Localization of insulinomas.

HYPOTHESIS: Intraoperative ultrasonography is more sensitive than preoperative and other intraoperative techniques for localizing insulinoma. DESIGN: Retrospective review. SETTING: A tertiary referral center. PATIENTS: All patients with a biochemical diagnosis of organic hyperinsulinism who were referred to University of California, San Francisco, from 1975 to 1998. METHODS: Sensitivities of the localization techniques for insulinoma were evaluated. RESULTS: The sensitivities of tumor localization with arteriography, computed tomography, preoperative ultrasonography, magnetic resonance imaging, magnetic resonance imaging with gadolinium, transhepatic venous sampling, palpation, and intraoperative ultrasonography were 47%, 24%, 50%, 30%, 40%, 55%, 76%, and 91%, respectively. Nine of the 11 nonpalpable and nonvisible tumors at operation were localized by intraoperative ultrasonography. CONCLUSION: The currently available preoperative localization tests are not reliable enough to be recommended when intraoperative ultrasonography is available.

Left paraduodenal hernia leading to ileal obstruction.

Left paraduodenal hernias are a rare cause of abdominal pain or obstruction. However, because there is high associated mortality (20 %), prompt and accurate diagnosis is essential. Because internal hernias are not detectable on physical examination, imaging is relied upon for pre-operative diagnosis. Although both computed tomography and barium studies demonstrate left paraduodenal hernias as a cluster of bowel located posterior to the stomach and to the left of the distal duodenum with absence of the normal interdigitation between loops, the findings may be subtle. Knowledge of these findings can avoid an unnecessary delay in diagnosis. We present the case of a 15-year-old girl with a left paraduodenal hernia, where initial CT and barium studies demonstrated nonobstructed jejunum within the hernia sac. Two weeks later a repeat study showed obstructed distal ileum, rather than proximal jejunum, within the sac.

Necrotizing fasciitis in two children with acute lymphoblastic leukemia.

Necrotizing fasciitis is a severe, soft tissue infection, and is an unusual condition in children. The cornerstone of therapy is prompt, aggressive surgical treatment. Despite vigorous treatment, mortality rates are high. We report the occurrence of necrotizing fasciitis in two children during the granulocytopenic phase of induction chemotherapy for acute lymphoblastic leukemia. The diagnosis and treatment of necrotizing fasciitis in these two children was made more difficult by their underlying disease and its chemotherapy. The successful treatment of their infections relied on a multimodality approach. Aggressive surgical debridement was the mainstay of therapy. Adjuvant therapy was vital to the successful outcomes and included meticulous wound care, intravenous hyperalimentation, appropriate antibiotics, and granulocyte transfusions.

Tracheostomy in children with emphasis on home care.

Tracheostomy in children is not a benign procedure. Tracheostomy-related mortality rates among children have been previously reported to be as high as 10% to 27%. Children with tracheostomies are especially vulnerable after home discharge with mortality rates of 0.5 to 2 deaths per 100 months at home. In order to assess the impact of extensive parental education and home nursing care on tracheostomy-related mortality, we report our experience over 9 years with 44 children receiving tracheostomies. Each child was maintained at home with a tracheostomy for an average of 19 months for a total of 635 months of home tracheostomy care. Indications for tracheostomy were tracheomalacia (32%), obstructive airway lesions (23%), central nervous system lesions (16%), vocal cord paralysis (9%), Pierre Robin syndrome (9%), and a list of miscellaneous conditions (11%). Our tracheostomy care regimen begins with intensive parental training in tracheostomy management for a minimum of 10 days prior to discharge. Home nursing was arranged for 77% of these children for an average of 11 hours per day at the time of discharge. Eighty-three percent had home apnea monitors. Discharge of these children was delayed or transfer to a secondary hospital was made when parents failed to show adequate proficiency in tracheostomy management with existing home nursing. Eight percent were ventilator dependent at discharge. As of January 1, 1989, 34% of these children have been decannulated. There were six deaths, all due to underlying disease. There were no tracheostomy-related deaths in hospital or after discharge home.(ABSTRACT TRUNCATED AT 250 WORDS)

Antenatal intervention for congenital cystic adenomatoid malformation.

