Ectopic Gland Research Articles

Correlation between ultrasound features and histopathological finding in cases of adenomyosis: a cross-sectional study

Background Adenomyosis is a gynecological disorder characterized by hypertrophy and hyperplasia of the surrounding myometrium and the presence of ectopic endometrial glands and stroma inside the myometrium. Aims To compare the accuracy of preoperative ultrasound (US) features with the histopathological results for the diagnosis of adenomyosis, and to determine its sensitivity for diagnosis. Patients and methods A total of 100 perimenopausal and postmenopausal patients (aged 40 to 60 years old) presented with abnormal uterine bleeding. Every patient underwent a thorough history taking, general examination, and pelvic examination. The selected patients were subjected to all laboratory investigations, abdominal and vaginal US, and abdominal or vaginal hysterectomy followed by histopathological examination. Results 45 (45%) patients had adenomyosis by transvaginal US and 52 (52%) patients had adenomyosis by histopathology with a P value of 0.105 which means that there is no significant difference between transvaginal US and histopathology in the diagnosis of adenomyosis. Transvaginal US had an 89.0% diagnostic accuracy for adenomyosis, with 82.7% sensitivity, 95.8% specificity, 95.6% positive predictive value, and 83.6% negative predictive value. Conclusion Transvaginal US is an important diagnostic technique for adenomyosis, and there is no significant difference between ultrasound and histology in the diagnosis of the condition.

The role of anatomical and functional orientation in identification of parathyroid glands for patients with parathyroidectomy.

To investigate diagnostic approaches for preoperative localization of secondary hyperparathyroidism, as well as to give surgeons with precise parathyroid gland localization and imaging so that surgery can be performed safely. The clinical data of 710 patients with secondary hyperparathyroidism who underwent surgery in our center from October 2009 to October 2023 were retrospectively analyzed. The changes in calcium, phosphorus, and parathyroid hormone levels were observed to ascertain the anatomical location and number of parathyroid glands. Among the 710 patients, 55 underwent total parathyroidectomy, the others underwent total parathyroidectomy with autotransplantation. In total, 2,658 parathyroid glands were removed, with 43 glands being removed in 35 reoperation cases. The median parathyroid hormone level at 6 months postoperatively was 13.40 (interquartile range, 7.00-29.80) pg/mL. The detection rates of the parathyroid glands before first and repeat surgeries were higher using 99mTc-MIBI SPECT/CT fusion imaging than ultrasound (P<0.05). The sensitivity of combined preoperative 99mTc-MIBI SPECT/CT and ultrasound was 92.31%, higher than that of either 99mTc-MIBI SPECT/CT fusion imaging or ultrasound alone (P < 0.05). The incidence of ectopic parathyroid glands was 23.8%, and the incidence of ectopic left lower parathyroid glands was 13.2%. The left lower parathyroid gland was the most prone to ectopia. 99mTc-MIBI SPECT/CT fusion imaging, paired with high-frequency ultrasound, can be utilized to diagnose SHPT preoperatively. The most common ectopia site is the left lower parathyroid gland, which is located primarily in the thymus and superior mediastinum. Understanding the functional anatomical distribution of the parathyroid glands is critical for developing effective surgical methods for secondary hyperparathyroidism.

Origin of Peritoneal Cancer With Features of High-grade Serous Carcinoma: A Detailed Molecular Analysis.

Primary peritoneal cancer has characteristics similar to high-grade serous carcinomas of ovarian and fallopian tube origin. However, the relationship between endometriosis and primary peritoneal cancer is not well understood. This study focuses on a case of peritoneal cancer in a patient who had undergone hysterectomy and bilateral salpingo-oophorectomy 5yr before. In addition to morphology, there was a positive for TP53 in immunohistochemistry and homologous recombination deficiency test, which were similar to high-grade serous carcinomas. However, WT1 was negative in the tumor, and extensive endometriosis coexisted. To reveal the clonal relationship between tumor and endometriosis, we dissected 3 sites each from the tumor and endometriosis and performed whole-exome sequencing analysis. As a result, we found that the tumors were of identical origin. Contrarily, no shared mutations were found in the 3 endometriosis sites. Furthermore, several shared mutations were found between the tumor and 1 endometriosis tissue, showing that the tumor and 1 ectopic endometrial gland originated from the same clone. This study indicates that several peritoneal cancers may be derived from endometriosis. We should consider the possibility of more diverse origins of peritoneal cancer than we speculated before.

