The natural history of bronchial atresia. Serial observations of a case from birth to operative correction.

A 10-year-old child presented with a history of intermittent respiratory symptoms since birth. Progressive shift of the mediastinum away from a hyperexpanded left upper lobe to the right side was evident on serial chest films, since birth. Bronchograms demonstrated atresia of the bronchus to the hyperexpanded segment. Xenon washout demonstrated prolonged half-time in the left upper lobe resulting from collateral ventilatory channels. Resection of the apical posterior segment of the left upper lobe was performed without complication. Bronchial atresia with collateral ventilation caused "lobar emphysema" in this patient.

Clamp/repair: a safe technique for treatment of blunt injury to the descending thoracic aorta.

Debate exists with regard to the use of pump bypass, shunt bypass, or clamp/repair techniques in treating injuries to the descending thoracic aorta. The objective in using any of these techniques is to minimize the complications of paraplegia and renal failure, while achieving the lowest possible mortality. During an eighteen-year period, 45 patients were seen with acute blunt injury to the descending thoracic aorta. The shunt bypass method of repair was used in 1; pump bypass in 8; and clamp/repair in 23. There were desperate unsuccessful attempts to resuscitate and control hemorrhage in 13 patients, 1 of whom was placed on portable pump bypass. Thirty-two patients survived resuscitation and operation, and 26 were long-term survivors. Among surviving patients with permanent paraplegia, 2 underwent pump bypass and 1, the clamp/repair technique. Four other patients were seen with paraplegia or paresis and had reversal of the paralysis. The clamp/repair technique was used in these patients with clamp times ranging from 35 to 62 minutes (mean, 47.4 +/- 13.3 minutes). Renal failure did not occur in any patient, despite clamp times of up to 62 minutes (mean, 37.5 minutes). Excluding patients seen in a moribund condition, mortality most often was secondary to neurological or multisystem injury. Debate continues concerning intraoperative management of this highly lethal vascular injury. The data presented here support the historical composite experience that clamp/repair is a safe and efficacious technique that minimizes paraplegia and mortality.

Surgical management of lung bud anomalies: lobar emphysema, bronchogenic cyst, cystic adenomatoid malformation, and intralobar pulmonary sequestration.

Recently we saw 9 infants with life-threatening respiratory distress. Four patients had bronchogenic cyst, 2 had cystic adenomatoid malformation, and 9 had congenital lobar emphysema. Another group of 14 older children had recurrent infection and hemodynamic abnormalities, which responded to operative intervention. Each child required an appropriate resection following definitive diagnosis. These lesions represent a spectrum of closely related anomalies that arise during an early stage of embryonic lung bud maturation. Bronchoscopy is rarely useful, but special roentgenographic studies, including perfusion scans and arteriography, are usually diagnostic. Our operative experience is used to emphasize the urgency of precise diagnosis and surgical management of this poorly recognized clinical syndrome.

Bacteremia after esophageal dilation: a clinical and experimental study.

Patients with esophageal stricture caused by caustic ingestion, reflux esophagitis, or esophageal anastomosis often require repeated dilation. These patients frequently have a short febrile course after dilation. After development of brain abscess following esophageal dilation in 1 patient, positive blood cultures were obtained in 4 patients immediately following esophageal dilation. Caustic strictures were produced in cats and esophageal dilations performed. Blood cultures were positive at one minute after dilation in 6 cats and at five minutes in 2 of those cats. The organism responsible in all clinical and three of four experimental examples was Staphylococcus aureus. It is suggested on the basis of this clinical and experimental data that patients undergoing esophageal dilation should have prophylactic coverage if they are immunosuppressed, if endocarditis prophylaxis is necessary, if they are infants, if they are diabetic, or if they had severe bacteremia following dilation.

The pathophysiology of neurofibromatosis. II. Angiosarcoma as a complication.

A patient with Von Recklinghausen neurofibromatosis and an angiosarcoma (which developed subsequent to two separate neurofibrosarcomas ) is described, and the association of neurofibromatosis and angiosarcoma is reviewed. The data showed that angiosarcoma is not coincidental with neurofibromatosis, and there is a need to focus investigative efforts on the vascular aspects of this disease.

Effect of pulmonary artery ligation on the developing fetal lung.

Ligation of the left pulmonary artery was performed in fetal lambs. The procedure was well tolerated and most animals survived to birth. At birth, the animals were in no distress but had mild pulmonary hypertension. The lung to which the pulmonary artery was ligated underwent normal intrauterine maturation.

Histochemical studies of experimental fetal intestinal obstruction.

Experimental intestinal atresia can be produced by mesenteric disruption in fetal lambs. In previous reports, a detailed histochemical study of the bowel in this atresia model demonstrated: (1) hyperplasia of ganglion cells in the dilated proximal segment, (2) involutional changes in the area of maximal distension, (3) decreased to absent adenosine triphosphatase (ATP-ase) production in the area of the atresia, (4) gradual increase of ATP-ase production to normal proximally, and (5) greater reduction of ATP-ase production along the antimesenteric border compared to the mesenteric border. In the present study, a model of fetal intestinal obstruction by simple ligation of the bowel has been created to observe the effects of pure obstruction of the lumen of the fetal bowel without the possible ischemic effects of any vascular interruption. Studies with this model reveal: (1) hyperplasia of ganglion cells in the dilated proximal segment, and (2) decreased ATP-ase production proximal to the obstruction, but (3) no involutional changes in the area of maximal distension. These findings show a pattern of disturbance of bowel morphology and function caused by obstruction of the fetal bowel that is similar to but less severe than that seen with intestinal atresia.

