David Tapper

Early recognition of neonatal abdominal wall necrotizing fasciitis

Necrotizing fasciitis (NF) of the abdominal wall occurring in newborns is associated with a 50% mortality rate. Improved survival requires early diagnosis followed by aggressive surgical débridement. During a 10-year period, we treated 7 infants who developed NF. During the same period, 32 infants were admitted with omphalitis that did not progress to NF. The patients with omphalitis and those with NF were compared. Tachycardia, abnormal white blood cell counts, induration, and violaceous skin discoloration were seen exclusively in the NF patients. Polymicrobial infections were documented in 28% of the omphalitis patients and 86% of the NF patients. All omphalitis patients survived, whereas 5 of 7 (71%) NF patients died. Adjuvant hyperbaric oxygen therapy was used for 4 infants with NF, 2 of whom survived (50%). NF is a highly morbid disease, that can be distinguished from other infant abdominal wall infections by the skin changes, white blood cell counts, heart rate, and microbiologic results. Prompt diagnosis of NF improves survival when combined with aggressive surgical débridement.

Lessons learned in the management of hemolytic uremic syndrome in children

Escherichia coli O.157:H7 is a serious and common human pathogen that can cause diarrhea, hemorrhagic colitis, and the hemolytic uremic syndrome (HUS). During a massive outbreak of infection with E coli O157:H7 in January 1993 in Washington State, more than 600 people, mostly children, acquired symptomatic infection, and 37 were hospitalized with HUS at Childrens Hospital and Medical Center in Seattle, and six at other hospitals in Washington. Twenty-one (57%) required dialysis. Nineteen (51%) had significant extrarenal pathology: gastrointestinal in 14 patients (38%), cardiovascular in 13 (35%), pulmonary in 9 (24%), and neurological in 6 (16%). Most patients were managed nonoperatively, but three required total abdominal colectomy and one a left colectomy. No child had perforation. Three patients died, all of whom had multisystem disease. The authors recommend (1) that all patients with bloody diarrhea undergo microbiological evaluation for E coli O157:H7 before any surgical intervention; (2) avoidance of antibiotics and antimotility agents in patients with proven or suspected infection with E coli O157:H7 until the safety and efficacy of such interventions have been established in controlled trials; (3) that patients with E coli O157:H7 infections be evaluated for microangiopathic changes consistent with HUS in the week after onset of diarrhea; (4) nasogastric suction for severe symptoms, and frequent abdominal evaluations, tests (electrolytes/amylase), and roentgenograms to exclude treatable abdominal disorders; and (5) institution of hemodialysis for oliguria/anuria, acidosis, or rising creatinine. The authors recommend surgical exploration for toxic megacolon, colonic perforation, acidosis unresponsive to dialysis, or recurrent signs of obstruction or colonic stricture.

Minimally invasive surgery and clinical decision-making for pediatric malignancy.

AbstractBackground: Minimally invasive surgery (MIS) is an ideal way to obtain biopsy specimens in children with cancer. We examined the safety, reliability and outcome of decisions made based on tissue obtained using MIS.nMethods: Fifty-nine oncology patients underwent 62 MIS procedures between January 1994 and July 1998. Complications, biopsy results, and outcomes were reviewed.nResults: The study population comprised 32 boys and 27 girls, with an average age of 8.8 years. There were 47 thoracoscopic and 15 laparoscopic operations. Laparoscopic procedures included initial biopsy, determination of resectability, and second-look exam. Thoracoscopic procedures included 40 lung biopsies and seven biopsies/resections of mediastinal masses. Diagnostic accuracy was 100% in all cases. No patient was found retrospectively to have been inadequately treated based on decisions made from tissue obtained by MIS.nConclusion: MIS is a safe and accurate means of obtaining tissue in pediatric oncology patients. Treatment decisions can be made accurately and with confidence using these techniques.

