David L. Collins

A new approach to congenital posterolateral diaphragmatic hernia

The theory is advanced that increased pulmonary vascular resistance, resulting in a state of fetal circulation, with right-to-left shunting through the ductus arteriosus, is the main reason that many patients do not survive after repair of a diaphragmatic hernia. Three patients are presented (who, by Raphaelys criteria, were destined for a fatal outcome) in whom the ductus was ligated, and vasodilator drugs were infused into the pulmonary artery. All three demonstrated definite improvement in oxygenation. Two expired after 6 days, one of whom was found at autopsy to have intestinal volvulus and gangrene, and the other multiple plumonary emboli. One case, so managed, survived. The suggestion is made that pulmonary hypoplasia is not the main reason for the high mortality rate after diaphragmatic hernia repair, and that additional laboratory and clinical investigation of the pulmonary circulation may lead to significant improvement in results.

Pre-term and particularly pre-labor cesarean section to avoid complications of gastroschisis.

Abstract The marked advantages and merit of pre-term and particularly pre-labor (PTPL) cesarean section (C-section) in the avoidance, and indeed, virtual elimination of severely disabling gastroschisis (GS) complications in infants diagnosed prior to birth by ultrasound has unfortunately remained controversial in the 10 to 12 years since it was first reported and strongly recommended by numerous authors. During this period, GS has remained one of the four major causes of the short-gut syndrome (SGS) in infancy and childhood and a major cause of prolonged, costly, complicated, and hazardous neonatal intensive care unit stays with requirements for total parenteral nutrition (TPN). The most serious and frequent complications of GS in infants born without PTPL C-section are the occurrence of the “peel”, which greatly enlarges and rigidifies the eviscerated gut, and of “complicated GS” (intestinal atresia/s, stenosis, necrosis, perforations) (CGS). The “peel” occurs in 100% of these cases and CGS in approximately 20%. “Peel” enlargement and rigidification of eviscerated intestine in the presence of a reduced peritoneal cavity causes great difficulty in covering the eviscerated, enlarged, and rigidified gut with abdominal wall, skin, a prosthesis, etc., and frequently produces gut ischemia from excessive pressure, which may lead to necrotizing enterocolitis (NEC) and SGS as well as prolonged hospital stays. The presence of a “peel” greatly complicates the hazards of dealing with cases of CGS, as resection and anastomosis are virtually impossible in the presence of a “peel.” The authors report personal experience with 77 cases of GS dating as far back as 1951; 44 of the infants were born after the onset of labor by vaginal or C-section delivery and all had some degree of “peel” formation. Of 320 cases from the literature (including some of the cases reported here), 61 (19.1%) involved CGS. Of the 33 cases born PT, and especially PL, there were no cases of “peel” and only 1 case of CGS (3.0%). This infant had a single atresia associated with a very small (1 cm) defect in the abdominal wall and no labor-induced “peel,” which was easily and successfully repaired by resection and anastomosis. The 6.4-fold reduction in the occurrence of CGS by PTPL C-section (3.0% vs 19.1%) was statistically significant by the chi-square test (P < 0.05), as was the 100% elimination of the disabling “peel.” If the single case of CGS associated with a very small defect and no labor or labor-associated “peel” is eliminated, the incidence of CGS in the remaining PTPL group of 32 cases falls to 0 (0% versus 19.1%, P < 0.007). PT and especially PL C-section may be expected to virtually eliminate “peel” formation and CGS and to remove GS as one of the four major causes of SGS. The findings of this report that PT labor prior to PT C-section may result in both “peel” formation and CGS further solidifies the role of labor in the production of both the “peel” and the equally disabling CGS. Failure to appreciate the central role of labor in GS complications has doubtless contributed to the persistent controversy concerning the value and importance of PTPL C-section for gastroschisis diagnosed in utero. The pediatric surgeon has an important responsibility with the obstetrician to monitor the possible occurrence of occult labor in the waning weeks of pregnancy and be prepared to do a prompt C-section if it occurs and there is adequate lung maturity. The achievement of “peel”- and CGS-free gut would greatly facilitate the use of the new Bianchi technique of gut reduction without anesthesia. The combination of the use of epidural anesthesia for the elective PTPL C-section with the Bianchi approach would spare both mother and baby any untoward effects of general anesthesia and present the potential for massive reductions in hospital costs with minimal patient manipulation and disturbance. For infants born with labor-associated “peel,” re-evaluation of the suitability and effectiveness of surgical “peel” decortication from involved gut is strongly urged.

