The thymus. Pediatric surgical aspects.

Since the original description of thymic death in an infant 400 years ago, the thymus has been recognized as an important structure to practitioners caring for infants and children. The source of many cysts, masses, and tumors in the neck and mediastinum, the thymus gland merits the pediatric surgeon's attention. The thymus is clearly an important lymphoid organ, the removal of which may be therapeutic in MG, but congenital absence leads to profound cell-mediated immunodeficiency. The immunologic sequelae of its neonatal extirpation remains obscure. It is apparent that further research is needed to clarify the functional role of the thymus gland in the developing immune system. Until better elucidated, a conservative approach to neonatal thymectomy may be justified.

Unsatisfactory experience with 'minimal intervention management' for gastroschisis.

PURPOSE: The authors report, for cautionary reasons, their trial with "minimal intervention management" for gastroschisis. After the successful innovative experience with this approach, which Bianchi and Dickson described, they utilized it in 4 consecutive patients. METHODS: In the delivery room a plastic bag was placed over the intestines, which rested in a dependent position to reduce edema. The stomach was decompressed and the patients kept warm. Intravenous fluid at a maintenance rate was given. After about 5 hours an attempt at closure was undertaken in the newborn intensive care unit without anesthesia. An assistant lifted the anterior abdominal wall by applying upward traction on the umbilical cord. Over about 25 minutes the intestines were placed in the coelom, which was closed with a single suture. RESULTS: The outcome was uncomplicated in the first of 4 consecutive patients. The second patient had abdominal compartment syndrome requiring a silo and subsequent resection and has chronic malabsorption 16 months later. The third had an enterocutaneous fistula at 5 weeks that required a small bowel resection. Bedside closure was abandoned in the final case because too much resistance was encountered. She underwent primary repair in the operating room and eventually died of sepsis with intestinal dysmotility. CONCLUSIONS: The "minimal intervention approach" can be effective in some patients who have gastroschisis. This experience suggests that selection criteria are needed before this method can be recommended.

Ovarian masses in the newborn.

Prenatal sonography uncovers many fetal ovarian masses that previously would have gone unrecognized. This challenges clinicians to learn the natural history of these asymptomatic lesions so as to provide the best care postnatally. Spontaneous resolution of simple ovarian cysts is expected by about 6 months of age, which is attributed to predicted changes in the postnatal hormonal milieu. After birth, levels of human chorionic gonadotropin (HCG) and estrogen plummet. Follicle-stimulating hormone (FSH) and luteinizing hormone (LH) rise until about 3 months of age and then fall as the "gonadostat" matures. Although symptomatic cysts demand intervention, simple asymptomatic cysts less than 5 cm in diameter should be left alone but reassessed sonographically. If simple cysts are larger than 5 cm in diameter the risk of torsion may be significant, and intervention often is advocated. However, the risk of torsion versus the likelihood of resolution is not well established. The therapeutic goal for a clinician managing a newborn with a simple ovarian cyst is to maximize ovarian salvage. Aspiration alone may be a reasonable option. The laparascopic approach to the neonatal ovarian cyst provides a view of both ovaries and allows aspiration, unroofing, cystectomy, or ovariectomy. The sonographically complex cyst usually represents adnexal torsion but could be a neoplasm and warrants intervention because the morbidity from untreated neonatal adnexal torsion can be significant beyond loss of the ovary (eg, hemorrhage, peritonitis, intestinal obstruction, or a wandering tumor).

Maximizing ovarian salvage when treating idiopathic adnexal torsion.

Idiopathic adnexal torsion threatens ovarian function. The best ways to maximize ovarian salvage have not been stressed widely. The authors describe 2 girls who illustrate the benefit of untwisting the torsion, limiting resection and performing bilateral gonadopexy to prevent both ipsilateral recurrence and contralateral occurrence.

Restorative proctocolectomy in children with ulcerative colitis utilizing rectal mucosectomy with or without diverting ileostomy.