In 2 fetuses with congenital cystic adenomatoid malformation (CCAM) with hydrops, in-utero resection of the enlarged pulmonary lobe reduced mediastinal shift and allowed expansion of normal lung tissue in both cases. In case 1, the 27-week-gestation fetus died from severe hydrops after premature delivery. In case 2, fetal CCAM resection was completed at 23 weeks' gestation. At 30 weeks a girl was delivered with no evidence of pulmonary hypoplasia. Fetal surgery can now be entertained for otherwise fatal space-occupying intrathoracic lesions.

Tracheobronchial obstructions in infants and children. Experience with 45 cases.

Forty-five infants and children with intrathoracic tracheobronchial obstructions requiring surgical treatment are reported. Segmental stenosis of the trachea is defined as involvement of less than one half the length of the airway, which affected six infants. Elongated stenosis involving more than one half the length of the trachea was seen in 12 infants, and complete annular cartilage rings, along the entire length of the trachea, were present in 11. Severe tracheomalacia occurred in six infants associated with aortic arch anomalies and in nine infants with esophageal atresia. Segmental tracheal resection was performed in 17 cases (two after failure of a rib cartilage graft), and anastomotic stricture developed in three. These three anastomotic strictures were resected, resulting in an excellent airway in two and restricture in one. Rib cartilage grafts were used in five patients: two of three with elongated stenosis with complete tracheal rings required subsequent resection, and one of two infants with tracheomalacia had excellent outcome. Approximately 50% of an infant's trachea can be resected, but rib cartilage grafts should be used for elongated stenosis. Resection of bronchial stenosis in two patients resulted in a widely patent bronchus. From this experience primary segmental tracheobronchial resection and re-resection of recurrent stenosis are highly successful. Anastomotic stricture is due to tension at the suture line and suture material inciting a fibrotic reaction. Rib cartilage grafts amounting to 25% or less of the circumference of the airway readily resurfaces with adjacent epithelium, but when 30% or more of the circumference is rib graft, epithelialization may be impaired.

Carotid artery reconstruction following extracorporeal membrane oxygenation.

Right hemispheric brain injury has been noted in surviving infants treated with venoarterial extracorporeal membrane oxygenation (ECMO). This phenomenon may be secondary to permanent ligation of the right carotid artery. At our institution, conventional ventilatory therapy failed in five neonates with respiratory insufficiency, and they were treated successfully with ECMO. In four of the five neonates, the right carotid artery was reconstructed at the time of decannulation. At discharge, all newborns with carotid artery repair showed no signs of unilateral brain injury and had excellent antegrade flow in the right carotid artery as assessed by both duplex and transcranial Doppler ultrasound scanning. Carotid artery reconstruction after ECMO is a technically simple procedure that may reduce the incidence of right hemispheric brain injury and long-term consequences of marginal cerebral perfusion.

Mediastinal pancreatic pseudocysts in children.

Mediastinal pseudocyst is an unusual complication of pancreatitis, with only four cases previously reported in children. The extent of the pseudocyst can be defined by computed tomography or magnetic resonance imaging scan and preoperative aspiration of cyst fluid for amylase level can establish the diagnosis. Endoscopic retrograde cholangiopancreatography to define ductal anatomy can help plan the appropriate drainage procedure. Although exceedingly rare, the diagnosis of pseudocyst should be considered for any cystic mass in the abdomen or thorax, even in the absence of elevated amylase or history suggesting pancreatitis.

The modified Puestow procedure for chronic relapsing pancreatitis in children.

