To compare histopathology with ADC values in strictured bowel segments in pediatric patients with known Crohn’s disease and surgical bowel resection.

Expression of CD43 by B cells is often used as a diagnostic criterion in favor of a B-cell lymphoproliferative disorder, including small lymphocytic lymphoma/chronic lymphocytic leukemia, mantle cell lymphoma, Burkitt lymphoma, precursor B-lymphoblastic lymphoma, and a subset of marginal zone B-cell lymphomas. Benign B cells generally do not coexpress CD43. The authors analyzed 20 biopsies of the terminal ileum for nonneoplastic disease for expression of CD43 and compared them with other sites and with CD20, CD138, and CD3 reactivity. The majority of cases (85%) showed strong coexpression of CD43 by benign perifollicular B cells. The presence of CD43 coexpression in B-cell populations of the terminal ileum, including those of Peyer’s patches, should not be used as a diagnostic parameter to differentiate extranodal marginal zone B-cell lymphoma of MALT type from reactive processes.

The authors describe a 10-week-old girl with infantile hepatic hemangioendothelioma who initially presented with difficulty feeding, hepatomegaly, and multiple hemangiomas of the skin. Six weeks of steroid therapy and 2 weeks of chemotherapy failed to produce clinical improvement. The patient underwent split liver transplantation. A definitive diagnosis of hemangioendothelioma type II was made. Imaging studies cannot differentiate between hemangioendothelioma and angiosarcoma. Treatment modalities for this condition remain unclear. The patient continues to do well.

Preeclampsia was reported to be a disorder of a faulty trophoblastic invasion and increased apoptosis. We previously demonstrated that soluble Fas is associated with preeclampsia. We hypothesize that Fas gene expression in the placenta is responsible for trophoblastic invasion in normal or preeclamtic pregnancies. We investigated the association between Fas-670 and Fas ligand (FasL)-124 gene polymorphisms in preeclamptic and normal pregnancies. Forty-eight singleton pregnancy placentas were studied. Twenty-four placentas were with preeclampsia and 24 were normotensive controls. DNA was extracted from placentas and analyzed by polymerase chain reaction-based restriction fragment length polymorphism for an adenine (A) to guanine (G) substitution at position -670 in the Fas promoter gene and at position -124 in the FasL gene. Chi-square and Fisher's exact tests were used for statistical analysis. There were no significant differences in race, parity, or maternal age between two groups. No Fas-670 AA wild genotype was observed in both normal control and preeclamptic placentas. In normal controls, placental Fas-670 GG mutation genotype was dominant and significantly more frequent than in preeclamptic placentas (92% vs. 21%, P < .0001). In preeclmaptic placentas, Fas-670 AG heterozygous genotype was significantly more frequent than in normal controls (79% vs. 8%, P < .0001). In contrast, both normal controls (except one) and preeclamptic placentas expressed Fas ligand-124 AA wild genotype. (1) Lack of expression of Fas-670 AA wild genotype indicates placenta to be a unique immunoprivileged organ; (2) Predominant Fas -670 homozygous GG mutation gene expression in normal pregnancy placentas may serve a protective role against apoptosis during trophoblastic invasion; (3) Genetically predetermined Fas -670 AG genotype appears to be associated with the risk for preeclampsia; (4) Fas polymorphism plays a more important role than FasL polymorphism in the pathogenesis of preelcampsia.

An unusual case of cavitary Rhodococcus equi pneumonia with endobronchial granulomas in congenital HIV infection is presented. The clinical features and radiological manifestations of pulmonary R. equi infection are discussed.

