Non-traumatic left-sided diaphragmatic hernia causing volvulus in an adult

Diaphragmatic hernias can be classified into congenital and acquired types. Most cases of congenital diaphragmatic hernia present early in life with respiratory distress while acquired diaphragmatic hernia usually presents following a history of trauma. Adult presentation of congenital diaphragmatic hernia is a rare finding and can remain asymptomatic for years until the herniated abdominal contents cause significant respiratory or gastrointestinal complications. This is a case report of a 55-year-old male patient presented to the emergency department with acute left-sided chest pain associated with dyspnea and abdominal distension. Chest roentgenogram showed gastric air bubble in the chest cavity. After initial resuscitation, CT thorax, abdomen, and pelvis was done which showed large diaphragmatic hernia with a wide central defect in the diaphragm with entire stomach and distal pancreas along with fat and omentum have been herniated into the thorax with organo-axial rotation of the stomach denoting volvulus and some degree of obstruction. Resultant marked distension of the stomach with air-fluid levels noted. Diagnosis of congenital diaphragmatic hernia can be challenging. Physical examination including auscultation of bowel sounds in the chest offers a diagnostic clue but the mainstay of diagnosis by chest imaging including chest roentgenogram and CT scan of the thorax and abdomen.     

先天性婴幼儿膈膨出的诊断和治疗

目的 探讨先天性婴幼儿膈膨出的病因、临床特点、诊断及治疗方法。方法 对近１０年来收治的１２例先天性婴幼儿膈膨出进行回顾性分析。结果 本组１２例中,行膈肌折叠手术治疗８例,３例为右侧因膨出程度及症状较轻而未手术,１例因合并多发畸形放弃治疗,术后死亡１例,其余效果良好。结论 先天性婴幼儿膈膨出病因不明确,可能与遗传、胎儿宫内感染以及药物有关,低体重和反复呼吸道感染是该症的主要临床特征。早期诊断和手术治     

Health-related quality of life in adults after repair of congenital diaphragmatic defects--a questionnaire study

Background and Aim

Long-term respiratory, gastrointestinal, and vertebral sequelae are common after repair of congenital diaphragmatic defects (CDDs). The aim of this study was to assess the effect of these sequelae on the health-related quality of life (HRQoL) of adult survivors after CDD repair.

Materials and Methods

A questionnaire, including 36-Item Health Survey Form (SF-36), 36-item Gastrointestinal Quality of Life Index (GIQLI), 55-item Psychosocial Survey, 9-item survey for Respiratory Symptoms-Related Quality of Life Index, and a symptoms query, was sent to 94 adult survivors of CDD and to 400 healthy control subjects. One SD lower than the age-adjusted national average in the 36-Item Health Survey Form score for physical or mental health was considered as low HRQoL.

Results

Sixty-nine patients with CDD (72%) and 162 (41%) control subjects returned the questionnaire. The initial presentation was critical in less than 10% of patients with CDD. Forty-five patients with diaphragmatic hernia had primary closure; in 1 patient with diaphragmatic hernia, a patch was used. Twenty-four patients had plication of diaphragmatic eventration. The incidence of gastroesophageal reflux (20% vs 2%), recurrent intestinal obstruction (7% vs 0%), and recurrent abdominal pain (12% vs 2%) was significantly higher in patients with CDD than in control subjects, whereas no difference in the incidence of respiratory, musculoskeletal, or other health problems not associated with CDD was found. Scores in GIQLI, Psychosocial Survey, and Respiratory Symptoms-Related Quality of Life Index did not differ between patients with CDD and control subjects. Health-related quality of life was low in 17 (25%) of 69 patients with CDD, which exceeded 1.5 times the expected value. There was no correlation between the type or severity of the primary defect and HRQoL at the time of the study.

Conclusion

Most adults with repaired CDD have good or satisfactory HRQoL. Congenital diaphragmatic defect-associated symptoms with or without acquired diseases significantly impair HRQoL in one fourth of the patients.     

Sutureless “button and hole” splenopexy

A new technique for fixation of a mobile spleen is described. It offers the advantages of a minimally risky procedure that very effectively keeps a mobile spleen in its bed without foreign materials and is feasible even when the gastrosplenic ligament is absent and the splenic vessels are uncovered. Our “button and hole” sutureless splenopexy was performed in six patients from 1979 to 1995. Three had a gastric volvulus (GV) and an extremely mobile spleen; one of these also had a diaphragmatic eventration. Another three had torsion of the spleen; in one it occurred 9 months after repair of a diaphragmatic hernia. We achieved good results with splenopexy as the sole operation in two cases where a wandering spleen produced a GV. Accepted: 11 February 1997     

Transtalar retrograde drilling of medial osteochondral lesions of the talar dome

Osteochondral lesions of the medial talar dome can cause prolonged ankle disability. Chronic symptomatic lesionshave traditionally been debrided with transarticular approaches using arthrotomy, malleolar osteotomy, or arthroscopy. All of these techniques require removal of cartilage to access the underlying bone. The use of arthroscopy combined with percutaneous retrograde transtalar drilling through the sinus tarsi allows healing of the bone lesion and sparing of intact articular cartilage. Short-term results have shown high patient satisfaction.     

