Down's syndrome as a factor in the diagnosis, management, and outcome in patients of Morgagni hernia.

PURPOSE: The purpose was to study the impact of Down's syndrome (DS) in the diagnosis, management, and outcomes of patients with Morgagni hernia (MH). METHODS: Twenty-two (22) patients with MH treated at a tertiary center were retrospectively studied for history, findings, associated anomalies, referral diagnoses, hospital admissions, radiological procedures for diagnosis, age at which operated on, operative procedure, complications, and recurrences. Eleven did not have DS (group 1); 11 others had associated DS (group 2). The ages at operation were compared in the 2 groups using the Mann-Whitney test. More than 3 hospital admissions for symptoms and signs relevant to MH before a diagnosis of MH were considered a "delayed diagnosis." RESULTS: Twenty-two patients (7 females, 15 males) aged 3 months to 10 years were seen. They presented with respiratory distress (n = 16), with vomiting (n = 5), with intestinal obstruction (n = 1), by serendipity (n = 2), and with recurrence from another hospital (n = 1). The mean age of group 1 was 14.5 months, and that of group 2 was 29.18 months; the difference was not significant (P = .621). Nine patients of group 2 were "delayed diagnosis" compared with 2 in group 1. Both delays from group 1 had severe associated anomalies. All patients underwent operative correction (17 open and 5 laparoscopic repairs). Two had recurrences, one operated on by the open method by us and another laparoscopically by the Lima technique at another center. Both had DS. Both were reoperated on by the open method. CONCLUSIONS: The diagnosis of MH may be strikingly delayed when associated with DS or other severe congenital anomalies. Morgagni hernia should be strongly considered in patients with DS admitted repeatedly for chest infections. Chest x-rays in 2 planes may avoid misdiagnosis of MH. Both open and laparoscopic methods have proven satisfactory as operative treatment of MH. Recurrences were seen in patients with DS, which may be corrected by laparotomy or laparoscopically. We feel that resecting the sac and approximating the posterior lip of the defect to the anterior abdominal wall, whether in open or laparoscopic methods, may give stronger repairs, which may avoid recurrence.

Operative innovation to the "Nuss" procedure for pectus excavatum: operative and functional effects.

BACKGROUND: The "Nuss" procedure for repair of pectus excavatum is performed with increasing frequency worldwide. We describe a technique of performing relaxing or "kerf" incisions along the cartilaginous ribs at the point of maximal chest wall concavity to facilitate retrosternal dissection and to reduce chest wall tension. The effects on operative parameters and long-term changes in cardiopulmonary function and appearance were evaluated prospectively. METHODS: Thirty patients underwent a Nuss procedure for the period from 2001 to 2004. Fifteen patients underwent a modified Nuss (MN) with sternocostal "relaxing" incisions, and these were compared to 15 patients undergoing a standard Nuss (SN). Data were prospectively gathered and included the number of relaxing incisions, bar number, blood loss, duration of epidural analgesia, number of days to discharge, and preoperative and postoperative cardiopulmonary function measures. RESULTS: The average Haller index of the SN group was 3.9 +/- 0.7 vs MN group index of 4.6 +/- 1.6 (P < .05). The median number of relaxing incisions in the study group was 4.0 +/- 0.7 (range 3 - 8). There were no significant differences between the study groups in any of the operative parameters analyzed, including blood loss, days of epidural requirements or days to reach functional independence. Nor were there differences in the improvement in subjective appearance, complications, pulmonary function, cardiac output at rest, oxygen transport or minute ventilation at maximal exercise. Subjectively, bar positioning and the intraoperative visualization was easier with the relaxing incisions. CONCLUSIONS: The addition of sternocostal relaxing incisions to the standard Nuss procedure appears to facilitate retrosternal dissection and bar placement, but no changes in long-term function or cosmesis were noted. The use of relaxing incisions appears to be safe and may facilitate operative visualization of retrosternal structures.

Congenital para esophageal hernia: a 10 year experience from Saudi Arabia.