BACKGROUND: Controversies continue concerning the best way to perform restorative proctectomy (RP) for ulcerative colitis (UC). Can rectal mucosectomy and hand-sewn ileoanal anastomosis (IAA) withstand the challenge posed by extrarectal dissection with a double-stapled technique and no mucosectomy? Is a diverting ileostomy mandatory after RP? METHODS: The authors describe 30 consecutive children with UC who underwent RP with rectal mucosectomy and hand-sewn IAA. The authors assess the results and compare the first 14 patients (group 1) treated with temporary diverting ileostomies with the next 16 consecutive patients (group 2) without diverting ileostomies. RESULTS: The average age (13.8 years in group 1 v 10.4 in group 2), duration of illness before resection (3.2 years in group 1 v 1.5 in group 2), and gender breakdown (10 of 14 were girls in group 1, 10 of 16 were girls in group 2) were similar between the two groups. Outcome was not significantly different between the two groups. Average bowel movements per 24-hour period was 5.5 in group 1 and 4.2 in Group 2. Occasional nighttime staining occurred in two patients in group 1 and five in group 2. No one suffered daytime staining in group 1, and one patient had occasional daytime staining in group 2. Average quality of life (on a scale of 0 to 5) as assessed by the patients or parents was 4.4 in group 1 and 4.9 in group 2. There were 10 total complications in group 1. One child required a permanent stoma for ileoanal separation. Two patients required reoperations for complications caused by the diverting ileostomy. The single instance of peritonitis was in group 1 caused by anastomotic leak after ileostomy closure. There were five total complications in group 2, of which, two required temporary stomas for ileoanal separations. CONCLUSIONS: RP with rectal mucosectomy and hand-sewn IAA in children with UC provides good functional results. Peritonitis did not occur in the absence of diversion. Eliminating routine diverting ileostomy avoids the considerable complications and morbidity from the stoma and its closure.

Pneumatosis intestinalis and portal vein gas after blunt abdominal trauma.

The authors report on a 2-year-old boy in whom pneumatosis intestinalis (PI) and portal vein gas (PVG) resulted from blunt abdominal trauma after child abuse. The presumed pathophysiology of PI and PVG is mechanical in this setting. Its presence establishes mucosal injury but does not necessarily imply intestinal necrosis.

Alterations in respiratory status: early signs of severe necrotizing enterocolitis.

BACKGROUND: Necrotizing enterocolitis (NEC) presents with well-recognized signs of intestinal inflammation such as bilious vomiting, bloody stool, abdominal distension, and tenderness. The authors observed otherwise unexplained changes in the respiratory status requiring increased respiratory support during the 24 hours before direct evidence of the intestinal disorder in patients with severe NEC. METHODS: To study this observation the authors collected data on 10 consecutive patients in whom NEC required an operation. RESULTS: Eight of these patients were recovering from respiratory distress syndrome (RDS). During the 24 hours before any direct sign of intestinal dysfunction seven of these eight had a respiratory prodrome needing increased respiratory support. Two patients required intubation and mechanical ventilation. Five needed increased supplemental oxygen. This prodrome included decreased oxygenation in seven, increased respiratory rate in five, and increased PCO2 in five, preceded by hypocarbia in three. CONCLUSIONS: These changes in the respiratory condition revisit the concept of high output respiratory failure. This term was introduced to describe the respiratory failure in adult patients who suffer acute intestinal illness. Increased metabolic demand from the intestinal illness was thought to stress the ability of the patient to delivery oxygen and remove carbon dioxide. The ability of the respiratory system to meet the increased demands is limited by the intestinal dysfunction itself (abdominal pain and distension). In our patients recovering from RDS the pulmonary reserve is inherently limited. Because they are carefully monitored, it is easy to retrieve evidence of respiratory changes that precede the direct signs of intestinal disease. In the earliest stages of intestinal illness before the direct signs of intestinal dysfunction, these patients often manifest unexplained signs of respiratory compensation and decompensation and require increased respiratory support. Regardless of the pathophysiology, these alterations in respiratory status represent an early warning sign of NEC.

Extraosseous chondroma presenting as a midline neck mass in a child.

We report an extraosseous chondroma (EC) presenting as a painless midline neck mass in a healthy 3-year-old boy. EC is a benign tumor of the soft tissues; malignant degeneration has not been observed and recurrence is unusual (5%-18%). The treatment of choice is local excision. With this report, EC can be included and considered in the assessment of any firm, smooth subcutaneous mass.

Magnetic resonance appearance in a case of lipoblastomatosis.

Lipoblastoma-lipoblastomatosis represents a rare, benign tumorous proliferation of adipocytes and their mesenchymal precursor cells. Seen almost exclusively in early childhood, it should be considered in the differential diagnosis of any rapidly expanding soft-tissue mass. We present a case of lipoblastomatosis and its magnetic resonance appearance, which has not been previously described.

Intra-abdominal pulmonary sequestration exhibiting congenital cystic adenomatoid malformation. Report of a case and review of the literature.