A 15-year-old adolescent girl with a history of congenital acquired immunodeficiency syndrome (AIDS) presented with severe respiratory distress. The patient had had an episode of left lower lobe pneumonia 1 month earlier, for which she had been admitted to another hospital and treated with multiple antibiotics. Two weeks after discharge from that hospital, she developed progressive shortness of breath, chest pain, and cough and was admitted to Westchester Medical Center. On examination, she was anemic, malnourished, and moderately dehydrated. Breath sounds were decreased at the left base, with crackles and wheezes. The abdomen was diffusely tender, with hepatosplenomegaly. A chest radiograph and computed tomography scan revealed a large cavitary lesion in the left lower lobe of the lung. Flexible bronchoscopy revealed near-complete obliteration of the left main bronchus and partial obstruction of the right bronchus. A lung biopsy obtained through the bronchoscope revealed granulomatous inflammation consisting of sheets of epithelioid macrophages containing round intracytoplasmic microorganisms (Figure 1). Acid-fast bacillus, Fite, and periodic acid–Schiff stains were used, all of which produced negative results. A Gram stain revealed intracytoplasmic gram-positive coccobacilli (Figure 2). A blood culture also grew gram-positive coccobacilli (Figure 3), which were positive with the Christie/Atkins/Munch-Peterson test. The patient received aggressive treatment with multiple antibiotics in addition to all supportive measures but developed multiorgan failure and died 3 days after admission.Examination of the lungs at autopsy revealed a 13-cm well-circumscribed gray-white cavity in the left lower lobe (Figure 4). The cavity was filled with necrotic material mixed with clotted blood. Histologic sections of the left lung had extensive necrotizing pneumonia with cavity formation.What is your diagnosis?The cavitary pneumonia in this AIDS patient was caused by Rhodococcus equi. Common etiologic agents of cavitary lesions in the lung in patients with AIDS include gram-positive cocci (especially Staphylococcus), fungi, and tubercle bacilli including Mycobacterium tuberculosis and M avium intracellularae. A definitive and prompt diagnosis is essential for appropriate treatment. The common diagnostic approach includes imaging studies, blood culture, or fine-needle aspiration of solid areas. A histologic diagnosis can be made via lung biopsy. Cytologic examination of bronchoalveolar lavage fluid, using routine and Fite stains, is useful in the diagnosis of R equi infection.1 Blood cultures are the criterion standard in diagnosis of R equi sepsis in a patient with rhodococcal pneumonia, as in this case.In lung biopsy material, the typical lesion of R equi infection is composed of epithelioid macrophages with pseudotumor formation or cavitation. The macrophages contain coccobacillary organisms, which may be seen in hematoxylin-eosin–stained sections but are also gram positive and may be acid fast with a Fite stain. A malacoplakia-like lesion has been described in persistent cases of treated R equi pneumonia.2Although uncommon in immunocompetent human hosts,3 rhodococcal infection has been described in immunocompromised patients, especially those with human immunodeficiency virus infection.45 Although many organs may become infected, including skin, peritoneum, gastrointestinal tract,6 eye, and prostate, pulmonary involvement is most common.7Rhodococcus belongs to the group of aerobic actinomycetes and, like these organisms, is a gram-positive, aerobic, catalase-positive, partially acid-fast filamentous bacteria that can fragment into rods and cocci. The most common pathogenic species in humans is R equi, which is a facultative intracellular bacterium that can survive and replicate in macrophages. Serotyping has been reported in animal isolates but is not yet possible in human isolates.8 The organism is identified in the laboratory by a combination of staining properties, colony morphology, and biochemical tests.7 The colonies typically show mucoid growth with salmon-pink pigment production. The organism has been isolated from both animal feces and soil. The usual mode of infection is inhalation, but a few cases have resulted from direct contact with a skin lesion.9Rhodococcal infection is treated by combination antibiotic therapy.5 The organism is usually sensitive to erythromycin, rifampin, ciprofloxacin, vancomycin, aminoglycosides, imipenem, and meropenem.3 However, resistance has been reported to penicillin G, oxacillin, ampicillin, carbenecillin, and cefazolin.3 The prognosis depends in part on the immune status of the host. In patients with AIDS, mortality ranges between 50% and 55% compared with 20% to 25% in immunocompromised patients without AIDS and 10% in immunocompetent patients.3

Renal tubular dysgenesis (RTD) is a rare form of noncystic renal disease characterized by paucity or absence of proximal renal tubules. Always lethal in the perinatal period, it has been associated with Potter sequence and with other congenital malformations. An autosomal recessive inheritance has been suggested. We present a case of renal tubular dysgenesis associated with fetal hydrops and trisomy 21, with a review of relevant literature.