OBJECTIVE: Para esophageal hiatal hernia is a rare childhood condition and reported series have had scant number of children which makes diagnosis a challenge. The authors sought to study the presentation and the outcome of treatment of congenital para esophageal hernias (CPEH) over a period of 10 years from a single tertiary care hospital in Saudi Arabia. METHODS: The records of 9 patients presenting between 1997 and 2007, were retrospectively analyzed for demographics, presenting features, referral diagnoses, investigations, management including operative procedures, their outcome and follow-up. RESULTS: Nine patients (3 males and 6 females) aged between 8 days to 34 months were seen. Respiratory distress (n=6), vomiting (n=5) and frequent respiratory tract infections (n=3) were the most common presentations. Cyanosis (n=2), cough and excessive crying were the other important symptoms. The referral diagnoses in these patients included congenital Bochdalek's hernias, lung abscess, bronchogenic cyst, pneumatocoele, bronchiolitis, and pneumonias which reflected a misinterpretation of their clinical findings and chest X-rays. Seven of these patients had other associated congenital anomalies. Three had cardiovascular abnormalities and 2 had lesions of the central nervous system. A pair of siblings had Marfan's syndrome. All the patients had abnormal chest C-rays and an UGS (upper GI series) proved to be diagnostic in 8 patients. The CT scans done in 4 patients corroborated the findings of the UGS. A laparotomy was done on most patients (n=8) which comprised of reduction of the stomach, resection of the hernial sac, tightening of the hiatus and a gastropexy or a gastrostomy. One patient, who underwent thoracotomy died of surgical complications. Two others died of causes unrelated to the surgery. The remaining six operated patients have been followed up for a median of 3.5 years and are doing well. CONCLUSION: CPEH is uncommon in children, presented with respiratory tract symptoms and vomiting, and may be associated with Martan's syndrome. It should be considered in the workup of a child with vomiting or frequent chest infections. Abnormal chest X-rays may indicate the diagnosis and a subsequent UGS, is confirmatory. The present study found the aparotomya good approach for repair of the wide hiatus. A gastropexy and a floppy fundoplication were added to prevent reherniation and post operative reflux though given the small numbers it is not possible to determine the place of either of these procedures. CPEH may be frequently associated with other congenital problems which may impact survival.

Laparoscopic resection of extra-adrenal pheochromocytoma--case report and review of the literature in pediatric patients.

Surgery for pheochromocytoma differs from that of other tumors owing to the potential release of catecholamines, which may lead to severe intraoperative hemodynamic changes. The present standard of care for resection of adrenal pheochromocytoma has become a laparoscopic approach for surgical excision. Extra-adrenal pheochromocytoma is a very rare entity, especially in the pediatric age group; the utility of the laparoscopic approach is not established in this population. We present a case report of a child with hormonally active extra-adrenal pheochromocytoma originating in the organ of Zuckerkandl that was resected laparoscopically. We found the laparoscopic approach gave excellent exposure, allowing for proper identification of the tumor's origin and its relation to surrounding structures; a complete resection with excellent control of the feeding blood vessels was performed. Herein we present the details of this case and a review of the relevant literature. After our initial experience we can recommend laparoscopic exploration for similar cases of suspected extra-adrenal pheochromocytoma as an appropriate tool to identify extension of the disease and estimate resectability. Proper patient preparation and monitoring are critical for success.

Prenatal diagnosis of intrathoracic stomach (gastric herniation).

Intrathoracic stomach is a rare and serious congenital abnormality. The anomaly may be complicated by gastric volvulus and can lead to ischemic gastric infarction in the neonate. If diagnosed antenatally, neonatal management can be planned in advance so as to reduce morbidity. This anomaly must be differentiated from the more common congenital diaphragmatic hernia, as associated pulmonary hypoplasia is common in the latter and rare with gastric herniation. We report an infant born to a mother with Marfan's syndrome with the antenatal diagnosis of intrathoracic stomach. The ultrasound and magnetic resonance imaging features of this congenital abnormality are described. A review of the literature would indicate that this is the first case report of gastric volvulus diagnosed in utero.