Primary fascial closure in infants with gastroschisis and omphalocele: a superior approach.

The optimal surgical management of neonates with gastroschisis and omphalocele remains controversial. Early attempts at primary closure were limited by respiratory and vena cava compromise and were gradually replaced by skin closure only, staged reduction of implanted prostheses, and escharification of the intact omphalocele. Advances in surgical technique, neonatal intensive care, and mechanical ventilation have made primary closure a viable alternative once again. We wish to report a consecutive series of 73 infants, including 54 with gastroschisis and 19 with omphalocele, over a 16-year period. Fifty gastroschisis patients were closed primarily, with four deaths (8%), and four with staged prostheses, with one death (25%). Sixteen omphaloceles were closed primarily, with three deaths (19%); two closed with a staged prosthesis, and one patient, closed with skin flaps only, died (100%). Overall survival for primary closure was 59/66 (89%) and for staged reduction, 5/7 (43%). In the past 5 years all patients have been closed primarily with no deaths (100% survival). All survivors were alive and well 1 to 15 years postoperatively. Our current operative technique involves vigorous manual stretching of the abdominal cavity, manual emptying of the entire Gl tract, and primary fascial closure, with no gastrostomy. Key points in postoperative management include total paralysis and mechanical ventilation, as the initially tense abdominal wall softens over 1 to 2 days, followed by weaning from the ventilator, and vigorous fluid support. Gl function returns more rapidly after primary closure (average of 3 days) and hospital stay is not prolonged. Other complications (intestinal obstruction, two patients; fistulae, one patient, etc) are much less frequent.(ABSTRACT TRUNCATED AT 250 WORDS)

Cervicofacial mycobacterial adenitis in children: Endemic to san diego?†

Nontuberculous mycobacteria (NTM) are an important cause of cervicofacial lymphadenitis in children. A dramatic increase has been seen in confirmed cases of NTM lymphadenitis in San Diego in the past few years. This report encompasses a span of 2 years 9 months, during which 22 children had confirmed NTM infection and another 14 had necrotizing granulomatous lymphadenitis in whom the specific diagnosis of NTM could not be made. This apparent increase in the prevalence of NTM as compared to previous reports in the literature suggests a possible endemic risk for NTM in the San Diego area. These cases are reviewed and our diagnostic and surgical approach to possible NTM infection is described. We also discuss possible explanations for the increasing occurrence of NTM in the San Diego area.

Hydrostatic reduction of ileocolic intussusception: A second attempt in the operating room with general anesthesia

Over a 3-year-period, standard treatment with hydrostatic pressure from a contrast enema failed to reduce ileocolic intussusception in 31 of 62 children. With the child anesthetized in the operating room, a second contrast enema was given before laparotomy. Of the 31 intussusceptions, 21 (68%) were reduced without complication, thereby avoiding the discomfort, longer hospitalization, complications, and expense of surgery. Nine of the remaining 10 intussusceptions were difficult to reduce manually during surgery or required resection. The overall nonoperative reduction rate for the 3-year period was 84%; for the last 2 years it was 90%. Success with the second enema may be related to the effects of general anesthesia. In addition, partial reduction with the first enema may improve blood flow from the intussusceptum so that it becomes smaller and easier to reduce with the second enema. Because it can easily be added to standard management protocols without increased risk, routine use of this second enema with anesthesia is recommended.

Chylous ascites: the first reported surgical cure by direct ligation.

