Salam Yazbeck

Childhood primary pulmonary neoplasms

Nine children (6 boys, 3 girls) were diagnosed with a primary endobronchial or pulmonary parenchymal neoplasm. The average age at diagnosis was 9 years. Presenting complaints included cough (7), fever (5), pulmonary infection (3), respiratory distress (3), weight loss (2), pain (2), and hemoptysis (1). Pulmonary x-rays showed persistent atelectasis, pneumonic infiltrates or mass lesions. A computed tomography scan was performed in 8. Five of six endobronchial tumors were diagnosed with bronchoscopy and biopsy. Treatment consisted of thoracotomy and pulmonary resection in 7 cases and laser resection in 2. The pathologic diagnoses were bronchial carcinoid (3), bronchial mucoepidermoid carcinoma (1), inflammatory pseudotumor (plasma cell granuloma) of the bronchus (2) and of the lung parenchyma (1), fibrosarcoma (1), and rhabdomyosarcoma (1). Postoperative chemotherapy was given only to the patient with pulmonary rhabdomyosarcoma; this child died. One child has developed a local recurrence while 7 children are alive and free of disease at an average of 2.4 years postresection. Pulmonary neoplasms are unusual in the pediatric age group and represent a wide spectrum of pathology. Including the present series, 383 tumors have been described. Seventy-six percent were malignant. Early investigation and surgical intervention are essential in children with persistent pulmonary symptoms or x-ray abnormalities. In most cases, the prognosis is excellent with complete surgical resection; however, malignancies other than bronchial adenoma are associated with significantly mortality.

Malrotation Presenting Beyond the Neonatal Period

The diagnosis of malrotation is easily made in the neonatal period, but is often delayed in older patients. Among 82 patients treated for malrotation in this institution, 45 patients presented with symptoms related to their malrotation, seven were diagnosed at exploration for concomitant intrinsic duodenal obstruction, and 30 patients had malrotations discovered as incidental findings at laparotomy or autopsy. Among the 45 symptomatic patients, 25 (56%) underwent surgery in the first month of life, whereas 20 patients (44%) underwent surgery at an older age. In this last group, the mean age at surgery was 51.5 months (range, 2 months to 16 years), the mean age of onset of symptoms was 2 years (range, 0 to 15 years) and the mean delay in diagnosis was 1.7 years. Although bilious vomiting was the presenting symptom among all patients undergoing surgery in the neonatal period, clinical features of older patients included intestinal obstruction (7), chronic abdominal pain (4), malabsorption/diarrhea (3), peritonitis/septic shock (2), solid food intolerance (1), common bile duct obstruction (1), abdominal distention (1), and delayed transit postappendectomy (1). The frequency of midgut volvulus was equal among both groups. Unusual forms of malrotation were more frequent in patients undergoing surgery beyond the neonatal period. In this group there was evidence of chronic venous and lymphatic obstruction with one case of superior mesenteric vein thrombosis and two cases of intestinal gangrene. A Ladds procedure was performed in all cases and the most frequent postoperative complication was adhesive intestinal obstruction. There were no deaths. Awareness of the unusual presentation in patients who present beyond the neonatal period may help reduce delays in diagnosis and surgical treatment. We believe that laparotomy is indicated in all patients with malrotation, even if they are asymptomatic.

Accuracy of doppler sonography in the evaluation of acute conditions of the scrotum in children

During a 12-month period, 65 boys (newborn to 18 years of age) with acute scrotal pain or swelling underwent Doppler sonography of both testes, followed by scintigraphy (15) and/or surgery (34) and close clinical follow-up for at least 4 months (23). Pulsed Doppler was performed with an ATL UM8 or Quantum II apparatus, using 7.5- or 10-MHz transducers. In 25 patients, color Doppler was also used. The testicular artery was deemed patent if Doppler shifts from branches within the parenchyma could be found. (Doppler signals from scrotal or marginal arteries were considered nondiagnostic). Through surgery, 19 testicular torsions were noted. Seventeen were diagnosed as such with Doppler. Two boys with torsion were deemed normal at the time of both Doppler and scintigraphy evaluation. One boy had a second Doppler examination 13 hours later, which showed no flow. The testicle was necrotic at the time of surgery. The second boy had had 8 hours of symptoms and had a viable testis at the time of surgery. This probably represented intermittent torsion. In six of the 65 cases, no signals could be found on either side, and they were deemed technical failures of the test. Doppler sonography was technically successful in 59 of the 65 patients (91%) and yielded a sensitivity of 89% and specificity of 100%. Comparing the normal and painful side helped to define technical failures. Pulsed Doppler with mechanical sector scanners was more sensitive than color Doppler. Intermittent torsion was missed with both Doppler sonography and scintigraphy. Although ultrasonography cannot replace clinical judgement, it may be very helpful in unclear cases.

