Cases reported "Hernia, Diaphragmatic"

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1/25. Ruptured hemidiaphragm after bilateral lung transplantation.

    A case of right hemidiaphragm rupture and abdominal herniation into the thorax occurring during the immediate post-operative course of double-lung transplantation is reported. This complication has not been reported previously. We examine the possible aetiology and suggest that the direct cause could be an increase in intra-abdominal pressure during chest physiotherapy.
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2/25. Ventral hernia with a skin-covered Silastic sheet for newborn infants with a diaphragmatic hernia.

    The persistently high mortality rate for newborn infants with a congenital diaphragmatic hernia, which is symptomatic and treated in the first 24 hours of life, is due to multiple pulmonary and vascular factors. This demands the exclusion of any additional compromising elements. The effects of increased intra-abdominal pressure due to replacement of the intestine into the abdomen can be avoided by the formation of a ventral hernia. A large hernia can be produced best by suturing a sheet of Dacron reinforced Silastic to the fascia of a midline abdominal incision and covering it with skin flaps. This nonreactive prosthesis will remain in place for several months and later can be removed simply. This technique was used successfully in three infants and should improve the survival rate of precariously balanced newborn infants operated upon during the first few hours of life.
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3/25. Unusual Bochdalek hernia in puerperium.

    The authors present an unusual case of a true left-sided posterolateral (Bochdalek) hernia containing stomach, transverse colon and spleen within a hernial sac in a 25-year-old female patient. They presume this voluminous herniation took place due to a rupture of congenitally preformed hernial ostium on account of increased intraabdominal pressure during repeated pregnancy. They describe an effective surgical transabdominal laparoscopic treatment.
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4/25. Treatment of rebound and chronic pulmonary hypertension with oral sildenafil in an infant with congenital diaphragmatic hernia.

    OBJECTIVE: We describe a case of chronic pulmonary hypertension in a 7-wk-old infant with congenital diaphragmatic hernia and an oral teratoma. Our patient was dependent on low-dose inhaled nitric oxide and was still very unstable with systemic right ventricular pressures leading to frequent oxygen desaturations. We administered sildenafil therapy to stabilize the infant with discontinuation of inhaled nitric oxide. We describe successful discontinuation of the inhaled therapy as well as a period of stabilization and improvement with continued sildenafil administration. DESIGN: Case report. SETTING: intensive care nursery in tertiary academic center. PATIENT: A 7-wk-old infant with congenital diaphragmatic hernia who was mechanically ventilated from birth. INTERVENTION: Oral sildenafil 0.3 mg/kg/dose every 12 hrs. Measurements and RESULTS: Right ventricular pressure (from tricuspid valve regurgitant flow) to systemic systolic arterial pressure was measured by echocardiogram. Right ventricular to systemic pressure ratio was marginally improved with the initiation of sildenafil therapy. Inhaled nitric oxide was successfully discontinued, and the patient clinically stabilized temporarily, but he ultimately succumbed to his pulmonary hypertension. CONCLUSION: Sildenafil may be a useful therapy for chronic pulmonary hypertension in congenital diaphragmatic hernia, but further studies of safety and efficacy need to be performed.
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5/25. Right-sided diaphragmatic hernia following group B streptococcal pneumonia and sepsis.

    A case of right-sided diaphragmatic hernia following group B streptococcal pneumonia and sepsis is reported herein. The clinical course was characteristic. The position of the right hemidiaphragm was initially normal. After an antecedent group B streptococcal infection, an abnormal shadow indicating either pneumonia or a pleural effusion on the chest x-ray was recognized and an elevation of the bowel and liver into the right hemithorax gradually appeared. Repair of the hernia was indicated and the postoperative result was excellent. The relationship between a delayed-onset diaphragmatic hernia and a group B streptococcal infection is still unknown. Increased intrathoracic pressure caused by mechanical ventilation coupled with an abnormal lung compliance due to inflammation may have resulted in the delayed herniation. Among various methods for diagnosis applied, chest x-ray and ultrasonography were noninvasive and useful.
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6/25. Augmented venous access in the problematic ECMO patient: a case report.

    extracorporeal membrane oxygenation (ECMO) provides an adjunct to support of neonates with respiratory distress. The rate of venous drainage is the limiting step to the amount of arterial flow that may be generated during ECMO. We present a 3-kg, 37-week gestation female with congenital diaphragmatic hernia (CDH) whose anatomic venous drainage was insufficient to allow total extracorporeal support. The internal jugular vein was of insufficient size to allow placement of a cannula large enough to support adequate bypass flow. We established minimally acceptable flow by simultaneous caudad and cephalad catheterization of the internal vein. We performed a right lower quadrant retroperitoneal dissection and cannulated the common iliac vein when systemic pressures deteriorated at 24 hours with marginal venous drainage. After triple cannulation the bypass flows were normal. The right leg developed mild cyanosis and edema immediately following cannulation, but these changes resolved over 24 hours with elevation. The common iliac vein provides a desirable route for augmentation of venous drainage in the patient with difficult standard, or cephalad, jugular venous access for extracorporeal support.
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7/25. Bilateral congenital diaphragmatic hernia with absent pleura and pericardium.

