Congenital pulmonary myofibroblastic tumor: a case report with cytogenetic analysis and review of the literature.

We report a case of congenital pulmonary myofibroblastic tumor, and review prior reports of this rare neoplasm to demonstrate its clinically benign behavior despite histologic features previously interpreted as sarcoma. The patient, a female neonate, presented with severe respiratory distress after cesarean section delivery. A large radio-opaque mass was detected in the right hemithorax and resected by right bilobectomy. The tumor mass, confined to the lung, was composed of interlacing fascicles of plump spindle cells showing myofibroblastic differentiation and complex cytogenetic abnormalities. Though sarcomatous in appearance, with highly cellular areas and numerous mitoses, there has been neither tumor recurrence nor metastases. The patient remains alive and well 1 year after surgery. Review of the few other reported cases confirms the uniformly benign behavior of this tumor.

Superior vena cava syndrome in childhood: report of ten cases and review of the literature.

Experience with ten children with superior vena cava obstruction is reported. Five patients had non-Hodgkin's lymphoma, two had Hodgkin's disease, two had benign lesions, and one patient was suspected on clinical and radiologic basis to have a lymphoma. The clinical situation at presentation was often critical and required rapid treatment. Radiotherapy and/or chemotherapy were used initially as lymphoma was the most frequent cause of obstruction. Urgent thoracotomy was resorted to when treatment failed. Survival with no evidence of disease for more than 5 years was observed in three children: one had a cystic lymphangioma, another had a mediastinal abscess, and the third had a Hodgkin's lymphoma. Review of the literature from 1951 to 1976 revealed that only 24/150 children reported with superior vena cava obstruction syndrome had mediastinal tumors; the remainder developed the obstruction after surgical procedures on the heart or vena cava.

Circular myotomy of the esophagus: clinical application in esophageal atresia.

Three infants born with esophageal atresia that was repaired by end-to-end anastomosis combined with a circular myotomy on the upper segment are reported. The distance between the free surgical margins of the esophagus ranged between 1.5 and 4.5 cm. The esophageal myotomy was used to reduce the tension on the anastomosis. The suture line healed in each patient without clinical or roentgenographic evidence of breakdown. Follow-up of these patients ranged between 10 months and 3 years. Roentgenographic evaluation of their esophageal motility showed efficient peristaltic activity in the distal esophageal segment. Two of the patients had a subsequent history of impaction of solid particles in the upper esophageal segment at the age of 13 months and 2 years. The possibility that the circular myotomy contributed to this increased incidence of impaction is raised.

Intravascular missile embolization in childhood: report of a case, literature review, and recommendations for management.

A collective review of 20 cases of missile embolization among children (1961 to 1988) is analyzed, one case added, and guidelines for diagnosis and management are outlined. Causative agents were bullets (14 patients), pellets (5), and fragments (2). Their trajectory was arterioarterial (11), venovenous (5), paradoxical (4), and mixed (1). Diagnosis was suspected when an exit wound was absent and the foreign body was traced on regional x-ray. Embolization was predominantly to the legs, with a tendency for the left (5 of 8 cases). Upper extremity emboli were exclusively to the right. Only one of five cardiac entries required closure to control bleeding compared with four of six aortic. Embolectomy was performed in 16 patients. The overall mortality rate was 9.5%. Factors predicting a favorable outcome are early presentation, diagnosis, and intervention; location of cardiovascular entry and embolus site; and presence of soft tissue tamponade at entry wound. Although embolectomy for cerebral, asymptomatic pulmonary arterial, and silent venous emboli is controversial, universal agreement prevails regarding removal of systemic arterial as well as venous emboli that are potentially problematic.

Primary repair of colorectal injuries in childhood.

A retrospective survey of 44 children with colorectal perforating injuries secondary to shrapnel and high velocity bullets is presented. Seven patients had the injury limited to the colon or rectum. All patients were operated upon within 7 hr from the their colonic wounds and 20 underwent a colostomy. Five of the patients repaired primarily had their injuries to the left side of the colon or rectum. None of the patients undergoing primary repair suffered from an anastomotic leak. The mortality rate after primary repair was 16.6% and after colostomy 10%. The mortality was significantly different in the group of patients who had additional injuries where a colostomy procedure was associated with a lower mortality rate (11% versus 21%). Shock on admission and during operation affected survival adversely in all patients. The average hospital stay of survivors was 24 days with "primary repair" and 36 days with "colostomy."

Neuroblastoma: report on a 21-year experience.

This is a review of 80 patients with neuroblastoma managed at the American University of Beirut Medical Center between 1963 and 1983. Three patients had ganglioneuroblastoma of whom one showed histologic evidence of maturation into a ganglioneuroma. Four patients were less than 1 month of age and 33 were less than 2 years of age. The site of origin was intra-abdominal in 56 patients of whom 34 were intra-adrenal. Intraspinal involvement was noted in 12 patients, of whom one was a newborn. Treatment and adequate follow-up were possible in 63 patients. Total excision of the tumor was performed in 17 patients, and partial excision in 14. The 2-year and 5-year survival rates were 36% and 25%, respectively. Age, site of the tumor, and degree of cellular differentiation were the only independent variables affecting survival. Eleven of 14 patients younger than 1 year were alive 5 or more years after diagnosis. Cervical, thoracic, and pelvic tumors had a better prognosis than abdominal tumors. Other factors affecting survival were the stage and the mode of therapy. Infants with stage IV-S congenital neuroblastoma had a very poor prognosis. The initial urinary VMA level as well as the presence or absence of calcifications within the tumor had no bearing on prognosis.

