypoplasia. There have been no complications which specifically could be related to the balanced drainage system.     

Familial congenital diaphragmatic hernia: Prenatal diagnostic approach and analysis of twelve families

Congenital diaphragmatic hernia is generally recognized as a sporadic malformation with little or no risk of recurrence. A family with three affected individuals in two generations is presented. In addition, new prenatal diagnostic techniques including ultrasonography and amniography are discussed. A comparison of associated physical characteristics in isolated versus twelve familial cases of diaphragmatic hernia is presented. In the familial group, there was a higher incidence of affected males (M:F ratio = 2.1 versus 0.67), a higher incidence of bilateral defects (20% versus 3%) and a lower incidence of additional life-threatening malforamtions 3.6% versus 47%). Analysis of available pedigree data favors multifactorial inheritance with a high male: female sex ratio as the most probable mode of transmission.     

大鼠膈腹膜间皮孔的电镜观察

用扫描电镜和透射电镜观察了正常大鼠膈腹膜间皮，并观察了腹膜腔内注射中国墨汁和兔血液后大鼠膈腹膜间皮的变化以及腹膜腔和间皮下毛细淋巴管的关系。