Isolated congenital esophageal stenosis

The incidence of congenital esophageal stenosis (CES) is approximately 1 in 25,000 to 50,000 live births. There is associated esophageal atresia in one third of cases; the remainder are classified as isolated CES. Histologically, the anomaly may include tracheobronchial remnants, a membranous diaphragm, or diffuse fibrosis of the muscularis and submucosa. The authors report their experience with three patients who had isolated CES. The patients were free of symptoms for the first 6 months of life. All had difficulty with feeding from 6 to 12 months of age, which corresponded with the introduction of solids. Most solid feedings were regurgitated. Evaluation consisted of cine-esophagogram, pH monitoring, manometry, and endoscopy, with biopsies to exclude the diagnosis of gastroesophageal reflux. All patients underwent hydrostatic dilatation, but the benefit was only transient. The patients were referred for surgical correction when symptoms recurred. Limited resection of the esophageal stenosis with primary anastomosis was performed on all three patients via a left thoracotomy. The stenoses were located in the distal third of esophagus, near the junction with the middle third. Pathological examination showed tracheobronchial remnants in one patient and fibrotic muscle in the other two. The average age at the time of surgery was 19 months. The hospital stay averaged 8 days. The contrast study 1 week postthoracotomy showed esophageal patency in all patients, with no leakage. All were discharged from the hospital, tolerating solid food. CES should be sought for in patients who present with dysphagia to solid food that begins after the first 6 months of life.(ABSTRACT TRUNCATED AT 250 WORDS)

Primary spontaneous pneumothorax in children.

In the absence of pediatric data, spontaneous pneumothorax is managed according to adult guidelines. Fifty-eight patients with primary spontaneous pneumothorax (PSP) were treated in our center over the last 20 years. The median age was 16.7 years, and the male:female ratio was 1.9:1. A total of 102 PSP were treated; 63% were left-sided. The risk of recurrence was 51% after one PSP and 56% after two. There were four metachronous bilateral PSP. Nonoperative management included tube drainage in 57% of the cases (mean extent of PSP, 53%). Forty percent of patients were treated by supplemental oxygen and observation, without drainage (mean extent of PSP, 23%). Eleven patients were treated as outpatients, with Heimlich valves (mean extent of PSP, 64%). Fourteen patients (28%) underwent bullectomy, with or without pleurodesis. Thirteen of the surgically treated patient had experienced at least two episodes of PSP. Primary spontaneous pneumothorax in children has male predominance. The risk of recurrence after one episode is greater than that for adults. Operative management by bullectomy, with or without pleurodesis, carries little morbidity, has a high success rate, and is recommended after the first recurrence. It is safe to manage younger children conservatively because the chance of recurrence is lower; thoracotomy was not necessary in children under 9 years of age.

Benign liver tumors in children: A 25-year experience

The most common benign liver tumors are hemangiomas and hamartomas, both of mesenchymal origin. Mortality for patients with these tumors has traditionally been substantial despite benign histology. Between 1965 and 1989, 22 patients were treated for a benign liver tumor. This represents 42% of all primary neoplasms of the liver observed during this period. Incidental findings of liver tumors at autopsy were excluded from this series. There were 9 boys and 13 girls with a mean age at presentation of 2.3 years (range, birth to 14 years). Sixteen had hemangiomas and presented earlier in life (mean age, 4.4 months). In this subgroup, high-output cardiac failure was present in 58% of the newborns. Seven hemangiomas were resected, four were observed, three were treated with digitalis, diuretics, and steroids, and one received epsilon-aminocaproic acid. Nonhemangiomatous tumors included four hamartomas, one focal nodular hyperplasia, and one nodular transformation. All six were resected. There was one death early in the series. At a mean follow-up of 38 months, 21 of the 22 patients are cured or asymptomatic. In the past, mortality rates of close to 90% have prompted many investigators to advocate resection of every symptomatic hemangioma. With the availability of more sophisticated imaging techniques and refinements in the treatment of cardiac failure, surgery can be used more selectively. Hepatic resections, once considered heroic, can now be performed with minimal morbidity and virtually no mortality. The 96% survival in this series of benign liver tumors contrasts with high mortality rates reported in the literature and illustrates the spectacular improvements that have been made in the diagnosis and management of these once ill-reputed tumors.

Mesenteric cysts in children

Mesenteric cysts are rare intraabdominal lesions of childhood that may vary in presentation from an asymptomatic mass to an acute abdomen. From 1970 to 1990, 15 children were diagnosed and treated for mesenteric cysts at Ste Justine Hospital in Montreal. The ages ranged from birth to 18 years (average age, 6 years). There were 9 boys and 6 girls. Ten patients required emergency surgery and five underwent elective surgery. The main presenting symptom was abdominal pain. Ten patients had preoperative ultrasounds that were diagnostic for a cystic mass in all patients. The second most frequent preoperative diagnosis was appendicitis. The cysts were located in the small bowel mesentery in 5 cases, the base of the mesentery with retroperitoneal extension in 4 cases, the transverse mesocolon in 4 cases, and the gastrocolic ligament in 2 cases. Operative procedures performed included complete cyst excision (9 patients), complete excision with intestinal resection (5 patients), and drainage of the cyst (1 patient). The only recurrence in this series occurred after drainage. One other patient had recurrence of a mesenteric cyst following resection performed elsewhere. Mesenteric cysts are rare in children, are usually symptomatic, and are most commonly misdiagnosed as acute appendicitis. Accurate preoperative diagnosis is possible with current ultrasonographic imaging techniques. Complete cyst resection is the procedure of choice and results in an excellent outcome.