A rare case of concomitant endometrioid adenocarcinoma arising from uterine adenomyosis and clear cell carcinoma arising from parametrial deep endometriosis

BackgroundCarcinomatous changes from the ectopic endometrial glands in endometriosis have been reported in many studies, but malignant transformation from uterine adenomyosis/adenomyoma is rare. And clear cell-like adenocarcinoma represents a seldom-encountered malignant pathological variant of ectopic endometrium.Case presentationThis case report presents a case of a 44-year-old nulliparous woman begun with abdominal pain and intestinal obstruction. Past medical history showed laparoscopic ovarian endometriotic cyst excision. Ultrasound indicated adenomyoma and a parametrial hypoechoic nodule with abundant blood flow signals and unclear boundaries. Deep invasive endometriosis was considered preoperatively. The patient underwent laparoscopic subtotal hysterectomy and bilateral adnexa resection. Chocolate cyst-like lesion was observed in the parametral lesion. Postoperative pathological examinations suggested endometrioid adenocarcinoma arising from eutopic endometrium and adenomyoma. Ectopic endometrium in the myometrium combined with atypical hyperplasia and formation of endometrioid adenocarcinoma. Left parametrial lesions suggested poorly differentiated endometrioid adenocarcinoma combined with clear cell carcinoma. CD10 + endometrial stromal cells were observed surrounding tumor cell masses. Combined with surgical founding and pathological characters of the left parametrial adenocarcinoma, the parametrial lesions were more likely to be carcinomatous changes of the original deep endometriosis.The patient underwent subsequent transabdominal tumor cell reduction surgery and chemotherapy.ConclusionWe herein present a rare case of combined endometrioid adenocarcinoma arising from uterine adenomyosis and clear cell carcinoma arising from parametrial deep endometriosis that may help inspire additional studies in the future. The patient underwent robot-assisted laparoscopic subtotal hysterectomy, bilateral adnexa resection, deep endometriosis lesion resection and bilateral ureteral stent placement. Following surgery, a chemotherapy regimen of Taxol and Carboplatin was administered.

Video-Assisted Thoracoscopic Surgery Is a Safe and Feasible Technique for Mediastinal Parathyroid Lesions.

Introduction: Hyperfunctional ectopic parathyroid glands in the mediastinum pose a challenge to diagnosis and require optimal surgical management. Video-assisted thoracoscopic surgery (VATS) has emerged as a promising minimally invasive approach, offering potential benefits in terms of both patient comfort and oncological principles. This study aimed to evaluate the effectiveness and safety of VATS for the treatment of hyperfunctional ectopic parathyroid glands in the mediastinum. Methods: Among the 538 patients with mediastinal tumors who underwent thoracoscopic surgery at Istanbul University (2008-2021), 11 exhibited hyperfunctional ectopic parathyroid glands. The localization of the glands was performed using various diagnostic techniques, including neck ultrasound, sestamibi scan, CT (computerized tomography), and SPECT (Single-photon emission computed tomography). VATS (Video-assisted thoracoscopic surgery) was used to remove ectopic parathyroid glands in all 11 patients, with no need for conversion to open surgery. Results: The pathological results showed that VATS successfully removed the ectopic glands in all 11 patients. Serum parathyroid hormone (PTH) levels were monitored intraoperatively, and frozen sections were used to confirm the presence of parathyroid adenomas in all cases. Postoperative analysis showed that PTH levels dropped by at least 50% within 10-15 minutes after adenoma removal. Conclusion: VATS is a safe and effective method for the treatment of hyperfunctional ectopic parathyroid glands in the mediastinum with a low risk of complications.

Robotic-assisted ectopic mediastinal parathyroidectomy: a single institution experience and operative review for the thoracic surgeon.

Parathyroidectomy remains the only definitive cure for primary hyperparathyroidism (PHPT). In rare cases, ectopic hyperfunctioning glands are located in the mediastinum, necessitating a thoracic surgical approach. The objective of this project was to review a single high-volume institutional experience of this presentation, with specific attention to the use of a robotic-assisted thoracic surgery (RATS) approach. This was a single-center, 5-year retrospective cohort study. All patients who underwent RATS mediastinal mass resection (MMR) for PHPT at the University of Colorado Anschutz Medical Campus were targeted for inclusion. Patient cases were reviewed for demographics, history, operative data, laboratory values, and postoperative course. Eight patients underwent RATS-MMR for PHPT between 2018-2023. Median [interquartile range] operative time was 178 [138-213] minutes, and length of stay was 2.0 [1.5-2.0] days. One patient experienced post-operative chylothorax requiring dietary modification. There were no other 30-day complications or readmissions. Final pathology confirmed intrathymic parathyroid tissue in all patients. All patients achieved cure of PHPT. The robotic-assisted approach has low morbidity and associated hospital length of stay and can be safely used to cure PHPT. As this is a rare pathology with an infrequently utilized surgical approach, it is important to critically discuss the diagnostic evaluation and operative course, aimed at educating the thoracic surgeon who may encounter and assist in the management of these patients.