Improved survival with primary anastomosis in the low birth weight neonate with esophageal atresia and tracheoesophageal fistula

Management of esophageal atresia with tracheoesophageal fistula (TEF) has evolved over the past 5 years at Childrens Hospital and Medical Center, Seattle, in the premature patient weighing less than 2.5 kg. Traditionally, these low birth weight infants underwent gastrostomy and fistula ligation followed by repair after a period of weight gain. However, since 1980, of the 31 neonates weighing 2.5 kg or less with type-C TEF, a primary repair was performed in 22 within the first five days of life. There were no mortalities and only one subclinical anastomotic leak. All anastomoses were done extrapleurally in a single-layer interrupted fashion. Staged repair was elected for nine neonates. Seven had severe respiratory distress requiring mechanical ventilation and two in the staged group expired prior to repair. The survival for the entire group of 31 neonates was 94%. Primary repair may be performed safely in the premature neonate. The presence of severe pulmonary disease is the critical factor that might necessitate a staged repair.

Asymptomatic inferior vena cava abnormalities in three children withend-stage renal disease: Risk factors and screening guidelines for pretransplant diagnosis

We report two children with end‐stage renal disease (ESRD) found to have inferior vena cava (IVC) thrombosis at the time of renal transplantation. The children suffered from renal diseases that included congenital hepatic fibrosis and portal hypertension as part of their pathophysiology. Neither child had evidence of hypercoaguability or clinical symptoms of IVC thrombosis. Prior to transplantation, the renal replacement therapy consisted primarily of peritoneal dialysis. During their hospital courses, these children had central venous catheters placed for temporary hemodialysis, episodes of peritonitis and numerous abdominal surgeries. The medical literature to date has not identified a link between IVC thrombosis and portal hypertension, nor has an association between the patients’ primary renal disease and IVC thrombosis been found. We also report the finding of asymptomatic IVC narrowing in a third patient with obstructive uropathy, colonic dysmotility and numerous abdominal surgeries. IVC narrowing was diagnosed by CT scan during his pretransplant evaluation. In this paper, we consider similarities between these three patients that may have predisposed each of them to asymptomatic IVC pathology, including large‐bore central venous access as young children and/or recurrent scarring abdominal processes. A discussion regarding appropriate screening of the ‘high‐risk patient’ for IVC pathology prior to kidney transplantation and surgical options for children with this rare complication are presented.

Identification of Multiple Proliferative Growth Factors in Breast Cyst Fluid

Gross cystic disease is a common benign breast disease that is associated with a twofold to fourfold increase in breast cancer risk. Both diseases are hormonally induced and may share a common biochemical environment conducive to abnormal proliferative responses. A large collection of breast cyst fluid samples was analyzed for growth factors associated with cell proliferation: epidermal growth factor (EGF), insulin-like growth factor I (IGF-I), insulin-like growth factor II (IGF-II), platelet-derived growth factor (PDGF), transforming growth factor-alpha (TGF-alpha), and transforming growth factor-beta (TGF-beta). The data demonstrate that significant amounts of proliferative growth factors are present in breast cyst fluid of all cyst subtypes. The presence of IGF-II, PDGF, and TGF-beta in breast cyst fluid was confirmed for the first time. EGF, PDGF, and TGF-beta concentrations in breast cyst fluid were several times greater than reported for serum; IGF-I and IGF-II concentrations were several times lower. In the first 100 samples tested, no TGF-alpha was detected. Only EGF and IGF-II levels demonstrated a consistent correlation with apocrine type 1 cysts. These results demonstrated that effective concentrations of proliferative growth factors are in breast cyst fluid and suggest that adjacent breast tissue may be a probable source of synthesis. Growth factor profiles of breast cyst fluid may indicate the presence in breast tissue of a hormonal and proliferative environment permissive to subsequent cancer growth.

Individualized management of colonic atresia

Atresia of the colon represents the least common cause of neonatal intestinal obstruction, comprising less than 10 percent of gastrointestinal atresias. Eleven patients underwent surgical repair of colonic atresia during a 5 year period. Five of the patients were premature. Two had associated gastroschisis and other congenital anomalies were present in three patients. Hypaque enema was utilized to diagnose intestinal atresia and laparotomy was then undertaken. Primary repair could be performed in four patients, one with type I and three with type 3 atresias. These four patients did well and were discharged within 13 days after operation. Three deaths occurred in patients with associated anomalies, yielding a mortality rate of 28 percent. Operative therapy of colonic atresia should be individualized, based on the condition of the patient at presentation and the presence of associated birth defects.