A new percutaneous technique for occluding arterial flow to massive congential A-V malformations to prevent major hemorrhage during resection.

In this experience, life-threatening hemorrhage and operative excision of large cranial congenital A-V malformations were managed in two young patients. The major feeding arteries were selectively occluded, shrinking the masses dramatically and greatly facilitating resection which could then be followed by immediate skin grafting. This technique employs standard technology for arteriography and catheterization and should have widespread usefulness in children for control of large Congenital AV Malformations and other vascular masses, including malignant tumors.

Pectus carinatum: results of surgical therapy.

There are a variety of protrusion defects of the anterior chest which are collectively referred to as pectus carinatum. Over a 14-yr period, 13 patients have had surgical correction of pectus carinatum at The Johns Hopkins Hospital. Technique includes costal cartilage resection and a sternal osteotomy. There was no mortality, complications were few and results are good. Repair is recommended for patients in late adolescence due to the cosmetic nature of the procedure, the apparent lack of an associated postural deformity and the good results of operation in this age group.

Avoidable complications of resection of major nerve trunk neurofibromas and schwannomas.

Neurofibromas and schwannomas of major nerve trunks may present with a variety of symptoms and other clinical concerns. These include: (1) the question of malignancy, (2) pain and paresthesias, (3) cosmesis, and (4) symptoms and impending problems related to compression of adjacent structures. For these reasons, patients with neurofibromas and schwannomas may have valid reasons for surgery. Precise delineation of the anatomical relationships of the lesions and their location within peripheral nerve trunks is essential for decision making regarding when and how they should be excised; judgement regarding risks vs. benefits must be made carefully. Microsurgical dissection can be utilized to remove some nerve trunk lesions with preservation of most or all motor and sensory functions. An important goal in resecting benign lesions is to avoid sacrificing major motor and sensory functions. Three cases are presented to illustrate these concepts: 2 cases with significant motor loss following partial resection of large neurofibromas involving peripheral nerve trunks and 1 case with a large femoral nerve neurofibroma excised from the nerve trunk with microsurgical dissection, leading to relief of symptoms and complete preservation of motor function.

Emergency thoracotomy for injury.

Emergency thoracotomy is required in 10-15 per cent of all patients with thoracic injury. Nine specific indications for emergency thoracotomy have been described for injured persons. A variety of incisions is available to the surgeon, and selection of the appropriate incision is based on radiographic and clinical findings. Thoracotomy in the emergency room is occasionally indicated, but it should be performed only by surgically trained individuals. Patients requiring emergency thoracotomy who are not dead on arrival have a greater than 75 per cent chance of survival.

Ventricular septal defect from blunt chest injury.

The spectrum of cardiac trauma resulting from blunt injuries to the chest or decelerating injuries is wide and ranges from mild cardiac contusion to cardiac rupture. Ventricular septal defect can result from such injuries, either immediate or delayed, and is best corrected electively, but severe cardiorespiratory disturbance may necessitate emergency operation. Repair is performed electively if possible, but emergency repair can be successfully accomplished when necessary and requires careful reinforcing of sutures and patches. This is a case report of a successful emergency repair of ventricular septal defect resulting from blunt chest injury.

Management of patients with modified Hunt pouch esophagojejunostomy after total gastrectomy for carcinoma of stomach.

The Hunt pouch and its various modifications have been used as an esophagojujunostomy technic after total gastrectomy or esophagogastrectomy for benign and malignant conditions. This pouch procedure may be particularly useful for palliative gastric resections in which the patient's ability to ingest adequate foodstuffs might be enhanced by a larger reservoir, thereby improving nutritional status and possibly facilitating chemotherapy or radiation therapy or both. Intravenous hyperlimentation has been used to advantage preoperatively and postoperatively to help nutritionally depleted patients better tolerate such an operation and has been useful during treatment of complications related to the procedure. Three recent cases are discussed to illustrate the above propositions.

Effects of unilateral, low-frequency, neuromuscular stimulation on superficial circulation in lower extremities of patients with peripheral vascular disease.

The effects of unilateral, low-frequency, neuromuscular stimulation on the circulation in skin of the lower extremities were studied in eight subjects with peripheral vascular disease and eight control subjects with normal peripheral vasculature. Sixty minutes of stimulation (at 2 Hz), of sufficient intensity to produce visible contraction of musculature, was applied through cutaneous electrodes placed over the common peroneal nerve and dorsum of the foot. Systolic and diastolic blood pressure, heart rate, bilateral great-toe photoplethysmographic waveform, and bilateral pedal skin temperature were recorded at 30-min intervals during stimulation and 30 min after stimulation. Mean differences in recordings before and after stimulation were then calculated for each parameter, showing in subjects with peripheral vascular disease significant increases of 5.3 +/- 2.1 mm and 0.5 +/- 0.1 degree C for ipsilateral photoplethysmographic waveform amplitude and pedal skin temperature, respectively. Mean differences for the remaining parameters were not significant. Recorded parameters in the control group did not change after stimulation. These results demonstrate that low-frequency, neuromuscular stimulation produces regional cutaneous vasodilation in subjects with peripheral vascular disease. No evidence of generalized vasodilation after neuromuscular stimulation was found.