J. Alex Haller

Chest wall constriction after too extensive and too early operations for pectus excavatum

BACKGROUND AND METHODSnSince 1990 we have evaluated 12 children and teenagers in whom severe cardiorespiratory symptoms have developed due to failure of chest wall growth after very extensive pectus excavatum operations (removal of five or more ribs) at very early ages (< 4 years).nnnRESULTSnApparently these extensive procedures have removed or prevented growth center activity, which resulted in restriction of chest wall growth with marked limitation of ventilatory function. The forced vital capacity ranged from 30% to 50% of predicted and the forced expiratory volume in 1 second from 30% to 60%. All patients are symptomatic with mild exercise and cannot compete in running games. Our protocol for critical evaluation includes exercise pulmonary function studies and axial computed tomographic reconstruction.nnnCONCLUSIONSnThis report is an alert to recognize such patients and also to recommend delay in operative repair in small children until at least 6 to 8 years of age. The younger the patient the more limited the chest wall resection for pectus excavatum should be. Five of these patients have had a chest cavity expansion operation with encouraging early results.

Cardiorespiratory function before and after corrective surgery in pectus excavatum.

OBJECTIVEnTo determine whether pectus excavatum (PE) results in cardiopulmonary abnormalities, and whether surgical repair results in improvement.nnnMETHODSnWe performed pulmonary function testing and incremental exercise testing in 36 adolescents with PE (aged 16 +/- 3 (SD) years) and 10 age-matched, healthy control subjects. Fifteen PE subjects were reexamined postoperatively, as were six control subjects.nnnRESULTSnPreoperatively, PE subjects had a significantly lower forced vital capacity than control subjects had (81% +/- 14% vs 98% +/- 9% of the predicted value; p < 0.001). Chest computed tomography ratios of internal transverse to antero-posterior diameters correlated inversely with total lung capacity (r = 0.56; p < 0.01). Fifty-eight percent of PE subjects had subjective complaints of exercise limitation. PE subjects exercised at a workload similar to that of control subjects. Maximal heart rate and O2 pulse did not differ between the two groups. Respiratory measurements during exercise were similar between the two groups. Respiratory measurements during exercise were similar between the two groups. Postoperatively there was no change in forced vital capacity (as a percentage of the predicted value). The PE subjects exercised for a slightly longer period and had a slightly higher O2 pulse, whereas control subjects showed no change.nnnCONCLUSIONnSome subjects with PE have mild restrictive lung disease, which is not affected by surgical repair. Postoperatively they have a slight increase in exercise tolerance and O2 pulse, which suggests improved cardiac function during exercise. However, the clinical implications of this modest improvement are unclear.

Hemodynamic effects of primary closure of omphalocele/gastroschisis in human newborns

To determine whether they could establish reliable, objective criteria that would predict safe, primary closure of abdominal wall defects (omplialocele/gastroschisis) in newborn infants, the authors measured intraoperative changes in intra-gastric pressure (IGP), central venous pressure (CVP), cardiac index (CI), systolic arterial blood pressure (BP), and heart rate (HR). Eleven neonates, who averaged 2.7 kg (range 1.5–4.1 kg) and 36 weeks gestation (range 30–41 weeks) were anesthetized with fentanyl (7.5–12.5 μg/kg), metocurine (0.3 mg/kg), and oxygen. Three infants had defects that were too large to close primarily. Of the eight infants who underwent primary closure, four required re-operation within 24 h because of oliguria or poor peripheral perfusion. Infants who required re-operation had intra-gastric pressures of 20 mmHg or more, a decrease in CI of 0.78 1 · min · m2 or more, and an increase in CVP of 4 mmHg or more. Heart rate, BP, and systemic vascular resistance did not differ in infants requiring and not requiring reoperation. The authors conclude that intraoperative measurement of changes in IGP, CVP, and/or CI can reliably predict success or failure of primary operative repair of abdominal wall defects in human neonates.

Outcome of pectus excavatum in patients with Marfan syndrome and in the general population.