Strategies for reoperative parathyroidectomy

Reoperative parathyroidectomy presents unique challenges and increased risks compared to initial surgeries. A thorough preoperative assessment is crucial, incorporating history, physical exams, laboratory evaluations, imaging studies, operative notes, pathology reports analysis, and postoperative complication reviews. Ectopic parathyroid glands further complicate reoperative procedures, necessitating careful consideration in surgical planning. Radioguided parathyroidectomy offers a safe and successful option for reoperative cases, and is particularly beneficial for detecting ectopic glands.

First report—parotid tissue choristomas within and adjacent to TMJ ligaments

PurposeTo report the first examples of ectopic salivary glands of TMJ ligaments & capsules. Methods and MaterialsNine subjects with chronic TMJ disorders were found incidentally to have ectopic salivary glands (choristomas) within or near damaged TMJ ligaments and capsules. Clinicopathologic characteristics of 12 TMJs are summarized. ResultsThe 9 subjects were ages 22-64 years (average = 43.0); 6 were female. Microscopic examination revealed major, almost complete ligament replacement by dense, avascular fibrous scar tissue in all but 1 sample. Chronic inflammatory cells were present in the stroma of 3 samples. A total of 33 lobular, independent salivary structures of variable sizes where embedded in or near ligaments and/or capsules. Half were multiple in the same joint; 13 were bilaterally located in the joints of a single subject, with 8 small glandular lobules in one TMJ. All glands consisted of histologically normal serous acini except 1 which showed a focal lobule of mucus acini and another comprised only of mucus acini. Serial sections showed no connection to parotid glands. Some choristomas showed the aging phenomena of fatty infiltrates (n = 7) and oncocytosis (n = 7). Additionally, 3 of the 12 TMJ samples contained preauricular lymph nodes, with 3 nodes in 1 joint and bilateral involvement in 1 subject. Of the 5 identified nodes, all but one was hyperplastic, with nonspecific inflammatory histiocytosis. ConclusionsWe report, for the first time, independent salivary gland choristomas (n = 33) within the soft tissues of the TMJ (n = 12), with one joint containing 8 such choristomas. All consisted of normal serous tissue except one, a mucus gland. Additionally, a fourth of TMJ surgical samples contained hyperplastic preauricular lymph nodes.

Dual-tracer subtraction scintigraphy combined with SPECT/CT in preoperative patients with primary hyperparathyroidism.

Abstract.

Fordyce Spot Symptoms and Treatment: An Updated Review

Fordyce granules occur frequently, and most people consider their quality to be a typical variation of the oral mucosa. The multiple yellow granules that primarily show up in the lower lip are a clear sign of Fordyce granules, a type of benign ectopic sebaceous gland. Most often, it affects adult males. Although it has been proven that such glands have been there since the time you were born, their tubes flow directly onto the skin’s surface. When they develop as a consequence of steroid hormones released by the gonadal and adrenal glands, they are not visible until pubescence. Around 70 to 80% of the population are adults. A comprehensive analysis of the literature search was conducted. A number of databases have been searched using the common terms “sebaceous glands,” “oral mucosa”, “Fordyce patches”, “Fordyce granules,” “Fordyce spots,” and “Fordyce glands” using Science Direct, Springer PubMed and Google Scholar as search engines. We have focused on the reviews and research papers published between 2001 and 2024. Although they are a common occurrence in the body, they are not connected to any disease or condition and are not contagious. They can be treated with isotretinoin, picrolimus, 5-aminolaevulinic acid, CO2 laser therapy, and micropuncture therapy. Hormonal fluctuations, greasy sebaceous glands, and hyperlipidemia bring on Fordyce spots. This is a rare but serious condition that causes lesions on the skin’s surface along with pain and discomfort in the affected part of the body.

