Infiltrating, Quasi-Cancerous Rectal Lesions: Unique Manifestation of Visceral Basidiobolus ranarum.

OBJECTIVE: To report the finding of transmural rectal involvement, in four children with lesions which seemed to be neoplastic in appearance and progression. METHODS: The case records of four children presenting with rectal involvement by the fungus Basidiobolus ranarum, were retrospectively analyzed for clinical presentation, hematological and radiological investigations, the procurement of histopathological material for diagnosis and the findings thereof, the treatment of these patients and the follow-up. RESULTS: The four children presented with non-specific symptoms of fever, loss of weight and appetite, constipation and bleeding per rectum. One presented with excoriation and ulceration of the perineum and perianal skin. Examination generally unremarkable, however, showed the rectum extensively involved by a lesion which narrowed the lumen. Hematological investigations showed leukocytosis with eosinophilia and raised levels of acute phase reactants like ESR, platelets, and C-reactive proteins. Ultrasonogram (USG) and CT scan confirmed the lesion to be involving all layers of the rectum and compromising the rectal lumen. The right colon was also involved in one patient. One underwent a colonoscopy and biopsy which proved inconclusive. All four underwent a Tru-cut biopsy which was diagnostic. Histopathologically the disease was based in Basidiobolus ranarum, a fungus which is emerging as a cause of visceral abdominal involvement. CONCLUSIONS: Lesions involving the rectum, and appearing to be neoplastic may be caused by the fungus Basidiobolus ranarum. The symptomatology and presentation may be non-specific. High levels of eosinophils in the blood, a raised ESR, and C-reactive protein may be useful pointers to the diagnosis. USG and CT scans localize the lesions and also provide a guide for biopsies. The characteristic histopathological findings are diagnostic and based on these, treatment with Itroconazole / Voriconazole is beneficial.

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.

Diagnostic dilemmas, course, management and prognosis of traumatic lung cysts in children.

The patients were 8 and 9 years old respectively. Both were passengers in a vehicle and suffered multisystem injuries. Case no.1 suffered a fractured occipital bone, lung contusions and a small pneumothorax in addition to the traumatic lung cysts in the left lung. Case 2 sustained contused and lacerated liver and right lung cysts. CT examination showed cystic areas in the lung which were diagnostic in case 2. In case 1 a traumatic rupture of diaphragm could not be ruled out and the patient underwent an exploratory laparotomy to deal with the same. The lung cysts in both the patients were treated conservatively and both showed resolution in repeat CT scans done at 6 months.

Air filled, inflammatory, lung pseudo-cyst: etio-pathogenesis and management.

Lung cysts in children are usually of congenital origin. Necrotising pneumonias, trauma, cystic fibrosis and kerosene poisoning may be the cause of acquired cysts in the lungs during childhood. We describe a child who presented with pneumothorax and was later discovered to have a cyst in his right lung. The cyst was resected and on histology found to be of inflammatory origin with no epithelial cell lining i.e. a pseudo-cyst. The immuno-histochemical staining of the inflammatory cells from the pseudo-cyst wall showed them to be predominantly histiocytes i.e:, CD68 antigen positive. The cells showed negative staining for S-100 (marker for Langerhans cells), cytokeratins (epithelial markers) and smooth muscle actins excluding the possibility of histiocytosisX, epithelial and congenital cysts respectively. This case illustrates the occurrence of acquired pulmonary pseudocysts unrelated to known etiologic agents which should be considered in the work-up for pneumothoraces.

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.

Lung cysts following pulmonary artery operations: diagnostic and therapeutic implications.

We describe the case of a child suffering from congenital cyanotic heart disease - double outlet right ventricle (DORV) with transposition of great vessels (TOGV). She underwent a left Blalock-Tausig (BT) shunt at one month of age followed by a Glen procedure with left pulmonary artery augmentation at six months. Following the second procedure she developed extensive cyst formation in the upper lobe of the left lung and pneumothorax. She was managed by intercostal drainage of the pneumothorax. The cysts were observed and on a CT scan X-rays taken at one month and six months no cysts were seen. This case illustrates the occurrence of pneumatoceles after pulmonary artery manipulation, their proclivity for causing pneumothoraces and involution on follow-up. Cysts noted in such a setting should be monitored carefully and followed up to resolution.

Congenital pouch colon syndrome in a Saudi Arabian neonate.

Congenital pouch colon syndrome is partial or complete replacement of the colon by a pouch-like structure and anorectal malformation. Commonly reported from northern India, we believe this is the first report of congenital pouch colon syndrome in a Saudi Arabian neonate. He was referred with an imperforate anus and diagnosed as having a high anorectal malformation. The patient underwent a sigmoid colostomy. Continued abdominal distension after the colostomy prompted sonography and computed tomography, which showed an air- and fluid-containing cystic structure in the abdomen. Exploration revealed a thick-walled cystic structure in continuity with the distal colostomy and with a blood supply mainly from the superior mesenteric artery. The ureters were dilated and tortuous and the pouch opened into the posterior vesical wall. The pouch was resected and an end colostomy fashioned. The etiopathogenesis classification and management of the congenital pouch colon syndrome are discussed.