Howard C. Filston

Malrotation—The ubiquitous anomaly

Although anomalies of rotation and fixation of the gastrointestinal tract are recognized to be associated frequently with anomalies of the abdominal wall and diaphragmatic hernia, the frequency with which these common anomalies are associated with other congenital and acquired lesions of the gastrointestinal tract has not been sufficiently emphasized. Sixty-two percent of our series of 34 patients were associated with such lesions as upper gastrointestinal atresias and stenoses, intussusception, and Hirschsprungs disease. The embryologic and anatomic features of rotational anomalies, their clinical presentation, and radiologic features are presented with the suggestion that knowledge of this information, along with the appreciation of the frequent association of malrotation with other common congenital and acquired abnormalities, will help to prevent the devastating morbidity and mortality of undiagnosed midgut volvulus.

Elective cholecystectomy in children with sickle hemoglobinopathies. Successful outcome using a preoperative transfusion regimen.

Twenty-seven children with major sickle hemoglobinopathies underwent elective cholecystectomy for cholelithiasis. All were managed with a preoperative transfusion regimen to achieve a hemoglobin concentration of 11-14 g/dl with greater than 65% hemoglobin A. Intraoperative cholangiography revealed common bile duct stones in five patients, although only one case was diagnosed by preoperative ultrasonographic examination. Twenty-four children underwent incidental appendectomy by total intussusception. There were no vaso-occlusive events nor any other perioperative morbidity or mortality. Four months after cholecystectomy, one boy had a small bowel obstruction requiring surgical re-exploration. No patients had transfusion-acquired infection, although one boy had erythrocyte allosensitization to Lewis A antigen. This preoperative transfusion regimen and careful perioperative management permits safe elective cholecystectomy in children with sickle cell disease.

The Currarino triad: complex of anorectal malformation, sacral bony abnormality, and presacral mass

The Currarino triad is a unique complex of congenital caudal anomalies including anorectal malformation, sacral bony abnormality, and presacral mass. The usual symptomatology is constipation due to anorectal stenosis. Contrast enema and computed tomographic myelography are the imaging modalities of choice for diagnostic confirmation and clarification of the anomalies. The clinical features, unique radiologic appearance, and importance of a correct diagnosis of the Currarino triad are reviewed.

Occult spinal dysraphism in patients with anal agenesis

Recent reports have suggested an association between congenital anorectal anomalies and occult spinal dysraphism. Eighty-seven patients with anal agenesis have been treated at this institution over the last 14 years. Two of these patients had spinal cord anomalies recognized at birth (a myelomeningocele and a tethered spinal cord). Two additional patients presented with progressive neurologic deficits in early childhood and were each found to have a tethered spinal cord. To further assess the magnitude of this problem, we have used magnetic resonance imaging (MRI) of the spine to survey prospectively 23 infants with anal agenesis. Twenty-one former patients who were asymptomatic were recalled and also studied. Four of 44 patients (9%) were found to have significant occult spinal dysraphism; each child had undergone neurosurgical operation without morbidity. MRI found each child to have a tethered spinal cord, either as an isolated lesion (2) or in association with a syrinx (1) or lipomyelomeningocele (1). One of these patients had a neurologic deficit detected on careful preoperative evaluation. The other three, two of whom were less than 2 years old, had no detectable deficit. Neither the extent of the anorectal malformation, the absence of associated congenital anomalies, nor the demonstration of normal vertebral anatomy on plain radiographs of the spine precluded the presence of occult spinal dysraphism. Therefore, we recommend that all patients with anorectal anomalies undergo MRI imaging of their spines during initial evaluation to screen for occult spinal dysraphism. In addition, consideration should be given to recalling older patients for MRI evaluation.

Controlled study of treatment for disseminated intravascular coagulation in the neonate

Thirty-three neonates with disseminated intravascular coagulation were assigned randomly to one of three treatment groups: (1) exchange transfusion, (2) administration of fresh-frozen plasma and platelets, and (3) control (no therapy directed specifically at the coagulopathy). The three groups were comparable for degree of abnormality in initial coagulation studies and underlying pathologic processes. Shock was a common accompaniment of DIC and occurred in 85% of all infants. In all cases, underlying disease states and shock were treated aggressively. Resolution of DIC and survival were not different in the three treatment groups. Outcome of DIC was dependent on the success of treatment of the underlying pathologic process and aggressive supportive care, including restoration of blood pressure, but was not altered by therapy specifically directed at the coagulopathy.