Scar endometriosis as an easily misdiagnosed abdominal mass: a case report and review of the literature

Scar endometriosis, which is characterized by ectopic endometrial-like glands and stroma surrounding scar tissue, is rare and presents diagnostic challenges due to its inconsistent presentation. We report a case of a 40-year-old woman who had previously undergone two cesarean sections and a hysterectomy, in whom a mass in the subcutaneous layer of the lower abdomen was incidentally discovered by computed tomography (CT). A physical examination revealed a linear cesarean scar with a palpable, pigmented mass. An enhanced abdominal CT scan revealed an approximately 3.5-cm ill-defined soft tissue mass infiltrating the rectus abdominis muscle. A desmoid tumor was suspected based on the radiological findings, and endometriosis was also considered in light of the patient’s medical history. Wide mass excision was performed, ensuring 5-mm margins. The mass was completely excised, and a histopathological examination revealed endometriosis. Due to its rarity, scar endometriosis in the abdominal area can be easily misdiagnosed. Therefore, a heightened suspicion of scar endometriosis should be maintained in female patients with a history of abdominal or pelvic surgery presenting with an abdominal mass. Although imaging modalities can play a supportive role in the diagnosis, an extensive medical history assessment and comprehensive physical examination remain crucial.

The value of ultrasound in combination with 99mTc-MIBI imaging department of ultrasound medicine, for the diagnosis of ectopic parathyroid glands in the thyroid gland in patients with secondary hyperparathyroidism.

To investigate the value of preoperative ultrasound combined with 99mTc-MIBI imaging for the diagnosis of ectopic intrathyroid parathyroid gland (ETPG) in patients with secondary hyperparathyroidism (SHPT). One hundred and eleven patients with SHPT who underwent total parathyroidectomy plus forearm transplantation from January 2015 to January 2022 in the Third Hospital of Hebei Medical University were selected. All patients underwent routine preoperative ultrasonography and 99mTc-MIBI imaging, and with pathological diagnosis as the gold standard, the clinical data of ETPG patients were selected, including clinical manifestations, laboratory tests, preoperative ultrasonography and 99mTc-MIBI imaging for localization and diagnosis, intraoperative exploration and postoperative pathology, and postoperative follow-up. To analyze the ultrasound manifestations of preoperative parathyroid hyperplasia and the results of 99mTc-MIBI imaging in patients with ETPG. Among 111 patients with SHPT, there were 5 patients with ETPG, 1 male and 4 females with a mean age of (45.00 ± 5.05) years, and 6 ectopic parathyroid glands were located in the thyroid gland. The incidence of ETPG was 4.5% (5/111), 4 were detected by ultrasound, 2 were not detected with a diagnostic accuracy of 66.7% (4/6), 3 were positive for 99mTc-MIBI imaging, 3 were negative with a diagnostic accuracy of 50.0% (3/6). Among them, one was not detected by ultrasound, but was positive for 99mTc-MIBI imaging, 2 with negative 99mTc-MIBI imaging, but all were detected by ultrasound, and one with negative 99mTc-MIBI imaging was detected by ultrasound but misdiagnosed as a thyroid nodule. A total of 5 ETPGs were detected by ultrasound combined with 99mTc-MIBI imaging, with a diagnostic accuracy of 83.3% (5/6). Patients' postoperative serum calcium and serum parathyroid hormone (PTH) levels were normalized or significantly decreased from preoperative levels. Ultrasound combined with 99mTc-MIBI imaging can achieve higher accuracy than either examination alone in the preoperative localization and diagnosis of ETPG in SHPT patients.

Superior Parathyroid Masquerading as Retropharyngeal Lymph Node.

Ectopic parathyroid glands have been reported with an incidence of 2-22%. Undescended parathyroid glands, defined as glands situated above the carotid bifurcation or > 1cm cranial to the superior pole of the thyroid gland, comprise 2-7% of all ectopic parathyroid glands. We report a case of incidentally discovered parathyroid gland located in the retropharyngeal space at the level of the oropharynx. The patient in this report was a 60-year-old female with squamous cell carcinoma of the tonsil T2N0M0 with MRI showing a hyperintense ovoid structure, medial to the carotids at the level of the oropharynx, corresponding to the location of the lateral group of retropharyngeal lymph nodes. Patient underwent transoral ultrasonic radical tonsillectomy along with a retropharyngeal lymph node dissection by the transcervical route. Postoperative histopathology revealed the retropharyngeal node sampled to be a normal parathyroid gland. "High" undescended parathyroid gland as reported here along with 4 other cases of infratemporal fossa parathyroid glands reported previously, can be the cause for recurrent missed adenomas. Being extracervical in location, these are likely to be missed if the skull base has not been included in the preoperative Tc-99 sestamibi scan and also during bilateral neck exploration.