    BACKGROUND: Bilateral congenital diaphragmatic hernia is a rare form of diaphragmatic hernia. Independently, pericardial defects are an extremely rare phenomenon. In the case presented, we provide the first complete description of an infant with bilateral congenital diaphragmatic hernia with complete agenesis of the pericardium and inferior parietal pleura. CASE: A male infant was born at 38 weeks of gestation with a prenatal diagnosis of left-sided congenital diaphragmatic hernia. After 1 week of aggressive management, the patient was taken to the operating room for repair. Intraoperatively, the patient was found to have absence of the diaphragm bilaterally, no pleura inferiorly, and no pericardium. A biological mesh was used to construct a diaphragm. At 6 months of age, the patient is growing normally, requiring only supplemental oxygen without pressure support. CONCLUSIONS: Embryologically, this anomaly represents complete lack of development of the pleurocardial folds, pleuroperitoneal folds, and transverse septum, which is previously unreported.
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8/25. Right-sided diaphragmatic hernia in infants after liver transplantation.

    liver transplantation is just as successful in infants as in older children, but more challenging. This relates to the low weight of the recipients and to their rapidly deteriorating clinical condition (malnutrition and end-stage liver disease) ( J Pediatr 1990;117:205-210; BMJ 1993;307:825-828; Ann Surg 1996;223:658-664; Transplantation 1997;64:242-248; J Pediatr Surg 1998;33:20-23). In addition, higher rates of diaphragmatic complications have been shown to significantly correlate with a younger age ( Transplantation 2002;73:228-232; Transpl Int 1998;11:281-283; Pediatr Transplant 2000;4:39-44), but diaphragmatic hernia has never been reported as a complication of liver transplantation. In this report, 2 patients who developed diaphragmatic hernia after liver transplantation are presented. The possible role of several contributing factors resulting in diaphragmatic hernia is discussed. These factors include (1) diaphragm thinness related to low weight and malnutrition, (2) direct trauma at operation (dissection and diathermy), (3) increased abdominal pressure after transplantation caused by the use of a slightly oversized liver graft, and (4) the medial positioning of the partial liver graft in the abdomen.
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9/25. Thoracoscopic repair of congenital diaphragmatic hernia by inflation-assisted bowel reduction, in a resuscitated neonate: a better access?

    Elective endoscopic diaphragmatic hernia repairs have been reported. But endoscopic surgery was regarded unsuitable for emergency repair of diaphragmatic hernia in ventilated newborn children in bad general condition. We report a new method for inflation-assisted reduction and thoracoscopic repair of congenital diaphragmatic hernia diaphragmatic in a vitally endangered neonate. From three 2.7 mm to 5 mm accesses warmed low-pressure, low-volume CO2 was inflated into the thorax at 100 ml/min and 2 mm mercury. This allowed spontaneous reduction of the thoracic viscera into the abdomen and diaphragmatic suture with minimal handling. The 65-min procedure was tolerated well without perioperative deterioration. The baby was weaned off the respirator and breast-fed within 2 days, mediastinal shift normalized in 6 days. In suitable infants thoracoscopic repair and inflation-assisted reduction of thoracic contents is a more physiological access to congenital diaphragmatic hernia than laparoscopy or laparotomy.
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10/25. Pathophysiology of congenital diaphragmatic hernia. III: Exogenous surfactant therapy for the high-risk neonate with CDH.

    Exogenous surfactant therapy (EST) in surfactant-deficient premature infants has been shown to improve lung compliance, decrease morbidity, and improve survival. Reports have demonstrated that newborns with congenital diaphragmatic hernia (CDH) have lung compliance, pressure-volume curves, and hyaline membrane formation resembling those changes seen in surfactant deficient premature newborns. We hypothesize that EST may also benefit infants with CDH. All high risk cases of prenatally diagnosed CDH at Children's Hospital of Buffalo from November 1988 to February 1991 were prospectively evaluated for EST. In those families who chose to participate, the surfactant preparation, Infasurf (100 mg/kg), was instilled into the newborn's lungs prior to the first breath. The remainder of the perinatal, neonatal, and surgical care was performed in a routine manner. Three high-risk prenatally diagnosed newborns with left CDH were treated with EST. All showed signs of decreased pulmonary compliance, but could still be adequately oxygenated and ventilated. Surgical correction was performed after stabilization and all required patch closures. Two of the three infants suffered no life-threatening episodes of pulmonary hypertension and all survived. These infants had many known indicators for poor outcome in CDH with an expected survival of less than 20%. We believe that EST in these neonates with CDH contributed to their survival with minimum morbidity. These results suggest that surfactant replacement for the high-risk neonate with CDH warrants further consideration and a randomized clinical trial is being planned.
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