MRKH syndrome, or Mayer-Rokitansky-Küster-Hauser syndrome, a rare congenital disease, manifests as a complete or partial aplasia of the uterus and the vagina\'s upper two-thirds with normal external genitalia and functioning ovaries. Mayer-Rokitansky-Küster-Hauser syndrome can occur in isolation (type I) or in conjunction with other congenital extragenital deformities affecting the kidneys, skeleton, heart, eyes, or auditory system (type II). The diagnosis of Mayer-Rokitansky-Küster-Hauser syndrome typically relies on imaging studies, with transabdominal ultrasonography serving as the primary modality. However, magnetic resonance imaging is considered the gold standard for detailed assessment of internal genital anatomy. We present the case of an 18-year-old woman without any notable medical history who exhibited primary amenorrhea. Mayer-Rokitansky-Küster-Hauser syndrome type II was suspected on pelvic ultrasound and subsequently confirmed via magnetic resonance imaging. The patient was provided with psychological assistance and planned for vaginoplasty.

BACKGROUND: Isolated Patellar Aplasia Hypoplasia is a very rare autosomal dominant disorder. Its treatment depends on the clinical manifestations that can vary widely. The lack of active extension, which can be responsible for frequent falls due to a knee instability, is the most frequent and disabling manifestation. We report an original technique that is a modification of the Galeazzi technique for recurrent dislocation of the patella to gain active extension in case of PTLAH.
METHODS: A 7-year-old Caucasian boy with isolated Patellar Aplasia Hypoplasia and an extension lag of the right knee has been treated by a modified Galeazzi technique. The tendons of the semi-tendinous and gracilis muscles have been harvested and their distal insertion was kept intact. Both tendons were fixed over the top of the patella to restore knee active extension. After 6 years of follow up the patient is symptom free with a strong active extension of the operated knee.
CONCLUSIONS: Reconstruction of isolated hypoplasia of the patella by a modified Galeazzi procedure is a safe and reliable technique for skeletally immature patients offering satisfying long-term outcomes.

Bilateral variation of the parotid gland is an anatomically rare entity. In this study, we present a 91-year-old female cadaver with aplasia of the parotid gland on the left side of the face and hypoplasia of the parotid gland on the right side of the face. The accessory parotid glands on both sides were of normal size, while bilateral submandibular glands were found to be larger than normal. The surgical anatomy of the parotid gland is very important because this region of the face is a complex structure intertwined with important vessels and nerves. Patients with aplasia, atresia, agenesis, or hypoplasia of the parotid gland should be diagnosed early and proper treatment must be planned. Consequently, this case was found to be remarkable in terms of the coexistence of parotid gland aplasia and hypoplasia.

UNASSIGNED: Aplasia of the salivary glands, either partial or involving all the major and possibly the minor salivary glands, is a very rare cause of hyposalivation. The aim of this case report is to present a case of aplasia of the major and minor salivary glands and discuss the relevant literature.
UNASSIGNED: A 23-year-old woman, with a non-contributory medical and family history was referred due to rampant caries, that could not be attributed to any obvious aetiology. No sicca symptoms, previous parotid gland swelling or general symptoms were reported. Clinically, oral mucosa dryness and extended dental erosions and caries were observed, while the orifices of the excretory ducts of the parotid and submandibular salivary glands were not evident. Unstimulated and stimulated saliva flow rates were severely diminished, while a diagnostic biopsy of the lower lip revealed absence of minor salivary glands. Detailed hematologic and immunological investigations to exclude systemic disorders were also within normal limits. Ultrasound and magnetic resonance imaging revealed the absence of all major salivary glands, confirming the clinical diagnosis of congenital aplasia of the salivary glands.
UNASSIGNED: Oral hygiene instructions and dietary advice were given while dental products with fluoride and saliva substitutes were administered and appropriate dental treatment was implemented. Regular dental follow-up was also advised.
UNASSIGNED: Timely diagnosis of aplasia of the salivary glands is important, considering the detrimental effects of the absence of saliva on oral health. Management consists of the use of saliva substitutes, nutritional adaptation, maintenance of oral health and regular dental follow-ups.

