Postoperative functional and manometric evaluation of patients with Hirschsprung's disease.

Sixty-two patients with Hirschsprung's disease (operated on according to Duhamel, Swenson, or Soave) were evaluated for quality of defecation and studied manometrically. Follow-up was 30 years. Stools were normal in 30% to 50% of patients, while the rest had either constipation, loose, or increased frequency of stools per day. Continence was complete in 30% to 60%. Enterocolitis was seen in 12 patients preoperatively and in 20 patients postoperatively, regardless of type of operative procedure. This complication improved with age. To correct some of these problems, 18 had post pull-through internal sphincter myectomy with 50% improvement. Manometrically, 50% to 70% experienced a sensation of fullness and an urge to defecate following rectal balloon inflation. The resting external sphincter pressure was high in the majority of patients and further increased following rectal balloon inflation. The internal sphincter pressure showed a normal anorectal reflex in only 10% of patients postoperatively and did not seem to be related to clinical fecal continence. We concluded from the study that an appreciable number of patients in this select group with Hirschsprung's disease suffered from a variety of stooling disorders postoperatively. The majority of these complications were managed medically and/or socially but some were quite incapacitating.

Congenital liver cyst presenting as congenital diaphragmatic hernia.

A 5-day-old infant was found to have an unusual presentation of a congenital liver cyst. The cyst arose from the left lobe of the liver and herniated into the left thoracic cavity through a congenital defect at the central tendinous portion of the diaphragm. The association between a congenital liver cyst and the location of the diaphragmatic hernia suggests that the abnormality occurred during very early embryonic development when the liver rapidly expands and extends into the septum transversum at the base of the heart. A small amount of liver tissue might well have perforated through the thinnest central portion of the septum transversum and underwent cystic degeneration due to incarceration.

Chronic constipation. Manometric patterns and surgical considerations.

We studied 80 children who had severe chronic constipation that was refractory to dietary, medicinal, and psychiatric therapy by means of barium enemas, rectal biopsies, and manometry. Four patients had Hirschsprung's disease and three patients had segmental dilatation of the colon. The remaining 73 patients had radiologically dilated anorectums with inpaction. Only 49 patients underwent rectal wall biopsies. All of the specimens were positive for ganglion cells. Manometric study of the 73 patients showed 18 patients who had elevated internal sphincter pressures with good relaxation following balloon inflation (group A); 45 patients who had normal internal sphincter pressures but no relaxation following balloon inflation (similar to Hirschsprung's disease) (group B); and ten patients who had normal pressures with good relaxation. Twenty-five patients in groups A and B had internal sphincter myectomies. Eight patients resumed normal bowel movements, 11 patients required minimal medicinal help, and two patients' conditions did not improve. Postoperatively, there was a reduction to normal pressure levels in group A patients and relaxation of the internal sphincter in group B patients.

Radioisotope spleen scan in patients with splenic injury.

The technetium Tc 99m sulfur colloid liver-spleen scan is a valuable aid in diagnosis and treatment of patients with splenic injury. After reviewing the charts of 47 patients who were ill as a result of splenic trauma, we came to the following conclusions: (1) the scan identified the injury, accurately mapped its extent, and indicated the presence or absence of associated liver injuries; (2) the scans were useful in following the extent and rate of healing of the splenic injury; (3) the scan is an indirect measurement of of return of splenic fuction; (4) the procedure can be performed in a reasonable time frame with no serious morbidity; and (5) the indications, contraindications, and timing of scans are now reasonably well established.

Littre's hernia in infancy presenting as undescended testicle.

Littre's hernia is defined as any hernial sac which contains a Meckel's diverticulum. It has been reported in association with inguinal, umbilical, femoral, sciatic, ventral, and lumbar hernias. This hernia is rare, particularly in children, in whom the umbilical variety is reported by some to be most common. Littre's hernia is difficult to diagnose, but should be suspected in patients with gastrointestinal bleeding, incompletely reducible hernias, and fecal hernial fistulas. It may be confused with cryptorchidism when Meckel's diverticulum adheres to and envelops the testicle making palpation of the gonad difficult. Recommended treatment is resection of the Meckel's diverticulum from within the opened hernial sac followed by herniorrhaphy.

Congenital diaphragmatic hernias: eleven years' experience.

Fifty-five patients with congenital diaphragmatic hernias (1966 to 1976) were studied and compared to 65 similar patients reported previously (1953 to 1963). The mortality was found to be the same in the two groups despite the fact that in the current series, patients were diagnosed and operated on at a younger age. Initial uncorrected pH value was found to be of prognostic importance. All babies whose initial uncorrected pH was greater than 7.0 survived and almost all those whose pH was less than 7.0 died, with the group in-between having a 50% chance of survival. Earlier operation and correction of acidosis did not substantially improve the chance of survival. Hypoplasia of the lungs and major cardiovascular anomalies contributed to death in a number of patients. Pulmonary hypertension causing a right-to-left shunt was responsible for the deaths of others and, therefore, the use of vasodilator drugs deserves further evaluation.

Preservation of the injured spleen.

Experience with suture repair of splenic lacerations in 14 children and 8 adults is reported. The youngest patient was 3 days old and the oldest 60 years. Adequate healing was confirmed by postoperative splenic scans with or without arteriography. The 2 deaths in this series were not related to the splenic repair and were due to associated major injuries. The course of illness in the remaining 20 survivors did not reveal a failure of this technique or a complication related to the use of this conservative approach. It is expected that salvage of the traumatized spleen will eliminate the risk of fulminant postoperative infections that may be fatal.

The role and safety of early postoperative feeding in the pediatric surgical patient.

The authors report their experience with early postoperative feedings in a variety of pediatric surgical patients utilizing a needle catheter jejunostomy. A small caliber catheter, similar to that used for antecubital central venous cannulation, is inserted in the antimesenteric border of the jejunum providing a subserosal tunnel. A purse-string suture is placed around the catheter and then it is secured to the abdominal wall, and the catheter is brought out through the abdominal wall via a needle puncture wound. Our experience with 27 insertions in 25 patients ranging in age from 1 day to 17 yr, has demonstrated the ease of placement and feasibility of immediate postoperative feeding. Patients were generally started on a dilute elemental diet through the jejunostomy within 12 hr of the operative procedure. Patients were administered between one and 3.5 g of protein kg/day and between 45 and 100 cal/kg/day depending on age and weight. Duration of treatment with enteral nutrition ranged from 10 to 150 days. There was no catheter-related complications utilizing the technique described. Technical details of catheter placement and protocol for administration of early postoperative feedings are discussed. The ability to provide nutritional support via the gut has obviated the need for total parenteral nutrition in the majority of these patients.