Chronic relapsing pancreatitis in children is an unusual condition that often goes undiagnosed and untreated for years. In light of recent reports in adults that endocrine and exocrine function may be preserved by early pancreaticojejunostomy, we reviewed our experience with this procedure (one Duval, 10 Puestows) in 10 children between 1969 and 1989. The underlying etiology was familial pancreatitis in four patients, one case of unknown etiology, congenital ductal anomalies in four (one pancreas divisum, one annular pancreas, one choledochal cyst, and one ductal stenosis), and posttraumatic in one. All 10 had intractable recurrent abdominal pain. Preoperatively, only three patients evidenced exocrine insufficiency and none had endocrine insufficiency. There was complete resolution of pain in eight patients and improvement in two during a mean observation period of 4 years (range, 7 months to 19.75 years). Exocrine insufficiency resolved in two patients but has persisted in the third patient now on Viokase. Endocrine insufficiency has developed during follow-up in one patient. Pancreaticojejunostomy provides excellent relief of recurrent pain in chronic relapsing pancreatitis in children. Endoscopic retrograde cholangiopancreatography (ERCP) is indicated when the diagnosis of chronic relapsing pancreatitis is suspected to define the ductal anatomy. Pancreaticojejunostomy may prevent the progression of exocrine and endocrine insufficiency if performed early in the course of the disease.

Correction of congenital diaphragmatic hernia in utero, V. Initial clinical experience.

Review of our experience with 45 cases of prenatally diagnosed congenital diaphragmatic hernia (CDH) confirms that most fetuses (77%) will not survive despite optimal pre- and postnatal care. Polyhydramnios, associated anomalies, early diagnosis, and a large volume of herniated viscera (including liver) are associated with a particularly dismal prognosis. After extensive experimental work demonstrated the efficacy, feasibility, and safety of repair in utero, we attempted to salvage six highly selected fetuses with severe CDH by open fetal surgery. Five had liver incarcerated in the chest: three died at operation because attempts to reduce the liver compromised umbilical venous return. In one, a Goretex diaphragm was constructed around the liver, but the baby died after birth. The last two fetuses, one with incarcerated liver, were successfully repaired. Both demonstrated rapid growth of the lung in utero, had surprisingly good lung function after birth despite prematurity, had the abdominal patch removed at 2 weeks, and subsequently died of nonpulmonary problems (an unrelated nursery accident in one and intestinal complications in the other). The only maternal complication was amniotic fluid leak and preterm labor. All six women are well and four have had subsequent normal children. From this phase I experience, we conclude that fetal surgery appears safe for the mother and her reproductive potential, that fetal CDH repair is feasible in selected cases, and that the fetal lung responds quickly after decompression. However, fetal repair remains a formidable technical challenge.

Gastrointestinal tract obstruction in the fetus.

Surgical advice is often sought when a prenatal diagnosis of gastrointestinal tract obstruction is made. We reviewed our experience with 17 such cases during a 4-year period. Eight fetuses had complete proximal obstruction. Seven of the 8 did well after maternal transport to a perinatal center and prompt neonatal surgery. Six fetuses had distal obstruction with dilated bowel and increased peristalsis. Two died after birth (1 with severe associated anomalies and 1 with short-bowel syndrome), and the other 4 did well. Three fetuses had a false-positive diagnosis of in utero meconium peritonitis. Two died and the other had no postnatal evidence of obstruction. Our data suggest (1) polyhydramnios may not be present early in gestation or with distal obstruction; (2) other anomalies, including a family history of cystic fibrosis, should be sought; (3) dilated bowel with increased peristalsis is diagnostic of fetal gastrointestinal tract obstruction, whereas intra-abdominal calcification and ascites are nonspecific findings; (4) late development of ascites in a fetus with documented obstruction may be an indication for early delivery; and (5) prenatal diagnosis permits appropriate counseling, planned delivery, and prompt postnatal resuscitation and surgery with a good prognosis in most cases.

Medullary thyroid carcinoma. The need for early diagnosis and total thyroidectomy.