The congenital absence of major salivary glands, particularly the submandibular gland, is a rare condition. Although the etiology of aplasia is unknown, it is thought to occur owing to defects that emerge during early fetal development. Agenesis of 1 or more of the major salivary glands may occur alone or in association with other congenital anomalies. Very few cases of bilateral submandibular gland aplasia have been reported in the literature. Patients with this condition can be either symptomatic or asymptomatic. Due to the probability of there being additional anomalies, patients and their families should be carefully evaluated. We present the ultrasound and computed tomography findings for a case of bilateral submandibular gland aplasia that was detected incidentally. A review of the literature on major salivary gland aplasia was also conducted.

OBJECTIVE: Our goal was to investigate the cases of bilateral aplasia of the anterior cerebral artery (ACA).
METHODS: The macro- and microdissection of the target human brain arteries of 388 cadaveric cases was applied under the magnifying glass. Each case was photographed and diagrammatically represented in the workbook. The length and the outer diameter of the corresponding arteries on the photos were measured using a computer software program.
RESULTS: There was only one case (1/388 or 0.25%) of bilateral ACA aplasia that belonged to a male adult cadaver. Except for the variations of the posterior communicating artery on one side and the basilar artery, the instance of cerebral pathology was not recorded in this case. We compared the recent case with available literature cases.
CONCLUSIONS: Summarizing small number of literature cases, the recent case of bilateral ACA aplasia as the fifth case discovered so far, represents a true morphological rarity.

BACKGROUND: Congenital anomalies of lower lateral cartilages are very rare. Difficulties to detect before the operation may cause problems during the surgery. In this paper, we presented a unique deformity with the combination of aplastic and hypoplastic segments of lower lateral cartilage.
METHODS: A 21-year-old female patient who underwent rhinoplasty were enrolled in this study. Airway impairment, prominent nasal hump, droopy nasal tip and nostril asymmetries were observed in physical examination.
RESULTS: Partial aplasia and hypoplasia on the right-sided lobular segment and hypoplasia on the left-sided lobular segment of the middle crus were detected. Hypoplasia of the columellar segment and footplate segment of the medial crus was observed on both sides. Also, intercrural soft tissue volume was low. Defective medial crus was reconstructed with the septal cartilage graft.
CONCLUSIONS: Although a rare occurrence, awareness of congenital lower cartilage anomalies is important for surgical planning and reconstruction of deformities. Cartilage tissue reflection to the skin surface and examination of each tip segment is crucial to detect anomalies preoperatively.
METHODS: 4 (Therapeutic).

Despite severe immunosuppression due to conditioning chemotherapy for acute myeloid leukemia, COVID-19 did not lead to clinical deterioration or death, thus raising the question of the impact of immunosuppressive treatment on clinical course evolution.

Kartagener\'s syndrome (KS), characterized by a triad of bronchiectasis, chronic sinusitis, and situs inversus, is a subset of an autosomal recessive hereditary disorder of primary ciliary dyskinesia. We report the case of a 35-year-old male who presented with a history of intermittent episodes of productive cough, breathlessness, and cold since childhood. High resolution computed tomography of chest revealed bronchiectatic changes, dextrocardia, and right-sided aortic arch. Computed tomography (CT) scan of the abdomen revealed situs inversus. CT of the paranasal sinuses revealed combined aplasia of bilateral frontal and sphenoid sinus with sinusitis. Based on these findings, a diagnosis of KS was made. There was no complaint of infertility, which usually accompanies KS, even though an analysis of his seminal fluid revealed reduced count and reduced motility of sperms. The uniqueness of our case is that our patient was a male aged 35 years; besides, the third decade is an unusual age for presentation of combined aplasia/agenesis of bilateral frontal and sphenoid sinuses with hypoplasia of maxillary and ethmoid sinuses. Even though these findings have been reported in children and young adults, there are very few case reports of such a presentation in adults in literature.

OBJECTIVE: To assess the frequency of frontal sinus aplasia using computed tomography (CT) of the paranasal sinus among Saudi Arabian population.
METHODS: A retrospective case review of medical records from January 2019 to December 2019 in the Otorhinolaryngology Department, King Abdulaziz University, Riyadh, Saudi Arabia.
RESULTS: A total of 449 cases were reviewed. The incidence of bilateral frontal sinus aplasia was 3.3%. The incidence of right sinus agenesis was 5.12% and left was 1.33%. The mean age of reviewed patients was 39.15 years.
CONCLUSIONS: The frequency of frontal sinus aplasia is low; however, this must be considered before surgical operations related to the sinuses.