We reviewed the records of 28 patients with Marfan syndrome and 30 age-matched control patients with presumed isolated pectus excavatum to determine the outcome of surgical repair of the pectus deformity in Marfan syndrome. One third of the patients with Marfan syndrome underwent repair of the pectus excavatum before diagnosis. Of the 30 patients with isolated pectus excavatum, 17 had findings by history or physical examination, such as mitral valve prolapse, scoliosis, or a relative with pectus excavatum, suggestive of an underlying disorder of connective tissue. Pectus excavatum of more than moderate severity recurred in 11 of 28 patients with Marfan syndrome and was associated with young age at initial surgery and lack of temporary internal stabilization of the chest after surgery. Only two of the control patients had recurrence of the defect; one of these patients had findings suggestive of an underlying heritable disorder of connective tissue. We conclude that pectus excavatum may indicate the presence of an underlying heritable disorder of connective tissue such as the Marfan syndrome. In patients with Marfan syndrome, and possibly other inherited connective tissue disorders, surgical repair should be delayed if possible until skeletal maturity is nearly complete and should employ internal stabilization.

Spiral ct with 3d reconstruction in children requiring reoperation for failure of chest wall growth after pectus excavatum surgery:Preliminary observations

Pectus excavatum is the most common congenital chest wall deformity. Extensive corrective surgery prior to age 3 may disturb chest wall growth and result in a constricted thorax. We describe our surgical and radiologic experience with eight such cases, paying particular attention to the role of spiral computed tomography (CT) with 3D reconstruction in patient management. Spiral CT was performed on children who had developed restrictive chest walls following pectus excavatum surgery. These children then underwent a unique operation to elevate the sternum and attempt to correct their restrictive chest wall defects. In several cases, postoperative spiral CT was performed. Spiral CT with 3D reconstruction defined the orientation of the ribs and costal cartilages and their relationship to the sternum, allowing exact preoperative measurement of the bony rib cage and guiding individualized operative correction. Computed thoracic volumes in select cases correlated well with subjective patient reports of increased exercise capacity. Repair of pectus excavatum defects prior to age 3 may result in constrictive thoracic abnormalities. Surgical correction can increase thoracic volume and improve prospects for normal thoracic function. Three-dimensional reconstruction of spiral CT data is useful in both preoperative and postoperative evaluation.

Criteria for safe cost-effective pediatric trauma triage: Prehospital evaluation and distribution of injured children

In an effort to maximize staff utilization, all pediatric trauma patients were triaged by emergency room personnel to one of two tiers, based on information reported by prehospital providers over radiotelephones. A total of 952 patients less than 15 years of age were evaluated during a 1-year period. The triage criteria had a sensitivity of 86% in predicting which trauma patients would require operating room and/or pediatric intensive care, while maintaining a specificity of 90%. Fifteen patients died; however, by TRISS methodology there were no unexpected deaths and four unexpected survivors. All eventual deaths were initially captured from field data by the severely injured triage criteria. The study data suggest that physician-controlled two-tiered field triage criteria can safely serve to maximize staff utilization in the emergency room.

Surgical correction of mandibulofacial deformities secondary to large cervical cystic hygromas

The clinical features of cystic hygroma are presented. The effect on mandibular morphology is described, and surgical correction of the deformities is discussed.

Acute pancreatitis associated with congenital cyst of the common bile duct

Summary A case of congenital cystic dilatation of the common bile duct with an associated pancreatitis is presented. The literature is reviewed in an attempt to find the frequency of such an association. Five other cases were found in which both conditions coexisted. The symptoms of pancreatitis are not always sufficiently different from those of choledochus cyst to ensure the simultaneous diagnosis of both diseases when they occur together. Therefore, an awareness of the possible association may be helpful.

Improved silastic tracheostomy tubes for infants and young children

Abstract An improved Silatic tracheostomy tube which has been used in 18 infants and young children avoids the problems of tracheal trauma and encrustation which are common in the metal tracheostomy tubes. In addition, the tube facilitates the use of respirator assistance because of its elastic qualities and ease of application. It is radiopaque and can be steam sterilized. There have been no serious complications related to the use of the Silastic tracheostomy tube and further clinical trials seem indicated.

Caring for the injured children of our world A global perspective

This article describes several countries that began and continue active pediatric trauma programs, which can serve as examples for developing countries. Emergency medical services for children need to be developed so that the special needs of children with serious injuries are addressed.