An unusual cervical swelling in a child.

Congenital thymic cysts are rare benign lesions, frequently seen in the neck and mediastinum, but because of their infrequent occurrence and similarity to other more common cystic swellings, their preoperative diagnosis is not considered. Congenital thymic cysts although rare should be considered in the differential diagnosis of cervical and mediastinal cystic swellings in children. This is a case report of a multilocular cervical thymic cyst in a child.

A case of Turcot's Syndrome in a child with malignant transformation.

Turcot's syndrome or the glioma polyposis syndrome is a rare variant of the polyposis syndrome and it is characterized by colonic polyposis and central nervous system neoplasm typically a glioblastoma or a medulloblastoma. We present a case of Turcot's syndrome in a child with malignant transformation.

Congenital Morgagni's hernia in infants and children.

Morgagni's hernia is an uncommon type of diaphragmatic hernia in the pediatric age group. Out of 52 children with different types of congenital diaphragmatic hernia that we have treated, 5 (9.6%) had Morgagni's hernia. There were 2 infants and 3 children including one with Down's syndrome. All suffered from repeated attacks of chest infection, and only after a chest X-ray was the diagnosis of Morgagni's hernia suspected. In 2 cases this appeared as an opacity in the anterior mediastinum adjacent to the pericardium; diagnosis was confirmed by barium enema in one and a CT-scan in the other. The remaining 3 cases showed anterior herniation of bowel loops on chest X-ray which was bilateral in one. This bilaterality was confirmed pre-operatively by CT scan. Associated anomalies were present in all cases, including 2 with malrotation. All patients were treated surgically via a transabdominal approach. Our study shows a relative high frequency of Morgagni's hernia in our patients and, although late-presenting Morgagni hernias are relatively benign, it can cause significant morbidity. This calls for early diagnosis and early referral for surgery. Chest X-ray is to be strongly advocated in children with repeated attacks of chest infection.

Intrauterine torsion of a wandering spleen presenting as an abdominal cystic swelling.

Wandering spleen is a rare clinical condition that presents commonly with splenic infarction secondary to torsion. Intrauterine torsion of a wandering spleen, however, is extremely rare. An unusual case of intrauterine torsion of a wandering spleen presenting as an abdominal mass is reported.

Congenital spigelian hernia and cryptorchidism: cause or coincidence?

Congenital spigelian hernia (SH) is very rare in the pediatric age group. This is a report of two cases of SH in 1-week and 3-month-old male infants. A review of the literature revealed only 35 cases of SH in children younger than 17 years of age, bringing the total including our 2 cases to 37. There were 25 males and 12 females, a ratio of 2.1:1. Their ages ranged from 6 days to 17 years (mean 4.52 years). The hernia was situated on the right side in 13, the left side in 19, and was bilateral in 4. In one case the side of the hernia was not mentioned. In 29 cases the hernia was spontaneous while in 5 it was caused by trauma. In 3 children the hernia developed postoperatively, in 2 following repair of a congenital diaphragmatic hernia and in 1 following excision of a mediastinal neuroblastoma. Two children presented with a strangulated SH. Eleven of the 35 previously reported children had associated conditions; in 5 there was an ipsilateral undescended testis (UDT). Our two infants with SH also had an ipsilateral UDT. The significance of this association is discussed.

Intrathoracic gastric volvulus in infancy.

Intrathoracic gastric volvulus is a very rare surgical emergency. Early diagnosis and treatment are of great importance to prevent gastric gangrene and perforation or gastric obstruction and dilation, which may lead to cardiorespiratory arrest. We report two infants who presented with intrathoracic gastric volvulus. This was associated with recurrent diaphragmatic hernia in one and congenital paraoesophageal hernia in the other. Aspects of diagnosis and treatment are also discussed.

Congenital paraesophageal hernia in infancy and childhood.

OBJECTIVES: Congenital paraesophageal hernia is a rare condition in the pediatric age group. The symptomatology of these patients is usually non-specific in the form of repeated attacks of chest infection and/or recurrent attacks of vomiting but can be associated with serious complications such as intrathoracic gastric volvulus. METHODS: Between 1989 and 1997, 6 children with paraesophageal hernia were treated at our hospital. RESULTS: Six children (4 males and 2 females) were treated for congenital paraesophageal hernia. Their age at presentation ranged from 2 days to 21/2 years (mean 1.3 years). Two presented with recurrent chest infection, while 3 others had recurrent attacks of vomiting with fullness and pain in the epigastrium in one of them. One of our patients presented acutely immediately after birth with respiratory distress while another was found to have intrathoracic gastric volvulus. Chest x-ray was suggestive of paraesophageal hernia in all of them but the diagnosis was confirmed by Barium swallow and meal. Intraoperatively there was a hernial sac in all of them. The surgical treatment consisted of excision of the hernial sac after reducing the stomach and tightening of the crura of the esophageal hiatus. Nissen's fundoplication was added in 3 patients, but in one of them this was dismantled because of tight repair. Anterior and fundal gastropexy was added in one patient, while 2 had tightening of the crura only. CONCLUSION: Congenital paraesophageal hernia, although rare in the pediatric age group, can present acutely with respiratory distress or intrathoracic gastric volvulus. Physicians caring for these patients should be aware of such a presentation and complication and paraesophageal hernia should be included in the differential diagnosis of children with repeated attacks of chest infection and/or vomiting. The rarity of this condition in children makes it difficult to evaluate the true necessity of adding an antireflux procedure in these patients. We feel some form of gastropexy may be a more appropriate procedure to be added to the repair.