Thoracoscopic resection of paraesophageal ectopic mediastinal parathyroid adenoma in the superior posterior mediastinum: a case report

BackgroundThe ectopic superior parathyroid in the tracheoesophageal groove and paraesophageal region is rare. Hyperparathyroidism results when these glands become hyperfunctioning. That may necessitate surgical intervention in the form of parathyroidectomy, which requires a transsternal or transthoracic approach due to a deeply seated mediastinal parathyroid gland. Minimally invasive strategies have emerged recently as an alternative approach with less morbidity.Case presentationWe present a case of the paraesophageal ectopic parathyroid gland in the superior posterior mediastinum, which was successfully treated with thoracoscopic resection.ConclusionThe current imaging tools improve the thoracoscopic management of mediastinal parathyroid glands. Video-assisted thoracoscopic surgery (VATS) can provide access and exposure to ectopic parathyroid adenoma with low morbidity and financial burden.

Impact of Sonic Hedgehog-dependent sphenoid bone defect on craniofacial growth.

The main objective of this study was to evaluate how an apparently minor anomaly of the sphenoid bone, observed in a haploinsufficient mouse model for Sonic Hedgehog (Shh), affects the growth of the adult craniofacial region. This study aims to provide valuable information to orthodontists when making decisions regarding individuals carrying SHH mutation. The skulls of embryonic, juvenile and adult mice of two genotypes (Shh heterozygous and wild type) were examined and measured using landmark-based linear dimensions. Additionally, we analysed the clinical characteristics of a group of patients and their relatives with SHH gene mutations. In the viable Shh+/ - mouse model, bred on a C57BL/6J background, we noted the presence of a persistent foramen at the midline of the basisphenoid bone. This particular anomaly was attributed to the existence of an ectopic pituitary gland. We discovered that this anomaly led to premature closure of the intrasphenoidal synchondrosis and contributed to craniofacial deformities in adult mice, including a longitudinally shortened skull base. This developmental anomaly is reminiscent of that commonly observed in human holoprosencephaly, a disorder resulting from a deficiency in SHH activity. However, sphenoid morphogenesis is not currently monitored in individuals carrying SHH mutations. Haploinsufficiency of Shh leads to isolated craniofacial skeletal hypoplasia in adult mouse. This finding highlights the importance of radiographic monitoring of the skull base in all individuals with SHH gene mutations.

The anterior lobe of the parotid gland：A CT sialographic study.

To investigate the imaging and anatomic features of the anterior lobe (AL) of the superficial parotid gland. Computed tomographic sialography examinations were undertaken for 142 parotid glands in 77 patients. Whole computer tomography (CT) data were analyzed using multi-planar reformation and maximum intensity projection to generate sialographic CT images. The tributary ducts of the superficial parotid gland were analyzed to classify the parotid morphology. Three-dimensional analyses were used to investigate the AL and its relationship with adjacent anatomic landmarks. Four major types (I-IV) and two minor types (V-VI) of the AL and the superficial parotid gland were observed. Type I AL (83/142) was contiguous and not separated from the retromandibular parotid gland. Type II AL (16/142) was detached from the retromandibular parotid gland with one to four tributary ducts. Type III AL (12/142) showed a small isolated lobe above the Stensen's duct around the anterior edge of the masseter. Type IV (28/142) showed the absence of the AL. Type V (3/142) shows the absence of the retromandibular parotid gland. Type VI (3/142) showed the presence of ectopic salivary gland beneath the Stensen's duct anterior to the retromandibular parotid gland. The AL gives rise to the morphological variations of the superficial parotid gland. AL also gives rise to the accessory parotid gland when it is detached from the retromandibular parotid gland.

Intraoperative radio-guided localization of parathyroid adenomas using 3D freehand SPECT technology.

