Possibilities of Early Ultrasound Diagnostics of the Herlin — Werner — Wunderlich syndrome (Case Report)

Herlin — Werner — Wunderlich syndrome or OHVIRA (Obstructed hemivagina and ipsilateral renal anomaly) is a rare type of uterine and vaginal doubling defect (0,1 – 10,0 % of all uterine abnormalities) which is characterized by a triad of symptoms: uterus didelphys, unilateral obstructed hemivagina, and ipsilateral renal dysgenesis. The manifestation of the disease usually present after menarche, is accompanied by severe pain syndrome, leads to redoubted complications and organ-carrying operations with decreasing the fertility. At an earlier age, it can manifest itself as an infection of the genitourinary system. The diagnosis is based on clinical and anamnestic data using instrumental methods, the most accessible and safe of which is ultrasound. This article presents three clinical cases of patients 5 – 6 years old with a description of the ultrasound picture of the OHVIRA syndrome.

UNILATERAL SCROTAL HERNIA WITH DUAL URETER HERNIATION: THE FIRST EXPERIENCE OF SUCCESSFUL SURGICAL CORRECTION

Objective of the study: the purpose of the research, based on the example of a specific clinical case, is to draw the attention of specialists to a specific approach to the diagnosis and treatment of the inguinal hernia with atypical contents. A rare, unpublished in the Slovak Republic, observation of the left-sided scrotum hernia with dual ureter herniation in combination with congenital kidney pathology in a 3-month-old boy is presented. The diagnosis was based on physical examination, ultrasound examination, radiography, scintigraphy, and urethrocystoscopy. This anomaly has been successfully corrected through several successive phases of the operation. Conclusion: the combination of scrotal hernia and congenital renal anomaly may indirectly indicate a possible hernia in the distal part of the ureter and requires a thorough examination, including additional imaging methods.

CASE OF VACTERLASSOCIATION WITHOUT THE "V AND L": ANAESTHETIC CHALLENGES

Aim & Objective: To highlight the importance of anesthetic management in a small neonate with cleft lip, cleft palate, single kidney, atrial septal defect, posted for trachea-oesophageal stula repair. Case description:Afemale baby born at 35 weeks of gestation to a 23-year-old primigravida mother by caesarean section in view of foetal distress with breech presentation .Baby had difculty in breathing and froathing from mouth. Cleft lip and cleft palate was present. Anasogastric tube was not going beyond 11 cm per oral route. Chest x ray showed coiling of tube in upper esophagus.F/S/O Type C Tracheo esophageal stula. . Plain Xray abdomen showed presence of bowel gas. Ultrasonography of the abdomen showed right renal agenesis. The left kidney was normal. Echocardiography showed presence of atrial septal defect with left to right shunt, tiny patent ductusarteriosus, mild pulmonary arterial hypertension, mild tricuspid regurgitation. Based on the presence of tracheoesophagealstula, atrialseptal defect, unilateral renal agenesis and absence of features, suggestive of alternative diagnosis infant, meet criteria of vacteral association. Discussion: VACTERL is a cluster of congenital malformations based on the non-random association of various congenital malformations in a single patient. Here “V” denotes vertebral defects or vascular anomalies (single umbilical artery), “A” anal atresia, “C” cardiac abnormalities, “TE” tracheoesophageal stula, “R"renal (kidney) abnormalities and “L” for limb anomalies) Diagnosis of VACTERL association is done only when at least three of the above mentioned congenital malformations are identied in a patient. Although 80% of these cases have vertebral defects, our case is unique as patient does not have one of the commonest occuring association i.e., vertebral anomalies. The other highlight of this case is although reports say that VACTERL babies with ipsilateral renal disorder have the same side limb defects, our case has a renal anomaly with no limb anomaly. Conclusion: Anaesthetic challenges were difcult airway, endotracheal tube placement, low respiratory reserve, small maximum allowable blood loss, long duration of surgery, risk of hypothermia, aspiratedlungs, risk of right to left shunt, difculty in securing intravenous line and intra arterialline. this case needs continuous monitoring of ECG, invasive blood.