Primary rectal lymphoma in childhood is rare. We report a case in a 10-year-old boy who presented with rectal bleeding and a single rectal polyp. Histologic examination, immunophenotyping and molecular genetic study of the polyp showed a diffuse B-cell lymphoma, Burkitt-like type. The literature on this topic is reviewed and pathologic examination of childhood rectal polyps is emphasized.

ABSTRACTBackgroundLiver biopsy findings are important in diagnosing extrahepatic biliary atresia. Diffuse ductular proliferation is a characteristic finding. We describe four patients with conjugated hyperbilirubinemia in whom the initial liver biopsy findings showed a lack of ductular proliferation, despite subsequent development of biliary atresia.ResultsOn initial biopsy, paucity of intrahepatic bile ducts was present in three of four patients, with a bile duct to portal space ratio of 0.3 to 0.4 (normal, 0.9–1.8). A normal bile duct to portal space ratio of 1.0 was observed in the fourth patient. Ductular proliferation became apparent in three subjects between 9 and 12 weeks of age, and biliary atresia was noted at the time of a Kasai portoenterostomy. The fourth child had well‐developed biliary cirrhosis at liver transplantation.ConclusionsChanges characteristic of biliary atresia may appear even after 9 weeks of age. Bile duct paucity and normal bile duct to portal space ratio do not preclude the subsequent development of biliary atresia. Infants with unexplained conjugated hyperbilirubinemia and acholic stools should undergo sequential liver biopsies until clinical improvement occurs or until biliary atresia can be excluded from the differential diagnosis.

Objective: To determine the perinatal effects of histologic chorioamnionitis on preterm neonates and the effectiveness of antenatal steroids in the presence of histologic chorioamnionitis.Methods: We studied neonates at our institution who weighed 1750 g or less at birth from January 1990 through December 1997. The population was stratified primarily by presence of histologic chorioamnionitis and secondarily by exposure to antenatal steroids. Subgroups were compared by various perinatal outcomes and confounding variables. Student t test, chi(2), Fisher exact test, and logistic regression were used for analysis.Results: Among 1260 neonates entered, the placentas of 527 had evidence of histologic chorioamnionitis and 733 did not. Those with histologic chorioamnionitis had a lower mean gestational age, lower birth weight, and higher rate of major neonatal morbidities than those without it. After adjusting for confounding variables, histologic chorioamnionitis independently associated with lower gestational age, lower birth weight, and neonatal death. Among neonates exposed to antenatal steroids who had histologic chorioamnionitis, there was a significantly lower incidence of low Apgar scores (18% compared with 33.5%, P <.001), respiratory distress syndrome (RDS) (39.6% compared with 55.9%, P <.001), intraventricular hemorrhage and periventricular leukomalacia (21.9% compared with 36.9%, P <.001), major brain lesions (7.7% compared with 18.4%, P < .001), patent ductus arteriosus (14.8% compared with 23.7%, P = .018), and neonatal death (8.3% compared with 16.2%, P = .02), with no increase in rate of proven neonatal sepsis (18.3% compared with 14%, P = .24).Conclusion: Histologic chorioamnionitis increases major perinatal morbidity through its association with preterm birth and is independently associated with neonatal death. In the presence of histologic chorioamnionitis, antenatal steroids significantly decreased the incidence of RDS, intraventricular hemorrhage and periventricular leukomalacia, major brain lesions, and neonatal mortality, without increasing neonatal sepsis. (Obstet Gynecol 2000;96:333-6. (C) 2000 by The American College of Obstetricians and Gynecologists).