Parathyroidectomy for primary hyperparathyroidism (PHPT) could have poor outcomes, even with accurate preoperative localization of the adenomas, because their intraoperative localization can be challenging. Freehand single photon emission computed tomography (fhSPECT) is a new technique for radio-guided intraoperative navigation. Its use during parathyroidectomy could be useful and such data are limited. We herein present our experience on the feasibility of fhSPECT for intraoperative detection of abnormal parathyroid glands. We retrospectively reviewed the clinical data of 55 patients (30-77years old) with PHPT due to parathyroid adenomas, that were subjected to parathyroidectomy from 12/2017 to 7/2022. In average, 111 ± 74MBq of Tc-99m Sestamibi were injected intravenously, approximately 2h before the operation and fhSPECT was used to generate 3D images during parathyroidectomy. Measurements of PTH and calcium levels were performed preoperatively, postoperatively and 4-6months after the procedure. FhSPECT successfully identified the parathyroid adenoma in all the patients. It took 3min (median time) for fhSPECT to detect at least one radioactive spot in all patients. The mean duration of the operation was 66.6 ± 7.3min. Forty-nine patients out of 55 had solitary and 6/55 had multiple adenomas, whereas 6/55 had ectopic abnormal parathyroid glands. None of the patients had persistent hyperparathyroidism during follow-up. To the best of our knowledge, this is the largest series of patients with PHPT that underwent fhSPECT assisted parathyroidectomy. Our data suggest that this navigation system is helpful in identifying parathyroid adenomas intraoperatively.

Role of the gut microbiota in the pathogenesis of endometriosis: a review.

Endometriosis is classically defined as a chronic inflammatory heterogeneous disorder occurring in any part of the body, characterized by estrogen-driven periodic bleeding, proliferation, and fibrosis of ectopic endometrial glands and stroma outside the uterus. Endometriosis can take overwhelmingly serious damage to the structure and function of multi-organ, even impair whole-body systems, resulting in severe dysmenorrhea, chronic pelvic pain, infertility, fatigue and depression in 5-10% women of reproductive age. Precisely because of a huge deficiency of cognition about underlying etiology and complex pathogenesis of the debilitating disease, early diagnosis and treatment modalities with relatively minor side effects become bottlenecks in endometriosis. Thus, endometriosis warrants deeper exploration and expanded investigation in pathogenesis. The gut microbiota plays a significant role in chronic diseases in humans by acting as an important participant and regulator in the metabolism and immunity of the body. Increasingly, studies have shown that the gut microbiota is closely related to inflammation, estrogen metabolism, and immunity resulting in the development and progression of endometriosis. In this review, we discuss the diverse mechanisms of endometriosis closely related to the gut microbiota in order to provide new approaches for deeper exploration and expanded investigation for endometriosis on prevention, early diagnosis and treatment.

Surgery for nonlocalizing hyperparathyroidism in high volume center.

Patients with nonlocalizing hyperparathyroidism pose a significant challenge to surgeons when undergoing neck exploration for parathyroidectomy. We evaluated 536 patients that had parathyroidectomy for primary hyperparathyroidism (PHPT) from 2005 to 2018 at a single tertiary academic center, and 155 (29%) had standard nonlocalizing preoperative imaging (negative ultrasound and sestamibi scans). There were a total of 102 (66%) non-ectopic single adenomas in the nonlocalizing group and 325 (85%) single adenomas in the localizing group. There was no significant difference (p = 0.09) in adenoma quadrant between localizing and nonlocalizing single adenomas, but the most common location in both groups was right inferior. Patients with nonlocalizing scans were more likely to have double adenomas (21% vs. 9%, p < 0.001), ectopic glands (10% vs. 5%, p = 0.052), and multi-gland disease (13% vs. 8%, p = 0.002). Nonlocalizing PHPT patients experienced similar cure and complication rates as localizing PHPT, but required more bilateral explorations and increased operative time.

Neonatal Severe Hyperparathyroidism: A Case Report

Background: Neonatal severe hyperparathyroidism is an extremely rare condition that presents in the first few months of life. Recognition at the early stage followed by prompt surgical intervention are of vital importance for survival. Hypotonia, respiratory distress, lethargy and delay in growth and development occur in association with the elevated serum parathormone levels and hypercalcemia. Definitive therapy involves total parathyroidectomy. Case Description: We are presenting a case with Neonatal severe hyperparathyroidism, who underwent total parathyroidectomy. The patient had been followed up with medical therapy until. Parathormone levels rapidly declined following total parathyroidectomy and the patient was discharged with full recovery. Literature Review: Sestamibi scintigraphy might fail to show an ectopic parathyroid gland. In such conditions, on table confirmation of parathyroid glands by frozen section and Intraoperative parathormone monitoring is vital at this point. Persistently elevated parathormone levels might suggest a remnant parathyroid tissue at the surgical site or an ectopic parathyroid gland that should be excised. Clinical Relavance: Neonatal severe hyperparathyroidism is a life-threatening disease. In the present case surgery performed at the early is life-saving due to the failure of medical therapy to control the disease.