A patient with apparently intractable chylous ascites was finally explored after the administration of preoperative lipophilic dye. This made identification of a leaking lacteal possible and ligation resulted in a cure.

Multiple intestinal atresia and amyoplasia congenita in four unrelated infants: A new association

Four unrelated infants with multiple segments of nonduodenal intestinal atresia and amyoplasia congenita are described. The extreme rarity of each of these conditions as a single entity indicates that their concurrent incidence in four separate unrelated patients is of significance with respect to pathogenesis. The nature of the relationship between the two conditions is unknown.

Operative intervention for pediatric liver injuries: Avoiding delay in treatment

To identify the physiological and anatomic factors that characterize the need for operative management of blunt pediatric liver injuries, the case records of 106 pediatric trauma victims with liver injuries over a 6-year period were reviewed. Sixty-nine patients were managed without operation (nonoperative) and 37 underwent operation, 7 with penetrating and 30 with blunt liver injuries. Of these 30 patients, 21 underwent laparotomy due to blunt liver injuries (operative); the remaining 9 patients required operation due to associated intraabdominal injuries. Nine (45%) of the 21 operative patients had major hepatic vein or retrohepatic vena caval injuries, 7 of whom died. Overall mortality was 9.4% (10/106). When nonoperative and operative groups were compared, those who underwent laparotomy due to blunt liver injuries: (1) had significantly lower Champion and Pediatric Trauma Scores due to multisystem injury; (2) had 25% or greater lobar disruption with pelvic blood collections on computed tomography scan; (3) underwent early transfusion within 2 hours of admission (18/21); and (4) were frequently found to have a major hepatic vein or retrohepatic vena caval injury at the time of operation. Only one patient successfully managed without operation received greater than 30 mL/kg of blood products within 24 hours of admission. As selective nonoperative management of pediatric liver injuries gains widespread acceptance, the identification of factors that predict the need for operative intervention will limit the potential risks of delay in treatment.

Intussusception in hemophilia

Abstract The second successfully resected intussusception in a hemophiliac is reported. The mainstay of the antihemophilic therapy was cryoprecipitated globulin, an easily prepared blood fraction containing the antihemophilic globulin from 500 ml. of blood in a volume of 30 ml. It is suggested that with the use of the material, major surgery in hemophiliacs may be undertaken with less risk than formerly.

Separation of omphalopagus twins : Unique reconstruction using syngeneic cryopreserved tissue

Omphalopagus twin girls were admitted for evaluation of possible separation and repair at age 7 days. Prenatal sonographic diagnosis occurred late in the third trimester and was followed by cesarean section delivery shortly thereafter. Results of extensive evaluation over the next 7 days including x-rays, computed tomography and ultrasound scan of the head and torso, and cardiac catheterization showed: the gastrointestinal tracts were separate and normal, the livers were joined but had separate biliary and vascular systems, and the hearts were separate with vastly different anatomy and function. One twin (twin A) had a normal heart with a small insignificant VSD. Twin B had a single ventricle, an incompetent A-V valve, stenotic pulmonic valve, ASD, PDA, and congenital heart block. Hemodynamic support of twin B was almost entirely from twin A. The vascular communications between the two consisted of a major connection between the internal mammary arteries and large arterial and venous connections traversing the joined livers. Because of continued deterioration of twin B, separation was undertaken at age 15 days. The separation included dividing the liver and the multiple large vascular connections. Two teams then reconstructed each twin separately. Twin B began showing signs of cardiac decompensation shortly after separation in spite of placement of a pacemaker, pulmonary artery banding, and ligation of the PDA. Cardiac function rapidly deteriorated and she died. Tissue from her chest wall was cryopreserved and placed in the tissue bank. Twin A underwent closure of her abdomen, and received a temporary bovine pericardial patch over the chest defect. She subsequently underwent placement of a graft of twin Bs rib cage to bridge the bony chest defect and skin flap closure. She is presently taking a normal diet and thriving at home at age 18 months. The use of cryopreserved tissue from a syngeneic source provides a unique method of reconstruction in this situation.