Three newborn infants of mothers with insulin dependent diabetes mellitus (IDDMm) were found to have multiple congenital malformations consisting of 3 or more VATER ascertainment anomalies. In addition, each of the 3 infants also had left lateral abdominal wall hernias, not previously noted in VATER or IDDMm infants. The hernias had intact overlying skin, were easily reducible and approximately 5-8 cm in diameter. Abdominal wall hernias with intact overlying skin are unusual, especially on the left side. One infant came to autopsy and was found to have hypoplasia (amyoplasia) of the abdominal wall musculature. Each of these 3 infants also had bilateral toe pre-axial polydactyly which has been rarely reported in VATER or in IDDMm infants. While there is overlap in the anomalies of VATER Association and infants of IDDMm, these 3 infants of IDDMm suggest that the phenotypes are distinct with separate etiologies. In VATER, an early insult to the allantois and subsequent vascular consequences have been etiologically implicated. The pre-axial toe potydactyly and abdominal wall hypoplasias are related to mesenchyme. The presence of macrosomia and increase in number of cells with IDDM can result in excess mesenchyme at the time of limb bud development which has been linked to polydactyly. The focal abdominal wall amyoplasia derives from myotomal mesoderm which migrates from 3 directions to fuse in the midline. An excess of mesodermal cells can cause imperfect fusion and migration resulting in a focal defect.

We report the cytogenetic findings in a Wilms tumor from a 4-year-old boy. Karyotypic analysis revealed isochromosomes of 7q and 17q as coexisting clonal aberrations. The finding is notable in view of recent reports of i(7q) as a nonrandom event in Wilms tumor and the emerging evidence for genetic heterogeneity in this tumor.

Polypoid lesions of the gallbladder in children are rare. We report a case of a gallbladder polyp in a 14-year-old boy who presented with recurrent right upper quadrant abdominal pain. Ultrasound examination of the abdomen revealed a polypoid lesion of the gallbladder. His symptoms resolved after laparoscopic cholecystectomy. Histological examination of the gallbladder demonstrated a benign adenomatous polyp. Although the experience with polypoid lesions of the gallbladder in children is limited, we currently recommend cholecystectomy because these lesions are associated with acalculous cholecystitis, and because their long-term effects are unknown.

BACKGROUND. Prenatally diagnosed neuroblastomas have been reported in increasing numbers over the past several years, and there are now a few reviews based on up to 21 cases. The purpose of this article is to review the clinical and biologic features of prenatally diagnosed neuroblastoma based on a review of 55 cases.METHODS. A review was conducted of 3 cases seen at the study institution and 52 other cases reported thus far in the literature.RESULTS. Prenatal diagnosis was made usually after 32 weeks of gestation. Approximately 93% of the tumors were adrenal in origin, and 44% of these were cystic. Thirty-seven patients (67%) had Stage I disease, 12 (22%) had Stage IV-S disease, and only 3 (5%) had Stage IV disease. The DNA index was favorable (>1) in 14 of 16 patients studied. None of these 16 patients studied had amplification of the N-myc oncogene. Catecholamines were elevated in only 33% of the patients. The liver was the most common site of dissemination, which was observed in 25% of patients; bone involvement was not observed in any patient. Ultrasonography failed to detect existing hepatic metastasis in three patients. Primary surgical resec tion was performed in 47 patients (85%). Chemotherapy was given to five patients and radiotherapy to three. Of the 50 patients for whom follow-up information was available, 45 (90%) were alive at a range of 2-120 months from diagnosis.CONCLUSIONS. Prenatally diagnosed neuroblastomas are predominantly adrenal in origin and frequently cystic. The liver is the most common site of dissemination and bone involvement is notably absent. The vast majority of these infants have a favorable stage of disease (I, II, and IV-S) and favorable biologic features, and consequently have an excellent prognosis. Although surgery alone is curative for most patients, a period of observation may avoid surgery in some individuals who may achieve spontaneous regression. (C) 1997 American Cancer Society.

Intrascrotal and testicular masses in the pediatric population do not usually present as treatment dilemmas. Herein, we report an unusual case of an enlarging, intrascrotal capillary hemangioendothelioma in a 3-month-old male infant. Conservative management including watchful waiting in the case of purely cutaneous scrotal hemangiomas is the treatment of choice. However, scrotal lesions with a palpable testicular or scrotal mass do not lend themselves to conservative treatment and, as in this case, exploration with intraoperative evaluation and excision is warranted.