Does posterior sagittal anorectoplasty in patients with high imperforate anus provide superior fecal continence

The posterior sagittal anorectoplasty (PSARP) has become the primary surgical procedure for patients with high imperforate anus. Very few careful long-term follow-up studies have established the superiority of the PSARP procedure over other surgical repair techniques. The authors goal was to evaluate the operation and to identify the factors associated with improved continence. The medical records, operative reports, and radiographs of 53 patients (46 male, 7 female) who underwent PSARP for high imperforate anus at Childrens Hospital and Medical Center (CHMC) between 1982 and 1990 were reviewed retrospectively. Subjective follow-up data were collected by telephone questionnaire, assessing habits indicative of stool continence, and a fecal continence score (FCS) was calculated for each patient. A prospective, 7-day diary assessing similar patterns of fecal continence was completed by each patients family. The mean age of the patients studied was 8.0 years, and the mean follow-up period was 6.8 years. The telephone questionnaire was completed for 48 patients (94%). Toilet training for bowel continence was successful in 20 patients (42%) and occasionally successful in another 20 patients (42%), but 8 patients (16%) had no awareness of impending stool. Forty-five (94%) were physically active, but 16 (33%) reported social problems related to offending odor. The mean fecal continence score for all patients was 3.0 +/- 1.4 (5=excellent, completely continent), which was nearly identical to published scores for the other types of surgical repair. The FCS did not improve with age. The parents responses to the telephone questionnaire matched the results obtained from the prospectively collected continence diary data. The authors results for PSARP in patients with high imperforate anus do not differ substantially from those achieved by other repair techniques. Previously cited prognostic factors such as fistula anatomy and sacral anomalies did not appear to alter the functional results in the authors series. Aggressive postoperative bowel management should be anticipated in all patients who have high imperforate anus and may benefit those who otherwise would achieve less satisfactory continence.

The surgical management of giant cervicofacial lymphatic malformations

Giant cervicofacial lymphatic malformation, a potentially life-threatening congenital malformation, historically has been removed in staged resections. However, complete surgical extirpation is desirable and can be achieved with the aid of a multidisciplinary team. The authors present the case of a 12-year-old girl who had an extensively enlarged cervicofacial lymphatic malformation. Effective treatment involved aggressive surgical excision, facilitated by mandibular osteotomies to provide access to the floor of the mouth, the tongue, and the pharyngeal wall. In addition, total removal of involved facial and neck skin was performed. The remaining soft tissue defect was reconstructed primarily with an abdominal musculocutaneous free flap. In the 5 years since the procedure, there has been no recurrence. Based on this experience, excision of involved skin and mandibular access osteotomies are important techniques to help completely remove cervicofacial lymphatic malformations with oral involvement, thus potentially reducing the number of recurrences. In addition, these cases require the best efforts of a multidisciplinary team to achieve a successful result so that complete and radical excision can be performed with the hope of avoiding the pitfalls of multiple resections.

Early diagnosis and management of renovascular hypertension

Renovascular hypertension is more common in hypertensive children than in hypertensive adults, and renal artery stenosis is second only to coarctation of the thoracic aorta as a cause of surgically correctable hypertension. Three infants presented with uncontrollable hypertension secondary to renal artery thrombosis due to umbilical artery catheterization for respiratory distress in the neonatal period. They all responded to nephrectomy. A fourth infant had stenosis of a polar vessel secondary to umbilical artery catheterization and was cured by partial nephrectomy. Two infants with renal artery stenosis secondary to fibromuscular dysplasia benefited from revascularization and, at last follow-up, were normotensive and off all blood pressure medication. Ultrasonography, isotope scanning, angiography and selective renal vein renin assays should be used to identify patients with surgically correctable lesions. The use of fine suture material and microvascular surgical techniques, including ex vivo revascularization and autotransplantation, can salvage renal parenchyma and relieve hypertension. Infants with less than 10 percent renal function on the involved side should have a nephrectomy. The infant with an umbilical arterial catheterization line needs blood pressure monitoring and aggressive evaluation and treatment of persistent hypertension.