A safer system for percutaneous subclavian venous catheterization in newborn infants.

Percutaneous infraclavicular cannulation of the subclavian vein in even the tiniest infants can be performed with safety and ease using a plastic cannula metal needle stylet and following plastic catheter. This route for achieving superior vena caval catheterization provides a most satisfactory method for central pressure monitoring, long term antibiotic administration, total parenteral nutrition, and exchange transfusion. Few serious complications have been noted in over a hundred infant cannulations. The procedure can be performed in the intensive care nursery with a success rate of over 95%. Sepsis rates in clean cases are acceptably low. Most sepsis is due to other clearly identifiable sources of infection in these already critically ill infants.

Diagnosis and management of common bile duct stones in patients with sickle hemoglobinopathies

Nine of 35 patients with sickle hemoglobinopathies and cholelithiasis were found to have concomitant common bile duct (CBD) stones. We describe the diagnosis and management of these patients with CBD stones. Historical information, physical examination, and routine laboratory tests were unable to identify accurately the patients with CBD stones. Similarly, ultrasonography of the hepatobiliary system was an insensitive method for detecting CBD stones, as only 3 of 8 patients were correctly identified. In contrast, both an intraoperative cholangiogram and endoscopic retrograde cholangiopancreatography were sensitive procedures for detection of CBD stones. We conclude that CBD stones are relatively common in patients with sickle cell disease and cholelithiasis, and clinicians should have a high index of suspicion for their presence.

Juvenile secretory carcinoma and juvenile papillomatosis: Diagnosis and treatment

Carcinoma of the breast in the child is a rare pathologic entity, constitutes less than 1% of all breast lesions in this age group. The case of a girl with a left breast mass first noticed at 4.5 years of age is presented. An eccentric subareolar mass was excised at 6 years of age, sparing her breast bud. This biopsy contained juvenile secretory carcinoma, with additional pathologic changes characteristic of juvenile papillomatosis. Both the juvenile papillomatosis component as well as the juvenile secretory carcinoma component were devoid of estrogen and progesterone receptors in contrast to nondiseased breast tissue. The assay for these receptors was performed using a new antireceptor monoclonal antibody technique. Simple mastectomy was performed after completion excision demonstrated tumor at the pectoralis major fascia.

Prognostic Factors and Contribution of Preoperative Telescopic Endoscopy

In recent years, the surgical correction of esophageal atresia with distal tracheoesophageal fistula (TEF) has become increasingly successful. However, there remains a group of high-risk patients with specific anatomical abnormalities in whom the mortality remains appreciable. These associated disorders include cardiac, renal, and chromosomal anomalies as well as severe respiratory distress syndrome. These factors, rather than low birth weight or early gestational age, are primarily responsible for surgical mortality. Preoperative telescopic bronchoscopy has been a useful adjuvant confirming the diagnosis, identifying unusual variants, and permitting the proper anatomic placement of the endotracheal tube. A Fogarty balloon catheter can be passed bronchoscopically into the distal TEF in patients with severe respiratory distress syndrome to occlude the fistula and facilitate effective positive pressure ventilation. Thirty-two patients were treated for esophageal atresia among whom 28 had esophageal atresia with distal TEF, three had esophageal atresia alone, and one had esophageal atresia with proximal TEF. The higher-risk group comprised those with severe respiratory insufficiency as evidenced by a room air paO2 of less than 60 mmHg; this group accounted for nine of the ten deaths in the total series. There was one late death following surgical correction. In summary, in the absence of severe respiratory insufficiency or associated life-threatening congenital anomalies, the results of surgical correction for esophageal atresia are remarkably good and survival in this group approximates 100%.

Obstructive jaundice due to gastric trichobezoar

Abstract Among the causes of obstructive jaundice in childhood, a foreign body of the gastrointestinal tract is rare. Biliary tract obstruction can be caused by a foreign body large enough to occlude the periampullary portion of the duodenum and ampulla of Vater. When this occurs, obstructive symptoms of the proximal GI tract usually dominate the clinical picture. Gastric bezoars have been known to attain enormous size and frequently have “tails” that extend into the small bowel. Common complications of gastric and intestinal bezoars are obstruction, ulceration, and perforation, but biliary tract obstruction to our knowledge has not yet been reported. The atypical history and confusing laboratory tests in a jaundiced infant delayed diagnosis and treatment.