An Autopsy Report of Hypoplastic Coronary Artery Disease Incidentally Identified in a Young Man

Hypoplastic coronary artery disease (HCAD) is a rare congenital abnormality characterized by underdevelopment of one or more major branches of the coronary arteries, which may lead to myocardial infarction and sudden death. We report a case of HCAD that was incidentally detected during autopsy in a 25-year-old male soldier who died during underwater escape training. On macroscopic examination, the right coronary artery measured 1 mm in diameter, which was significantly smaller than that of other coronary arteries. We observed no anomalies in addition to HCAD. Death was attributed to drowning, which was consistent with autopsy findings and the circumstances of death. Although extremely rare, if sudden cardiac death is suspected in children and young adults, it must be confirmed through careful evaluation.

High Risk Features of an Anomalous Origin of the Right Coronary Artery

Anomalous aortic origin of the coronary arteries (AAOCA) is a rare congenital abnormality. It is usually asymptomatic and often found incidentally during coronary angiography. However, it can also be discovered during the autopsy of young healthy adults who have suffered from sudden cardiac death (SCD). AAOCA represents the second most common cause of SCD in young athletes. Herein, we report a case of a 39-year-old patient with left-sided right coronary anomaly with multiple high-risk features who presented with life-threatening symptoms for SCD but normal electrocardiography, echocardiography, and cardiac markers. The coronary computed tomography revealed an anomalous coronary artery from the left sinus of Valsalva with a hypoplasic origin and a high-risk path between the aorta and the pulmonary artery with a short intramural path. He was surgically managed with a coronary artery bypass with an uneventful follow-up.

Management of bladder exstrophy in an adolescent girl: a case report

Bladder exstrophy (BE) is a very rare congenital abnormality, especially in girls. We reported a 17-year-old girl with protruded bladder and urinary incontinence. Her vaginal orifice appeared stenotic and shifted anteriorly. She had an incomplete labia minora, short perineum, and anterior displacement of the anus. The pelvic radiography showed a pubic diastasis with a manta ray sign, and the ultrasonography examination showed a bicornuate uterus. She had undergone a functional reconstruction surgery with cystectomy, ileal conduit, appendectomy, and vaginoplasty along with the advancement of abdominal flap reconstruction with multiple Z-plasty. Functionally, she was able to urinate through the ileal conduit comfortably, had a regular menstrual cycle with minimal pain, had a wider vaginal canal, and had a smooth blood flow. Aesthetically, she had a good lower abdomen appearance, remained dry, and had an odorless urine. In clinical practice, this rare case report can provide additional knowledge and management of the same cases with effective results.

Zinner Syndrome with Ectopic Ureter Remnant

: Zinner syndrome is a rare congenital abnormality defined by a clinical triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst, and ipsilateral ejaculatory duct obstruction. Most patients are asymptomatic, but if the cystic dilatation of the seminal vesicle becomes significant, it can result in urinary symptoms such as dysuria and urinary retention. This rare developmental anomaly related to the mesonephric duct can also present with other abnormalities. Here, we report our experience of Zinner syndrome with bladder outlet obstruction and an ectopic ureter remnant.

Clinical Presentations and Usefulness of 99mTchnetium-Ethylene-Dicysteine (99mTc-EC) Renal Scintigraphy in Estimation of Thoracic Kidney Function in Children

: Thoracic kidney is a rare congenital abnormality with the lowest frequency of all renal ectopias. Most cases are discovered incidentally via routine imaging and have a benign clinical course. Here, we report three pediatric cases of thoracic kidney among 156 children with renal ectopy (1.9%). Thoracic kidney was asymptomatic in two children; in one child, it was associated with a recurrent diaphragmatic hernia. All diagnoses were made by chest ultrasonography (USG) and/or radiography in children and confirmed by computed tomography (CT) scan. Moreover, 99mtechnetium-ethylene-dicysteine (99mTc-EC) renal scintigraphy was carried out to visualize and assess the function of ectopic kidneys. All thoracic kidneys were left-sided, and their functions were comparable to their normally located counterparts. During an observation period of 5 - 10 years, two children with thoracic kidneys presented with unstable proteinuria/microalbuminuria and hypertension, respectively. The 99mTc-EC renal scintigraphy is an uncomplicated and safe method for imaging the thoracic kidneys and can be used for detecting the thoracic kidney function in children. Thoracic ectopic kidneys are associated with complications, such as hypertension and proteinuria. Children with thoracic kidneys need long-term follow-ups.