Forty patients with medullary thyroid carcinoma and 3 patients with C-cell hyperplasia were studied. Seventeen (40%) cases were sporadic and 26 (60%) were hereditary. Eight patients had type lla multiple endocrine neoplasia, 7 patients had type llb multiple endocrine neoplasia, and 11 patients had familial nonmultiple endocrine neoplasia medullary thyroid carcinoma. Mean follow-up was 6.3 years, with actuarial survival of 88% and 78% at 5 and 10 years (22 and 13 patients), respectively. Seven patients died 1.5 to 10 years after the initial operation; all had advanced disease at presentation (6 with distant, 1 with lymph node metastasis). No deaths occurred in patients with familial nonmultiple endocrine neoplasia medullary thyroid carcinoma, C-cell hyperplasia, or medullary thyroid carcinoma limited to the thyroid gland. Nineteen (68%) of 28 patients diagnosed without screening had regional or distant metastases, whereas only 6 (40%) of 15 patients diagnosed by screening had metastases. Twenty-six patients treated initially with total thyroidectomy and central neck clearance required an average of one reoperation, whereas those with lesser initial procedures required an average of two reoperations. We concluded that (1) familial nonmultiple endocrine neoplasia medullary thyroid carcinoma, early medullary thyroid carcinoma or C-cell hyperplasia, and asymptomatic patients have a good prognosis; (2) screening for medullary thyroid carcinoma by measuring serum calcitonin levels results in earlier diagnosis; and (3) total thyroidectomy and central neck clearance is the procedure of choice for medullary thyroid carcinoma.

Improvement in survival after excision of primary tumor in stage III neuroblastoma.

Fifty-eight patients with stage III neuroblastoma (according to the Childrens Cancer Study Group) underwent 86 operative procedures. Initial diagnostic or therapeutic operations were followed by chemotherapy and radiation therapy, and second- or third-look procedures were performed on patients whose tumors showed evidence of clinical response. Complete resection was obtained during the initial procedure in 12 patients, during the second operation in 12 others, and at the time of the third procedure in two additional children. Twenty of these 26 patients have survived with no evidence of disease for a median duration of greater than 3 years since initiation of therapy. Only nine of the 32 patients in whom complete tumor excision could not be accomplished are still alive. The chemotherapy and radiation therapy employed in this protocol may have enhanced resectability in many patients. The site of the primary tumor does not seem to influence ease of resectability or outcome in this group. The "favorable" prognostic factors of patient age and histopathology of the tumor do not necessarily predict that the tumor will be resectable. While patients with complete resection at the time of the second operation had an outcome superior to those with incomplete resections, the influence of the inherent tumor biology or other selection factors on the eventual tumor resectability in these patients is not known. Extensive operative procedures were required to completely resect stage III tumors, and a moderate complication rate may be anticipated. However, this may be justifiable if complete tumor resection can be achieved and ultimate patient survival possibly enhanced.(ABSTRACT TRUNCATED AT 250 WORDS)

Gastric infarction secondary to small bowel obstruction: a preventable complication after Nissen fundoplication.

Two infants who required a Nissen fundoplication for severe gastroesophageal reflux developed small bowel obstruction 5 and 21 months postoperatively. Their inability to belch or vomit, coupled with inappropriate airway management during resuscitation led to massive gastric dilation, and eventual infarction of their stomach, duodenum, and small intestines. These complications are preventable with appropriate management.

Ureteropelvic junction obstruction in the fetus.

Ureteropelvic junction (UPJ) obstruction is being detected with increasing frequency before birth. To clarify the natural history of fetal UPJ obstruction, we reviewed our experience managing 28 fetuses; there were 16 bilateral cases for a total of 44 renal units. None required decompression before birth. We found that fetal bilateral UPJ obstruction is associated with significant morbidity and mortality; resolution of fetal hydronephrosis secondary to UPJ obstruction is rare; antenatal diagnosis of UPJ obstruction improves clinical management by allowing early detection and appropriate treatment of otherwise clinically undetectable disease; oligohydramnios in the mature fetus with bilateral UPJ obstruction is an indication for early delivery and immediate repair; and prenatally diagnosed UPJ obstructions should be repaired as early as possible after birth.