We report a case of congenital pulmonary myofibroblastic tumor, and review prior reports of this rare neoplasm to demonstrate its clinically benign behavior despite histologic features previously interpreted as sarcoma. The patient, a female neonate, presented with severe respiratory distress after cesarean section delivery. A large radio-opaque mass was detected in the right hemithorax and resected by right bilobectomy. The tumor mass, confined to the lung, was composed of interlacing fascicles of plump spindle cells showing myofibroblastic differentiation and complex cytogenetic abnormalities. Though sarcomatous in appearance, with highly cellular areas and numerous mitoses, there has been neither tumor recurrence nor metastases. The patient remains alive and well 1 year after surgery. Review of the few other reported cases confirms the uniformly benign behavior of this tumor.

OBJECTIVE: Neonatal intraventricular hemorrhage and periventricular leukomalacia have a strong correlation with eventual neurologic deficit. Our objective was to correlate obstetric factors with the development of these lesions.STUDY DESIGN: Seven hundred forty-five consecutive inborn neonates with birth weights from 500 to 1750 gm were divided into three clinical groups: premature rupture of membranes, refractory preterm labor with intact membranes, and delivery initiated by the physician for maternal or fetal indications. Neonatal neurosonography was performed on days 3 and 7 of life and results were described as normal or abnormal. Abnormal scans included intraventricular hemorrhage seen within 3 days and echodense or echolucent periventricular leukomalacia seen within 7 days of life. Major abnormalities included intraventricular hemorrhage grades 3 and 4, intraventricular hemorrhage with periventricular leukomalacia, and echolucent periventricular leukomalacia. Abnormal scans were correlated with groups of origin and clinical and histologic chorioamnionitis.RESULTS: Abnormal scans occurred in 33% of cases of premature rupture of membranes and in 38.9% of cases of preterm labor compared with 17.7% of physician-initiated cases (p < 0.000001). Major lesions occurred in 17.6% of cases of premature rupture of membranes, 21.4% of cases of preterm labor, and 1.1% of physician-initiated cases (p < 0.0000001). Clinical chorioamnionitis occurred in 19.7% of cases of premature rupture of membranes, 11.9% of cases of preterm labor, and 1.1% of physician-initiated cases (p < 0.001) and was associated with a significant increase in the incidence (p less than or equal to 0.005) and severity (p less than or equal to 0.007) of these lesions. Histologic chorioamnionitis occurred in 59.9% of cases of premature rupture of membranes, 43.2% of cases of preterm labor, and 8% of physician-initiated cases and did not correlate significantly with the incidence or severity of abnormal scans. These findings were independent of gestational age.CONCLUSIONS: The incidence and severity of intraventricular hemorrhage and periventricular leukomalacia were significantly increased in premature rupture of membranes and preterm labor compared with the physician-initiated cases. Clinical chorioamnionitis increased the incidence and severity of these lesions.

