Tubular duplication of esophagus: rare congenital malformations require individualized and innovative procedures

Rare congenital problems generally have no standardized diagnostic or operative technique. Because of some variation within the spectrum, diagnosis and management has to be individualized and tailored according to the local findings to relieve the symptoms. We report a diagnostic dilemma and surgical management of a child with proximal tubular duplication of esophagus with complete remission of symptoms after surgery. To diagnose rare malformations a high index of suspicion is required when differential diagnoses are being considered

Superior vesical fissure, a curious variant of exstrophy: an experience of three cases in children hospital, Pims

To present experience of superior vesical fissure. Study of management of patients with superior vesical fissure: a variant of bladder exstrophy. The Children's Hospital, PIMS, Islamabad. Patients, cases of superior vesical fissure. Superior vesical fissure is a rare variant of bladder exstrophy. There is a wide spectrum of presentation of these patients. Pubic diastasis if present is minimal. Careful assessment of the extent of the superior vesical fissure is important. Treatment is tailored for the individual patient. Operative management is much easier and satisfying as compared to bladder exstrophy in terms of continence and function

Idiopathic infantile hyperinsulinism: a case report and review of literature

We are reporting our first experience with idiopathic infantile hyperinsulinism which has also been termed nesedioblastosis. There are several causes of hyperinsulinism and hypoglycemia in neonates. It is of prime importance to diagnose hypoglycemia in newborn early to prevent hypoglycemia related brain damage. In contrast to many hyperglycemic hormones, insulin is the only major hypoglycemic hormone. Normally there is a fine control of blood glucose level which if falls below normal levels insulin secretions stops and other hormones like epinephrine, norepinephrine, glucagon etc. are at once poured into the blood to combat hypoglycemia and vice versa. This fine control is lost in idiopathic hyperinsulinemic hypoglycemia of new born in which there is persistently high levels of blood insulin levels despite very low and life threatening levels of blood glucose. There are many theories of this idiopathic increase in insulin levels and so are the various models of treatment. Here we discuss our own experience with such a case

Bleeding Meckel's diverticulum - A known but rare entity: a case report

Meckel's diverticulum is a remnant of embryonic omphalomesenteric duct In 20-30 percent of the cases ectopic tissue is present, which may be either gastric or pancreatic. Mostly it is asymptomatic but may be associated with symptoms of bleeding due to ulcer formation, perforation, intestinal obstruction or discharge from the umbilicus. We are reporting a case of Meckel's diverticulum with bleeding diagnosed on the basis of positive radio-isotope scan

Glant sacrococcygeal teratomas

This is a report of two cases of giant sacrococcygeal teratoma [SCT] with completely different clinical appearance, histological pattern, and hematological findings. In the first case, the malignant nature of the tumor was apparent at birth, whereas the second patient had mature, non-malignant tumor and presented after eight months of birth. In the first case early and complete excision cured him completely of his tumor as was evident in his three years follow up with normal alpha-fetoprotein levels. Whereas in the second patient, the benign and mature nature of the tumor prevented malignant change although patient presented late. In both patients reconstruction of anatomical structures was performed. Early and late postoperative appearance was comparable to normal

Fusiform megal ourethra a case report

We report a case of congenital fusiform megalourethra with anorectal agenesis, rectovesical fistula and absent right kidney. The patient also had uremia. We performed pelvic divided colostomy and vesicostomy. Unfortunately, the patient died on fifth day of life due to septicemia

Neurofibrosarcoma of thigh arising in a case of neurofibromatosis a case report

This is a report of 14 year old boy who had neurofibromatosis Type-I, with plexiform neurofibromas, multiple cafe-au-lait spots, papillomas throughout the body and a large diffuse lesion of left thigh. The latter had completely disabled him. Investigations revealed this lesion to be a case of neurofibrosarcoma, which is a malignant peripheral nerve sheath tumor. Details of management are discussed in this report. A brief review of literature is also presented

Histopathological interpretative difficulties of a soft tissue tumour. a case report

This case is reported to explain the histological difficulties in differentiating soft tissue tumours. A childhood fibrosarcoma is reported which was misdiagnosed even on several reviews of histopathology slides. Though prognosis and treatment of soft tissue tumours are almost the same, there is still a need for proper histologic diagnosis, to place them in an appropriate group

Cleft lip and palate: experience with a common congenital anomaly at PIMS

Cleft lips and palates constitute the most common congenital anomalies seen at the children hospital, Islamabad, with 2052 cases operated on during a period of six years, from 1987-1993. One out of every 3.92 surgical admissions was for this reason; 58% of these were male while 42% were female. Mean age at presentation was approximately 2.6 years. Cleft lip and palate [group 2] was the largest category of the patients [47.3%]. Late presentation was the major cause of morbidity due to malnutrition and permanent voice defects. Our data was also compared with regional studies