An unusual anterior mediastinal mass in a child with B-thalassemia major.

This case report describes the delayed appearance of Morgagni's hernia in a 5 year old child with B-Thalassemia major to present as an anterior mediastinal mass. The progressive enlargement of the liver resulted in herniation of the left lobe of the liver through the already congenitally present Morgagni's hernia leading to its enlargement. The report also emphasizes the fact that a previously normal chest x-ray should not preclude the diagnosis of Morgagni's hernia. Morgagni's hernia should also be included in the differential diagnosis of anterior mediastinal masses in children.

Pneumoperitoneum and free meconium without gastrointestinal perforation in a neonate.

A newborn was referred to our hospital because of poor feeding and abdominal distension and was found to have pneumoperitoneum on abdominal x-ray. At operation there was free intraperitoneal air with no free fluid in the peritoneal cavity. In addition there was free air and meconium retroperitoneally on the left side but there was no evidence of gastrointestinal perforation. This case is rare and unique in that no demonstrable cause for the free air and free meconium could be demonstrated.

Pyloric atresia associated with duodenal and jejunal atresia and duplication.

A case of congenital pyloric atresia associated with duodenal atresia, jejunal atresia, and a duplication is described. A review of the literature revealed nine previously reported cases of congenital pyloric atresia and multiple intestinal atresias, but this is the first report of such an association and a duplication.

Splenic complications of the sickling syndromes and the role of splenectomy.

PURPOSE: To analyze the authors' experience with splenectomy for sickling disorders and evaluate the indications, complications, and outcome. PATIENTS AND METHODS: Over a period of 10 years (1987-1997), 113 patients with sickling disorders (100 with sickle cell disease and 13 with sickle-beta-thalassemia) had splenectomy at the authors' hospital as part of their management. The indications for splenectomy were hypersplenism (26 patients), major splenic sequestration crisis (MSSC) (23 patients), minor recurrent splenic sequestration crisis (MRSSC) (50 patients), splenic abscess (12 patients), and massive splenic infarction (2 patients). RESULTS: Splenectomy in patients with sickle cell disease (SCD) and sickle-beta-thalassemia (S-beta-Thal) was beneficial in reducing their transfusion requirements and its attendant risks, eliminating the discomfort from mechanical pressure of the enlarged spleen, and avoiding the risks of acute splenic sequestration crisis. It also was curative for patients with splenic abscess and massive splenic infarction. Twenty-four patients with SCD (24%) had splenectomy and cholecystectomy caused by concomitant gallstones. There was no mortality, and the postoperative morbidity was 7%. CONCLUSIONS: With careful perioperative management, splenectomy is both safe and beneficial in a select group of patients with SCD and S-beta-Thal.

Is splenectomy for massive splenomegaly safe in children?

PURPOSE: To study and analyze the causes, etiology, morbidity, mortality and therapeutic value of splenectomy performed for massive splenomegaly in children. METHODS: The medical records of 115 children less than 18 years old who had splenectomy for various hematological disorders were reviewed. Twenty of them had splenectomy for massive splenomegaly (spleen weight > or =1,000 g). The records of these were reviewed for age at operation, gender, hematological diagnosis, indication for splenectomy, operative procedures, postoperative complications, and outcome. RESULTS: Twenty children had splenectomy for massive splenomegaly. There were 16 males and 4 females. Their ages ranged from 4 to 15 years (mean 11.2). Twelve had sickle cell disease, 5 had sickle-beta-thalassemia, 1 had beta-thalassemia major, 1 had thalassemia intermediate, and 1 had chronic myeloid leukemia. The indications for splenectomy were hypersplenism in 11, recurrent splenic sequestration crisis in 8, and splenic abscess in 1. The transfusion requirements in the patient with beta-thalassemia major decreased markedly postoperatively from 18 transfusions/year to only 4 transfusions/year; and for those with hypersplenism, there was a marked improvement in their blood parameters following splenectomy. The patient with thalassemia intermediate required no more blood transfusions. There was no mortality. The immediate postoperative morbidity was 10% for those with massive splenomegaly compared with 6.3% for those with splenomegaly <1,000 g. CONCLUSIONS: With good perioperative management, splenectomy in children with massive splenomegaly is both safe and effective.

Intra-uterine testicular torsion: early diagnosis and treatment.

OBJECTIVE: To review the incidence and treatment of intra-uterine torsion of the testis which although rare is being recognized with increasing frequency. PATIENTS AND METHODS: From 1988 to 1997, five newborns (mean birth weight 3.62 kg, range 3.15-4.12) with unilateral torsion of the testis were treated; all underwent emergency exploration. The right testis was affected in three and the left in two boys. RESULTS: In all except one child, the affected testis was enlarged, firm to hard, tender, the overlying skin dark red and the affected testis higher than the contralateral testis. In one child the right testis was enlarged and higher, but soft to firm, and the overlying skin was oedematous and red. The exploration revealed extravaginal torsion of the testis which was gangrenous in four; in one after detorsion there was haemorrhage and haematoma of the cord and the tunica, and the testis was slightly congested but not gangrenous. This testis was preserved and bilateral orchidopexies performed; at 18 months both testes are palpable and of normal size. In the remaining four children the testes were frankly necrotic; they underwent orchidectomy and contralateral orchidopexy. Histology in all four revealed a totally infarcted testis with extensive haemorrhage and vascular congestion. CONCLUSION: The early diagnosis and treatment of intra-uterine torsion of the testis is essential.