Adnexal torsion in children

Adnexal torsion is rare in children and is usually reported as small series or case reports. We reviewed a series of 19 consecutive cases of children aged 3 to 19 years (mean, 9.6 years) who were treated in our institution between 1977 and 1988. Thirteen patients presented with torsion of a previously normal adnexa, while six presented with torsion of a diseased adnexa. The right adnexa was involved in 84% of cases. Detorsion with recovery of vascularization of the adnexa was possible in only four cases. All patients presented with lower abdominal pain, and onset was sudden in 78% of cases with an average of 5.2 days between the first symptom and hospital admission and a mean delay of 30.2 hours between consultation and surgical intervention. A previous history of abdominal pain was present in nine cases. Nausea or vomiting were present in 84% of cases. An abdominal mass was palpable in 42% of the patients and was associated with a delay in surgical intervention. Ultrasound confirmed the presence of a mass in 94% of cases. The preoperative diagnosis was accurate in 37% of cases, and the most common inaccurate diagnosis was appendicitis or appendiceal abcess. Our series confirms the predominance of right-sided lesions as reported in the literature. It is not clear whether this is an anatomic phenomenon or whether the suspicion of appendicitis leads to the more frequent diagnosis of right-sided lesions, whereas many left-sided adnexal torsions are being missed. We therefore advocate pelvic ultrasound in female patients who present with left lower quadrant pain.(ABSTRACT TRUNCATED AT 250 WORDS)

THE OTOLARYNGOLOGIC MANIFESTATIONS OF GASTROESOPHAGEAL REFLUX: WHEN IS A PH STUDY INDICATED?

PURPOSE The aim of this study was to evaluate the pertinence of pH studies for persistent ear, nose, and throat (ENT) symptoms and their eventual relationship to gastroesophageal reflux (GER). METHODS Retrospective analysis was performed of age, reason for referral, pH study, treatment, and follow-up of patients with ENT symptoms suspected to have GER. RESULTS Of 3,000 esophageal pH studies performed over 16 years, 105 children were referred for ENT symptoms by an otorhinolaryngologist to rule out GER. Mean age was 33 months; 65% were boys. Reasons for referral included (number and mean age): stridor (n = 31, 8 months), laryngomalacia (n = 18, 13 months), recurrent otitis (n = 12, 42 months), laryngitis (n = 16, 50 months), dysphonia (n = 14, 59 months), laryngeal papillomatosis (n = 8, 62 months), sinusitis (n = 5, 56 months), and dysphagia (n = 1). Overall, 41% of study results were positive: stridor (58%), laryngomalacia (61%), laryngitis (56%) and sinusitis (40%). Patients with otitis, dysphonia and laryngeal papillomatosis had GER in 1%, 14%, and 25%, respectively. Follow-up in the three larger groups of patients showed resolution of the ENT symptoms after medical treatment of the reflux in 83% of patients with stridor and reflux, 86% with laryngitis and reflux, and 80% with laryngomalacia and reflux. Four fundoplications were performed: one neurologically impaired patient, and four nonresponders. CONCLUSIONS The authors recommend that a pH study be performed in children with stridor, laryngomalacia, laryngitis, and sinusitis when faced with failure of the usual treatment. However, a pH study does not seem as beneficial for recurrent otitis, dysphonia, or laryngeal papillomatosis.

Cholelithiasis in newborns and infants

Cholelithiasis in infants is rare, and has usually been associated with hemolysis, ileal disease, congenital anomalies of the biliary tree, hyperalimentation, and prolonged fasting. With the increased use of abdominal ultrasonography (US), more cases of cholelithiasis are being discovered. We report our experience with 13 infants diagnosed on abdominal US to have gallstones. There were 9 boys and 4 girls with an average age at diagnosis of 2.6 months (range, 0 to 9 months). Predisposing factors could be identified in only 6 of the 13 patients. Two patients with obstructive jaundice underwent cholecystectomy and common bile duct exploration. One patient with choledocolithiasis and common bile duct dilatation was observed. His stone passed spontaneously, with resolution of symptoms. Ten patients without cholestasis remained asymptomatic, with disappearance of lithiasis in five of them. Neonatal cholelithiasis is more common than previously suspected; it seems to affect males more often than females and is usually not associated with known predisposing factors. It appears to be a temporary, self-limiting phenomenon, and an aggressive approach is not warranted in the asymptomatic infant. Surgical or radiological intervention should be reserved for the symptomatic patients or those with underlying lithogenic disorders.