Vol. 43, No 2, travanj - lipanj 1999
Vascular rings and slings: A 20 - year experienceVascular rings and slings are rare congenital anomalies that often result in obstructive respiratory or esophageal symptoms during early childhood. The authors report on 23 patients with vascular rings or slings that were surgically repaired during between 1978 and 1998. All surgical procedures were performed by a single pediatric surgeon (RA) and all patients were managed on the pediatric surgical service. Sixty-seven percent of the children in this series had a double aortic arch. The clinical features of these children are presented and compared to those of the largest reported series in the literature. The embryologic basis, pathologic findings, and a strategy for surgical management of the most common vascular rings and slings are reviewed.
Vascular rings and slings are a reasonably rare group of developmental anomalies that were described by D r s. R o b e r t G r o s s (1) and W i l l i s P o t t s (2) over a ten year period at midcentury. Many variations on the original descriptions have been reported over the last fifty years; but as more information has become available, it has been possible to separate them into two large groups (rings or slings) each having two major subdivisions. The purpose of this report is to describe a personal experience with these lesions over a 20-year period at 3 institutions and to review the relevant clinical and pathological findings associated with vascular rings and slings.
MATERIALS AND METHODS
The medical records of all patients with vascular rings or slings managed and treated by a single pediatric surgeon (RA) at three institutions (Oschner Clinic, Wyler Children<@146>s Hospital, Children<@146>s Memorial Hospital) between 1978. and 1998. were reviewed. All patients were managed on the pediatric surgical service. The pertinent clinical, radiographic and pathological findings associated with each case were reviewed. This limited series is compared to all children undergoing surgical repair of vascular rings or slings at the Children<@146>s Memorial Hospital, which holds the largest reported single-institution experience. A simple classification system and the embryological basis for these anomalies are presented. The authors<@146> strategy for surgical management of these patients is also described.
Significance and usefulness of platelets indexesThe importance of platelet count in blood is in their vital role in the hemostase process. Routine analysis of blood today includes, as well as the number of platelets, platelets indexes. This is made possible by automatization of the haematology analaysis system. The possibility of a variety of approaches to the mathematical calculation of platelets indexes, results in many variations on different types of analysers. This variation is one of the reasons why clinicians almost exclude these indexes from use. Another reason is the undefined significance of and insufficient research into this in different diseases. We describe here the most frequent platelet indexes: platelets number (Plt), mean platelets volume (MPV), plateletcrit (Pct) and platelets distribution width (PDW). As well as the definition and mode of calculation on different types of analysers, we describe and examine conditions in which platelets indexes significantly change. Recent studies demonstrate an attempt to decrease to variation by the introduction of new platelets indexes. They are present the difference between the expected and measured values. The name of these indexes, along with the forma name, have a subname: "residual", for example first of PDW. The particular significance of platelets indexes is that it affers the possibility of examining the differencial diagnosis of thrombocytoses and thrombocytopenies. We also describes of platelet distribution curves. Their significance and situations where this curve significantly differs from normal distribution. The appearance of the curve can be a reference for presence of a possible technical mistake or interference. If is important for analysts to detect it and to give a certain and correct interpretation.All this effort is aimed at a: clear definition of the usefulness of platelets indexes. As blood parameters which can be obtained in routine blood analyses, they can give simple and rapid directions for analysts and clinicians
Parental smoking and its effects on children1996, as part of the epidemiological research, the Multi-indicator survey in Croatia - the research into parental knowledge and behavior concerning childrens health and nutrition, a survey was also carried out about parental and maternal smoking habits. The method used in the survey was the cluster metod and it included 253 mothers and 245 fathers, and the total of children up to 5 years of age in these households was 1937. The survey points out the health hazards of passive smoking before conception and all through adolsecence and shows the factors that relate to parental smoking habits. The survey showed that, in households with children up to 5 years of age, 35.2% mothers smoke and 50,2% fathers smoke. Maternal smoking habits depend on the degree of their education, whereas, this correlation cannot be found in the case of fathers.
False negative antigliadin and antiendomysial antibodies in selective serum deficiencyIn this study we report two cases of gluten enteropathy diagnosed by clinical hystory, small bowel biopsy and clinical improvement on a gluten-free diet. Both had elevated serum IgG-antigliadin antibodies and false negative serum IgA-antigliadin and antiendomysial antibodies. Determination of serum IgA facilitates the interpretation of unexpected negative antigliadin class IgA and antiendomysial antibodies.
Experience of splenic mass saving procedures in childrenSplenic Mass Saving Procedures (SMSP) are used to prevent long-term risks of splenectomy. In order to assess the current state of SMSP in the surgery of the spleen, their efficacy and long term results, we reviewed the records of 289 children operated for the splenic lesion over a 15-year period. Splenectomy was performed in all cases of hematological disorders, with hemolytic disorders making up to 85% of the material. SMSP were effectively used in patients with epidermoid cysts (n=13), pseudocysts (n=2), parasitic cysts (n=2), hemangioma (n=1), and splenic injuries (n=14). The following SMSP were performed: partial splenectomy or cystectomy of epidermoid cysts; aspiration of parasitic cysts; unroofing and washing out the cavity with 20% saline; outer wall decapsulation with oversewing of the splenic wall of post-traumatic pseudocysts; suturing combined with ligation of the splenic artery in injuries involving major hilar vessels; suturing combined with excision of the emaciated lower pole. The postoperative course was free of complications in this series. Radioscintigraphic imaging and platelet counts showed preservation in splenic function in the follow-up period. It is concluded that: 1) SMSP represent 11% of splenic surgery in this series, 2) Non-splenectomy operations can be safely and effectively performed in order to avoid long risks of splenectomy, and 3) There is a variety of non-splenectomy operations that can be done in relation with the underlying pathology of the spleen.