We describe the fifth case, to our knowledge, of an intra-abdominal pulmonary sequestration that histologically displayed the features of congenital cystic adenomatoid malformation (CCAM) type 2. This mass was found during routine prenatal ultrasound in an infant with no other congenital malformations. A literature search found 13 previously reported cases of CCAM in extralobar pulmonary sequestration (EPS), nine of which were thoracic and only four were intra-abdominal. An analysis of our case and the 13 previously reported cases shows that the clinical features of EPS containing CCAM type 2 do not differ significantly from those of EPS occurring alone. The presence of CCAM type 2 in our patient is consistent with previously reported cases. Extralobar pulmonary sequestrations located in the abdomen are rarely diagnosed prior to excision, and the presence of CCAM type 2 in this situation may further compound diagnostic difficulties. The morphologic features separating CCAM into three distinct types and the exclusive association of CCAM type 2 occurring in EPS are also discussed.

True duplication of the vas deferens.

We report true duplication of the vas deferens found at routine inguinal hernia repair. This rarity is described in the context of the embryologic model for other vas abnormalities.

Retrograde jejunal-duodenal intussusception associated with a jejunal duplication cyst in a newborn.

Retrograde intussusception is a rare occurrence. We report a retrograde jejunal-duodenal intussusception associated with a jejunal duplication cyst, which presented as intermittent biliary and upper gastrointestinal (UGI) obstruction and a mobile abdominal mass in a newborn infant.

The risk of perforation when children with possible appendicitis are observed in the hospital.

The morbidity rate from perforation demands that appendicitis be diagnosed promptly in children with abdominal pain. Although admitting and observing uncertain instances of appendicitis can refine the diagnostic accuracy, it is often claimed, but not proved, that this necessitates increasing the number of perforations. To assess the risk of perforation while observing uncertain instances of childhood appendicitis, we admitted 150 consecutive referrals during a period of one and one-half years. Immediate appendectomy was performed for 74 patients (49 percent) with convincing clinical signs and symptoms for appendicitis. The remaining 76 (51 percent) with unconvincing clinical signs and symptoms were observed as inpatients. One-third of the patients admitted for observation (26 of 76) underwent appendectomy after an average period of 12 hours. Two-thirds (50 of 76) of the patients got better and were discharged from the hospital without an appendectomy after an average admission of two days. Seven of the 100 appendices removed were normal. Three of the observed patients had perforations, one of whom may have perforated during observation, but that child went on to do well. The 50 patients who got well without appendectomy were similar to the patients with appendicitis, but significantly less likely to have peritoneal signs (8 versus 70 percent), tenderness in the right lower quadrant (48 versus 81 percent) and guarding (19 versus 75 percent). We conclude that admission and active observation in the hospital of children with possible, but unconvincing, signs and symptoms of appendicitis is a safe and effective way to determine which patients need an operation.

CMV enteritis causing hemorrhage and obstruction in an infant with AIDS.

Serious gastrointestinal infections from cytomegalovirus (CMV) are often observed in immunosuppressed patients especially those with acquired immunodeficiency syndrome (AIDS). Hemorrhage and perforation have been frequent consequences. We present a case of CMV enteritis in an infant with AIDS who suffered massive hemorrhage and subsequent fatal small bowel obstruction from this condition. Gross findings at laparotomy were striking, diffuse, large yellowish plaques along the entire length of the small bowel. Each of these had a central ulceration. Each eventually caused a partial narrowing resulting in refractory small bowel obstruction. Biopsy of one of these lesions demonstrated many cells with typical cytomegalic inclusion bodies. This report illustrates a newly recognized type of CMV enteritis that can affect an infant and be readily recognized at laparotomy.

Gaucher disease: fate of the splenic remnant after partial splenectomy--a case of rapid enlargement.

In Gaucher disease, partial splenectomy has been suggested for alleviating the complications of splenomegaly as well as for avoiding the immunologic compromise and potential acceleration of bony and hepatic involvement that may follow total splenic resection. However, the fate of the splenic remnant has been reported rarely. A subtotal splenectomy (85%) was performed in a 19-month-old girl with rapidly progressing Gaucher disease and massive splenomegaly (12% of body weight). Within 3 months, the splenic remnant had increased four-fold in size. Previous reports indicated only three Gaucher patients had significant enlargement of the splenic remnant after partial splenectomy. These findings indicate that splenomegaly may recur rapidly in Gaucher disease following partial splenectomy.