Tetralogy of Fallot with double aortic arch: successful staged repair. Case report and review of literature.

The rare association of DAA with TOF is reported in a young boy who underwent successfully at the age of 3 months an emergency shunt between the anterior (left) hypoplastic aortic arch and the LPA after the vascular ring was divided. Total correction of the cardiovascular malformation was performed at the age of 4 years. The cardiac catheterization, angiographic and operative findings as well as the management of the case are described. Ten other patients with this combination of anomalies were found on review of the literature, eight of whom had only palliative systemic to pulmonary artery shunts. Knowledge of the morphology of the DAA by angiocardiography is helpful in planning the management. Surgical shunts when done early for the treatment of a patient with repeated anoxic spells may reduce the gravity of psychomotor retardation. A staged surgical repair of TOF is recommended for infants having hypoplastic pulmonary arteries.

Penetrating injuries of the heart and the great vessels.

An account of a personal experience with 18 cases of penetrating injuries of the heart and/or the great vessels due to shrapnels or bullets during the civil war in Lebanon is presented. Six of 18 patients were children. A survey of the literature did not disclose a description of these injuries in the pediatric group. Seven patients had signs of cardiac tamponade preoperatively. Fifteen patients had concomitant injuries to other organs. Emergency thoracotomy was used in patients within two hours after arrival to the hospital and was not preceded by pericardiocentesis. Cardio-pulmonary bypass was not used in any patient. There was one death which was related to associated injuries, giving a mortality rate of 5.5%. All survivors have resumed regular activities. Though the author is basically a pediatric surgeon, his expertise had to be stretched during the civil war to cover casualties in all age groups.

Cardiac surgery--challenges and surprises in a country at war.

The challenges and results of cardio-vascular surgery at the American University of Beirut Medical Center (AUBMC) during the civil strife in Lebanon are presented. Dependence on local resources is emphasized. Temporary interruption of open-heart surgery in a university setting may be the product of absence of essential materials and/or personnel. Ingenuity and team approach have contributed to the success and survival of the open-heart program. The operative results and mortality rate were indirectly related to the intensity of fighting in the vicinity of a Medical Center that served as a field hospital.

Congenital diaphragmatic hernia and eventration.

A review of 52 consecutive cases of congenital diaphragmatic hernia composed of 36 cases of Bochdalek hernia (B.H.) 13 of diaphragmatic eventration (D.E.), and 3 of diaphragmatic agenesis (D.A.) is reviewed critically. The operative mortality rate in Bochdalek hernia was 8.3% but became 20% with reference to the 15 infants with B.H. operated in the first 24 hrs. of life and 31.5% with reference to all 19 infants requiring repair of the diaphragmatic defect during the first day after birth. The operative mortality in D.A. was 100% and was nil in D.E. The mortality appeared related directly to prematurity, early age at operation, preoperative hypoxemia (PaO2 less than or equal to 60), hypercarbia, (PaCO2 greater than or equal to 60), and acidosis (pH less than or equal to 7.0), association with life-threatening anomalies affecting the lungs and its vessels, the heart, the size of the diaphragmatic defect and of the celomic cavity, the postoperative development of NEC and of obstructing intestinal adhesions. Eight of the 33 survivors (24%) with B.H. developed intestinal obstruction secondary to adhesions 2 months to 14 years after operation, of whom 7 required surgical intervention, and 3 bowel resections. The total mortality rate in B.H. was 14% and the rate in this series of combined defects was 15%.

Closed cardiovascular surgery in the first year of life--experience with 40 consecutive cases.

A review of 40 consecutive infants with cardiovascular lesions requiring surgical intervention, without the use of the heart-lung machine, during a 3 year period is presented. Twenty-one patients were cyanotic and 19 were non-cyanotic preoperatively. In the former group, an operation was performed on an urgent or emergency basis in 8 patients (38%). In the latter group, 12 patients (63%) were in congestive heart failure and needed digitalis and diuretics preoperatively. The most frequent lesions among both groups were TOF and aortic coarctation, each being encountered in 6 patients, to be followed by d-TGA, PDA, and vascular ring, each being observed in 5 patients. The youngest patient was 8 hours old, had ectopia cordis associated with omphalocele, and the oldest was 12 months old, had a vascular ring. The lightest infant weighed 1.6 kg and had ligation of PDA at the age of one month. The most common surgical procedure was a systemic pulmonary artery shunt (13 patients), closure of PDA (5 patients), followed by repair of aortic coarctation using the LSA as a patch (6 patients), and division of vascular ring (5 patients). The operative M.R. was 15% in the whole series, 19% among the 21 cyanotic babies, 11% among the 19 non-cyanotic infants and 40% among the 10 patients operated in the first month of life. No death was noted among the 14 patients operated after the age of 6 months. Late mortality occurred in 4 patients giving a total mortality rate of 38% in this group of 21 infants with cyanotic malformations.(ABSTRACT TRUNCATED AT 250 WORDS)