Achalasia is a motor disorder of the esophagus that presents clinically as a functional obstruction at the esophagogastric junction. Although uncommon, its occurrence in children is well described (1). Achalasia can be complicated by esophagitis, thought to be secondary to the functional obstruction and stasis (2). The following case presentation is that of a child with achalasia and Candida esophagitis. We believe that the Candida esophagitis occurred as a consequence of achalasia. CASE REPORT The patient was an 11-year-old white boy hospitalized following a 4-month history of dysphagia and weight loss. Dysphagia was worse for solids and was associated with chest pain and the regurgitation of meals. His chest pain was retrosternal, sharp, and short in duration and occurred only following food ingestion. He lost 8 lb in the 2 weeks prior to presentation. The patient had intermittent fever as high as 102°F for the 2 months prior to presentation. Fever occurred approximately 1-2 weeks apart, and febrile periods were associated with chills and malaise. His fever would last for no longer than 1 day. The patient also complained of intermittent nocturnal cough. Past medical history and family history were unremarkable. There was no history of foreign travel. On physical examination, he was well-developed, thin, and in no distress. His heart rate was 90/min, respiratory rate 18/min, blood pressure 110/68, and temperature 98°F. His height was 151 cm (75th percentile) and weight 33 kg (25th-50th percentile). Examinations of the head, ears, eyes, nose, and throat were normal. His chest was clear and heart sounds were regular, without murmur. The abdomen was soft, nontender, nondistended, with no hepatosplenomegaly or mass. On rectal examination, his stool tested Hemoccult-negative. His neurologic examination was normal. Admission laboratory values included a complete blood count (CBC) with a white count of 7,800/mm3, hematocrit of 44.6%, and 323,000 platelets/mm3. The differential count was 63% neutrophils, 29% lymphocytes, 6% monocytes, and 2% eosinophils. Serum electrolytes, liver function tests, and coagulation profile were normal. His erythrocyte sedimentation rate (ESR) was elevated to a value of 40 mm/h, and thyroid function tests, antinuclear antibody (ANA), immunoglobulins, HIV testing, monospot, and Lyme titers were all negative. The patient had an upper gastrointestinal (GI) series performed that demonstrated a dilated esophagus with tapering at the lower esophageal junction, which ended in a beaked appearance. Esophageal manometry, using the station pull-through technique (3), demonstrated no relaxation of the lower esophageal sphincter (LES), an elevated LES pressure of 50 mm Hg, aperistalsis, and an intraesophageal pressure which was higher than intragastric pressure. The esophagographic and manometric findings were consistent with achalasia. Esophagogastroduodenoscopy demonstrated a normal duodenum and stomach. The esophagus, however, was covered with a thick, white exudate, and the underlying mucosa appeared erythematous and friable. Biopsies of the esophagus were obtained for histologic evaluation, and mucosal brushings for gram stain and cytology were collected. Esophageal biopsies (Fig. 1) and mucosal brushings demonstrated the presence of Candida esophagitis. The patient was started on therapy with fluconazole, 150 mg once daily, as well as nifedipine, 10 mg 20 min prior to each meal. This combination therapy resulted in significant improvement of his dysphagia. In the course of his hospitalization, the patient's temperature rose to 103°F, at which time blood cultures, urine cultures, and a chest x-ray were all negative. When he was able to tolerate a regular diet, he was discharged from the hospital and gained 6 lb over the ensuing 2 weeks. Thereafter, fluconazole was discontinued and the patient was followed over the next 3 months while remaining on nifedipine. Repeat esophagoscopy was performed after discontinuation of the fluconazole and demonstrated complete resolution of the Candida esophagitis. Biopsies of the esophagus were normal. However, the patient continued to have mild dysphagia and nocturnal coughing. A repeat manometric study demonstrated findings consistent with achalasia. The patient, therefore, had esophageal dilatation, using a Microvasive/Rigiflex pneumatic balloon dilator, with a balloon length of 10 cm and outer diameter of 35 mm. A pressure of 15 psi was sustained for 1 min. This dilatation resulted in a complete resolution of his dysphagia and nocturnal cough, and the patient remained asymptomatic for the following 12 months. DISCUSSION This is a report of an 11-year-old child presenting with Candida esophagitis and achalasia, in the absence of predisposing factors such as immunosuppresive or antibiotic therapy or an underlying immunodeficiency. Immunocompetent individuals, such as this patient, have also been reported with fungal or viral esophagitis (4-7). The finding that the achalasia persisted despite resolution of the Candida esophagitis suggests that achalasia was likely his primary disorder. This association has been described in two case reports: in one, a 73-year-old woman with achalasia and Candida esophagitis also had a mediastinal tumor, which may have affected the immunologic status of the patient and therefore been a predisposing factor for Candidal growth (8,9). In a review of the use of pneumatic dilatation for treatment of achalasia in children, it was noted that one child in eight grew Candida from lower esophageal washings; nevertheless, esophagitis was not present in that patient (10). Candida