Nasal glial heterotopia: A rare interdisciplinary surgical challenge in newborns

Nasal Glioma (NG) represents a rare congenital abnormality of the neonate, which can be associated with skull defects or even a direct communication to the central nervous system. MRI serves valuable information for differentiation from encephalocele, dermoid cyst and congenital hemangioma. Complete resection remains the treatment of choice. We present two cases of NG, which were both suspected during prenatal ultrasound and MRI. In the first case, postnatal MRI showed a transcranial continuity. Mass excision was performed and the defect was covered by a glabellar flap allowing a good cosmetic result. Postnatal MRI excluded a trans-glabellar communication in the second case. After surgical excision, the resulting skin defect was covered with a full thickness skin graft harvested from the right groin. In cases of NGs complete resection and cosmetic appealing results can be achieved and might necessitate a multidisciplinary approach.

Complete Thoracic Ectopia Cordis, Dilemma of the Outcome: A Case Report

Background: Ectopia Cordis (EC) is a rare congenital condition where the heart is partially or completely lies outside the thoracic cavity (extrathoracic), uncovered by pericardium and skin. Many works of literature reported EC is a rare congenital abnormality with an incidence of about 5-8 per 1 million live births and includes about 0.1% of congenital heart diseases. Methods: This was a male baby, weighing 2.900 kg, received with cyanosis with a defect in the anterior chest wall and heart protruding out through it. On initial physical examination, split sternum with complete thoracic EC (beating outside the thoracic cavity with a complete absence of the pericardium with the apex pointing upwards) were reported. Results: The baby’s poor general condition did not allow further radiologic studies and echocardiography could not be performed. By the time, an arrangement had been planned for him to undergo cardiothoracic referral; unfortunately, he ran a downhill course and succumbed within 36 hours of life. Conclusion: The important for submitting such findings will assist our team: pediatric surgeons, obstetricians, pediatricians and, neonatal intensivists to develop future management strategies when they are enrolled or confronted with such cases, by improving the outcome through a precise workup design to provide the optimal evaluation, diagnosis, and management roadmaps of potential cases of EC.

A Case of Large Aplasia Cutis Congenita with Underlying Skull Defect: Effective Surgical Treatment with Integra® Dermal Regeneration Template

<b><i>Introduction:</i></b> Aplasia cutis congenita (ACC) is a rare congenital abnormality characterized by the absence of a portion of skin at birth which most commonly involves the scalp and can affect the galea, the pericranium, the bone, and the dura mater. It can be an isolated condition or associated with other disorders. <b><i>Case Report:</i></b> We present a case of ACC with a large defect of the scalp and the underlying bone treated with the use of Integra® Dermal regeneration template. At 5 months of follow-up, the wound is completely healed and the bony defect greatly reduced. Contraction of the area of alopecia was observed. <b><i>Discussion:</i></b> Several surgical and conservative options have been described to treat this congenital condition: advanced dressing, skin graft, local flaps, free flaps, and other methods. In our case, we used Integra® Dermal templates which provide a barrier for infections, promote cellular activity for a rapid vascularization, and improve healing.

Primary Closure of a Subtotal Sternal Cleft in a 7-Day-Old Neonate

Sternal cleft is a rare congenital abnormality that results from incomplete fusion of the two lateral mesodermal sternal bars. It is generally accepted that primary repair in the neonatal period is the best treatment option. However, significant distance between the sternal bars can be challenging because of cardiac compression. The aim. We report a case of a 7-day-old neonate with a subtotal sternal cleft successfully managed by direct closure. Material and methods. A full-term male neonate weighing 3 kg was referred to our clinic for evaluation of a chest wall defect. The chest X-ray and computed tomography were performed to evaluate the malformation. Results. Surgery was performed at the age of 7 days. Postoperative period was uneventful. The patient was discharged on the postoperative day 21. The first postoperative checkup after 3 months showed satisfactory cosmetic results and normal respiratory movements. Conclusion. Despite the significant diastasis between sternal bars, primary direct closure of the sternal cleft can be safely performed in neonates.