Intracardiac extension of Wilms' tumor. A report of the National Wilms' Tumor Study.

Extension of Wilms' tumor through the inferior vena cava into the heart presents a formidable clinical challenge. Excision of such a tumor without provoking emobilization may require cardiopulmonary bypass (CPB). The completeness of excision and the likelihood of tumor embolization during operation guide subsequent radiation therapy (RT) and chemotherapy. To help define these issues, the clinical records of 15 patients enrolled in three National Wilms' Tumor Studies (NWTS) who had intracardiac tumor extension (ICE) were reviewed. The median age at diagnosis was 4 years. One patient had clear cell sarcoma (CCS); the remainder had favorable histologic findings (FH). The clinicopathologic stage was stage II in one patient, stage III in eight patients, and stage IV in six patients. ICE was detected before operation in six patients, during operation in five patients, and after operation in five patients. CPB was used in 10 patients. Eleven patients (73%) had operative complications, with major intraoperative hemorrhage occurring most often (six patients). Complications occurred less often when ICE was recognized before operation (three of six patients) than when it was not (eight of nine patients). Embolization occurred in only two patients. There were no operative deaths. The patient with CCS died. Eleven of 14 patients with FH survived, with an actuarial event-free, 2-year survival rate of 86%. There were no patients in the first NWTS. Of the six patients in the second NWTS (NWTS-2), four died (67%). All nine patients in the third NWTS (NWTS-3) survived, but follow-up was shorter (median 4 years 9 months vs. 2 years 7 months). No particular surgical procedure was associated with an increased death rate. This review suggests Wilms' tumor with ICE presents a formidable surgical undertaking but has a relatively good prognosis. Embolization is an uncommon event in ICE (two patients, 13.3%), allowing a planned operative approach. Echocardiography and ultrasonography provide accurate preoperative diagnosis. And ICE should be suspected in patients with extensive vena cava thrombosis or who have hypotension or heart failure during examination or surgery.

Home support of patients with end-stage malignant bowel obstruction using hydration and venting gastrostomy.

Palliative terminal care of patients with malignant bowel obstruction is a major clinical and ethical challenge. These patients are often mentally alert and ambulatory, but are kept in the hospital for hydration, nasogastric suction, and pain control. Parenteral nutrition requires frequent metabolic monitoring, is expensive, and is ethically questionable. We have used an alternative method of home management for 27 patients who met the following criteria: inoperable bowel obstruction due to untreatable cancer, an estimated life expectancy of between 2 weeks and 3 months, and understanding of the goals and limits of therapy. Hydration was provided by 10 percent dextrose and electrolyte solutions administered as overnight infusions through long-term central venous catheters. Thirteen patients with complete bowel obstruction required a venting gastrostomy which, when connected to passive drainage, relieved nausea and vomiting. The mean duration of survival was 64 days (range 9 to 223 days). Acceptance by patients and families was excellent, although most acknowledged increased costs due to limited insurance coverage for outpatient care. Seven patients returned to the hospital for terminal care (average stay 3.2 days), and 20 chose to die at home. The mean daily expense for fluids and supplies was +73.50, with an overall cost decrease of $900,000 compared with inpatient care. Home support with fluids and gastric venting is a humane, cost-effective alternative to in-hospital care for selected patients.

Tapering duodenoplasty for megaduodenum associated with duodenal atresia.

Six neonates with duodenal atresia and megaduodenum were treated with tapering duodenoplasty in addition to duodenoduodenostomy. This technique is simple and helps restore effective peristalsis in the proximal duodenal pouch.