Coexisting left diaphragmatic hernia and esophageal atresia

Coexisting left diaphragmatic hernia (DH) and esophageal atresia, a rare phenomenon, is described in two newborns, one with tracheoesophageal fistula (TEF) and the other without. The coexistence of DH and TEF in the one resulted in uncontrolled gastrointestinal distention with severe respiratory distress. Transabdominal division of the TEF was feasible and was performed concurrently with the diaphragmatic repair. Severe hypoplastic lungs and persistent pulmonary hypertension resulted in early postoperative death in both cases. Offprint requests to: R. Udassin     

The creation and repair of diaphragmatic hernia in fetal lambs: morphology of the type II alveolar cell

A model for creating and repairing diaphragmatic hernia in fetal lambs has been developed. Morphometric studies of the type II alveolar cells were carried out in three groups of term lambs. The upper lobes only were sampled. Morphometric analysis of the 30 type II cells from each lobe showed that while there were no differences between the left upper lobe (LUL) and right upper lobe (RUL) cells in normal lambs, there were significant differences between sides in the experimental groups. In lambs with a nonrepaired diaphragmatic hernia (DH) the type II cells were significantly smaller in the LUL compared with the RUL. In lambs with a repaired DH, the LUL type II cells were significantly larger than those in the RUL. There were some trends when the groups were compared, but in general they did not reach statistical significance. These findings suggest that local factors profoundly influence the development of these cells.     

The whole truth: comparative analysis of diaphragmatic hernia repair using 4-ply vs 8-ply small intestinal submucosa in a growing animal model

Background

Diaphragmatic reconstruction remains a challenging problem. There is limited information concerning the use of small intestinal submucosa (SIS) in congenital diaphragmatic hernia repair. A canine model was used to evaluate the use of a SIS patch in diaphragmatic reconstruction.

Methods

Eleven beagle puppies (1.6-4.2 kg, 8 weeks old) underwent left subcostal laparotomy, central left hemidiaphragm excision (2 × 7 cm, 50% loss), and reconstruction with a 4-ply group I (n = 5) or 8-ply group II (n = 6) SIS patch. Chest radiographs were taken at time of operation and 3 and 6 months postoperatively. Animals were killed at 6 months. Adhesion formation (both pleural and abdominal), gross visual evaluation of the patch, and histology were compared.

Results

In group I (4-ply), 1 animal died at 3 months from patch deterioration accompanied by stomach herniation that resulted in respiratory failure. In the 4 remaining animals, chest radiographs showed no evidence of herniation or eventration. On physical examination, there was no evidence of chest wall deformity. During gross surgical examination, the 4-ply patches showed thinning, multiple defects, and liver herniation in 3 animals. In 1 pup, the patch was thickened, intact, well incorporated at the repair site, and adherent to the liver and spleen. In group II (8-ply), 1 animal died of cardiopulmonary failure in the early postoperative period. In the other 5 animals, chest radiographs showed evidence of eventration in 1. On gross examination the patch adhered to the liver in all 5 surviving animals. In 4, the patches were thickened, viable, but had some shrinkage. One patch pulled away from the native diaphragm laterally; however, no visceral herniation was present. In the 1 animal with eventration, there was no evidence of a patch. Adhesion scores (AS) were graded and determined by the sum of extent (0-4), type (0-4), and tenacity (0-3). Average abdominal AS in group I was 5.6 ± 0.8 vs 10.2 ± 0.2 (P = .079) for group II. Average lung AS was 0.6 ± 0.6 in group I vs 3.8 ± 1.1 (P = .0476) for group II. Histological examination showed group II patches had greater collagen deposition with central calcification and mild inflammation within the residual graft, whereas group I patches were much thinner and were composed of granulation tissue without evidence of residual graft.

Conclusions

These data indicate that 8-ply SIS repair of diaphragmatic defects was superior (80%; 4/5 to 4-ply, 20%; 1/5, success). Organ adherence appears to be necessary for neovascularization of the SIS composite. Eight-ply grafts appear to be more durable and persist for a longer period, which may improve neovascularization. Long-term follow-up to evaluate remodeling characteristics of the patch material is required.     

A neonatal case of thoracoabdominal duplication associated with right congenital diaphragmatic hernia, absent inferior vena cava, and congenital portoazygous shunt: etiopathogenesis and surgical management

We report on a neonatal case of thoracoabdominal duplication associated with a split notochord syndrome and multiple anomalies. A newborn girl had severe dyspnea and was transferred to our neonatal care unit. At laparotomy, the entire small bowel was herniated into the posterior mediastinum through a defect in the right hemidiaphragm. The small bowel mesentery was firmly fixed to the mediastinum such that a large part of the small bowel could not be repositioned into the abdominal cavity. Imaging studies revealed an absent inferior vena cava with an azygous continuation. The superior mesenteric vein joined the splenic vein to form a portoazygous shunt that ran caudally through the mediastinum and drained into the azygous vein. The patient's intrahepatic portal vein was completely absent. To the best of our knowledge, this is the first reported case of a thoracoabdominal duplication associated with a portoazygous shunt. The etiopathogenesis and surgical management of this complicated case are discussed.