Material and Methods Two hundred eighty nine children were operated for the splenic lesions during the between 1984 and 1998. Hemolytic disorders comprise 85% of this material, followed in order of frequency by splenic injuries, congenital cysts, thrombocytopenic purpura, myeloblastic syndromes, hydatid cysts, post-traumatic pseudocysts and Casabach-Merritt syndrome. Splenectomy was performed in all cases of hematological disorders. SMSP were performed on 32 patients (11%) with congenital cysts (13 cases), parasitic cysts (2 cases), pseudocysts (2 cases), hemangioma (1 case), hilar injuries (11 cases), and emaciation of the lower pole (3 cases) (Table 1.).
The age of patients submitted to SMSP ranged from 2 to 14 years. The male/female ratio was 1,2:1. There was a predominance in number of females over males in the benign <@147>tumors<@148> group and of males over females in the trauma group.
Seventeen patients were admitted with cysts, as follows: 13 epidermoid, 2 parasitic and 2 post-traumatic. One patient was admitted with haemangioma. The size of the cyst ranged from 4 cm to 15 cm. Left upper quadrant abdominal discomfort was the main symptom in patients with large cysts. The enlarged spleen was palpable in 14/17 patients. Imaging techniques were employed for diagnosis. These included ultrasonography and CT scan. Ultrasonography accurately provided the diagnosis of splenic cysts as confirmed by operative findings. Hemisplenectomy was performed in 10 children with epidermoid cysts and in one child with hemangioma. Three children with epidermoid cysts underwent cystectomy. In two children solitary hydatid cysts were emptied of their contents by aspiration, and the resulting cavity was washed with 20% saline solution to kill the contained scolices. Two children with pseudocysts underwent partial decapsulation and oversewing of the outer wall. All epidermoid cysts had trabeculated internal surface and multiple septa. The cyst fluid was light yellow. Microscopic studies demonstrated epithelial lining. Hydatid cysts had epithelial lining without trabeculation. They contained crystal clear fluid as well as mother and daughter cysts. Pseudocysts had no epithelial lining. They contained fluid consistent with hematoma. Fourteen patients were admitted following blunt abdominal trauma. The diagnosis of splenic injury was established by ultrasonography and computed tomography. Non-operative treatment initially followed the diagnosis, but had to be discontinued due to the deterioration of vital signs and laparotomy was undertaken. Eleven patients had hilar injuries involving major segmental vessels. They were treated by suturing, combined with ligation of the splenic artery. Three patients with emaciation of the lower pole underwent hemisplenectomy. Postoperative recovery was free of complications in all the patients submitted to SMSP. Splenic function was routinely screened at 3-6 months after the operation. Platelet counts were normal. Radionuclide scans demonstrated normal uptake in this series, including the patients with ligated splenic artery.
SMSP are enabled by the arterial network of the spleen and its anatomical arrangement. As it has been described by many authors, the hilar branches of the splenic artery are further divided into 5 or more branches, supplying the splenic parenchyma in a segmental fashion. The segmental vascularization of the spleen allows splenorrhaphy of transverse tears on the one hand, and partial splenectomy on the other hand (1, 7, 8, 10).
Partial splenectomy was performed on patients with epidermoid cysts, solitary hemangioma and injuries with emaciation of the lower pole. The main steps of the operation were: ligation of the segmental artery, mobilization of the spleen, sharp incision of the capsle, transsection on the demarcation line using the finger, hemostasis of the intraparenchymal vessels and suturing of the raw surface by either mattress or continuing sutures. No stapler was used in our patients (10). Not all epidermoid were dealt with by partial splenectomy. Instead, cystectomy was performed in 3 cases, by resection of the major portion of the cyst leaving behind a small part of the cyst wall (8). The collateral circulation of the spleen is derived mainly from the superior polar artery (which occurs within less than 2 cm before the bifurcation of the splenic artery in children), the short gastric arteries, and the left gastroepiploic artery whose branches traverse the gastroepiploic ligament. Additionally, minor vessels traverse the ligaments and peritoneal attachments of the spleen. If the spleen has not been mobilized and the splenic ligaments are intact, ligation of the splenic artery is permissible. In children, it was found that the spleen is visualized in radioscintigraphies after the ligation of the splenic artery in the hilum or the ligation of one of its branches (3, 4, 6). It was also demonstrated, by means of arteriographies, that the collateral arterial network develops rapidly after the ligation of the splenic artery (5). Finally, in experimental animals, revascularization was demonstrated by the development of small vessels bridging the two parts of the ligated artery (2). The ligation of the splenic artery as an adjunct to splenorrhaphy in rare injuries involving hilar vessels, has the immunologic advantage of preserving larger splenic mass than partial splenectomy. the latter is reserved for treatment of injuries with emaciation of splenic tissue.
Unroofing of pseudocysts and oversewing of the capsule was recommended by T o u l u k i a n (9) and gave excellent results in this series. Contrary to the recommendation that hydatid cysts should be treated by splenectomy (or at least partial splenectomy), we treated 2 patients by mere aspiration of the contents and washing out the cavity with 20% saline solution. This is the usual mode of treatment of solitary hydatid cysts in other organs, such as the lungs and occasionally the liver. Long term follow-up of 2 and 5 years respectively, confirmed the efectiveness of this minor procedure on solitary hydatid cysts of the spleen.
It is concluded from this material that the spectrum of indications of SMSP is large. SMSP are widely accepted in the surgery of the spleen and with increasing experience (11) some of them are now performed by means of laparoscopy.