is a commensal organism in the GI tract, and therefore its presence in that case may not have been significant. In a prospective study of Candida esophagitis, achalasia was described in one of 27 patients. No specific information, however, was given regarding other predisposing factors for Candida esophagitis in this patient (11). Esophagoscopy is the most sensitive and specific method for establishing the diagnosis of Candida esophagitis (4,11-13). The visual appearance is highly characteristic and is seen as patchy, creamywhite plaques which cover a very friable erythematous esophageal mucosa (13). Accepted diagnostic criteria for Candida esophagitis include the characteristic endoscopic appearance, microscopic evidence of pseudohyphae in endoscopic mucosal brushings, or invasive candidiasis on biopsy (13). The gross endoscopic appearance, mucosal brushings, and histologic findings in our patient were consistent with Candida esophagitis. Although we did not obtain fungal cultures, they are not necessary for the diagnosis, providing the other criteria are met. The relationship between esophageal dysmotility and the presence of Candida in the esophagus has been described in adults with progressive systemic sclerosis (PSS). The characteristic lesion of PSS is smooth muscle atrophy, which results in impaired peristalsis of the lower two-thirds of the esophagus and diminished or absent LES pressure (14). Gastroesophageal reflux (GER) and reflux esophagitis are, accordingly, commonly seen in these patients. Esophageal dysmotility secondary to PSS is thought to be responsible for the development of Candida esophagitis. Interestingly, antireflux therapy, using inhibitors of gastric acid secretion, resulted in increased candidal growth, thought to be related to the resulting increase in esophageal pH (14). In contrast, a study of adults with scleroderma-related erosive esophagitis showed that, while all patients had loss of peristalsis in the distal esophagus, the results of fungal cultures were similar to those from patients with scleroderma but without esophagitis (12). These data suggest that esophagitis is not necessary for candidal growth. We were unable to identify the cause of fever and elevated ESR in our patient. However, since both fever and nocturnal cough resolved after pneumatic dilatation, we speculate that they were a consequence of recurrent microaspiration of esophageal content secondary to achalasia. Microaspiration in achalasia is associated with pulmonary symptoms such as recurrent pneumonia, chronic bronchitis, chronic productive cough, and severe asthma (1). While there are no reports of fever associated solely with Candida esophagitis, fever has been described in a child with acute lymphocytic leukemia and Candida esophagitis. Interestingly, the patient became afebrile following treatment with fluconazole, suggesting a direct association between Candida esophagitis and fever (15). Several different pharmacologic agents have been used to treat Candida esophagitis, including topical medications such as nystatin clotrimazole, miconazole, oral ketoconazole, 5-fluorocytosine, and intravenous amphotericin B. Fluconazole has recently been shown to be less toxic, better tolerated, and more efficacious than topical therapy or ketoconazole in the treatment of Candida esophagitis in AIDS patients (13,16). Our patient tolerated fluconazole well, and resolution of the Candida esophagitis was documented endoscopically. The treatment options for achalasia include medications that relax smooth muscle, pneumatic dilatation, and surgical myotomy. Pharmacologic therapy, usually isosorbide dinitrate or nifedipine, has limited, often transient efficacy and is primarily used in patients with mild disease or who are poor candidates for more aggressive therapies (17). Nifedipine therapy has been shown to produce symptomatic relief in children with achalasia (18). Pneumatic dilatation remains the treatment of choice for achalasia (10,19,20), and myotomy should be reserved for therapeutic failures (10,19). Our patient was initially placed on a combination of nifedipine with antifungal therapy because of his Candida esophagitis. We believe that the initial resolution of his symptoms was due to both the treatment of achalasia and the eradication of Candida. Since both antifungal therapy and nifedipine were started simultaneously, it is impossible to determine which treatment was primarily responsible for the marked improvement of his symptoms. The patient remained asymptomatic during the 12-month follow-up period after pneumatic dilatation. In summary, we present the case of a child with achalasia and Candida esophagitis. Candida esophagitis has not been previously reported as a complication of primary achalasia in the pediatric population. The Candida esophagitis was most likely a consequence of aperistalsis and food stasis, which occurs in achalasia. Esophagoscopy should, therefore, be performed prior to pneumatic dilatation in patients with achalasia to rule out Candida esophagitis. Esophageal dilatation for achalasia with coexisting Candida esophagitis may place the patient at additional risk for complications such as perforation (21) or disseminated candidiasis, and we therefore recommend treatment of Candida esophagitis prior to pneumatic dilatation.FIG. 1.: Nonseptate pseudohyphae (arrow) are seen on the esophageal mucosal surface (top); fragmented pseudohyphae (open arrow) are present with necrotic esophageal epithelium and inflammatory cell infiltrate (bottom). The sample was stained with Gomori's methenamine silver; original magnification ×400.