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Background: Traumatic ulceration of the tongue due to congenital teeth is known as Riga-Fede disease (RFD). The literature on RFD consists largely of anecdotal case reports, thereby lacking valid clinical recommendations. While managing a newborn with RFD complicated by Ludwig’s angina, this knowledge gap was recognized. Therefore, this systematic review was conducted to gain insight into the optimal management and the possible complications of RFD. Methods: A literature search was conducted using PubMed, Google Scholar, and Science Direct, supplemented with African Journals Online, Indmed, and manual searches. Papers published between 1900 and July 2023 in any language were retrieved. Neonates with natal or neonatal teeth and ulceration of the oral mucosa were included. Papers with incomplete data and review articles were excluded. Results: A total of 84 newborns with RFD from 62 published reports (including the case reported herein) were analyzed. Most of the reports were from the Indian subcontinent, and the frequency of reporting has increased since the year 2000. Tongue ulceration in all cases was due to mandibular incisors. There was no significant difference in the median duration of healing between the tooth extraction group (14 days) and the conservative management group (18 days). There was only a very weak positive correlation between the duration of symptoms and healing time, which was statistically insignificant. Conclusion: Healing of mucosal ulcers in RFD is often protracted and does not appear to be influenced by the duration of symptoms or by the modality of treatment. Extraction of the offending teeth is more common (75% of cases) than conservative management, such as grinding (ameloplasty) or resin capping of teeth. RFD is mostly a benign disorder; however, serious complications such as meningitis, Ludwig’s angina, and soft tissue abscesses can rarely occur with Staphylococcus aureus infections.

Background: Autosomal recessive cutis laxa type-1C is also known as Urban-Rifkin-Davis syndrome (URDS). It is known to affect cardiopulmonary, integumentary, gastrointestinal, musculoskeletal and genitourinary systems. However, its frequent association with congenital diaphragmatic hernia has not previously been highlighted. Case Presentation: A newborn male with cutis laxa presented with respiratory distress at birth. The cause of dyspnoea was perinatal strangulation of the stomach in hiatus hernia. After surgical repair of the hernia, his respiratory distress temporarily improved but kept recurring periodically by various mechanisms in sequence namely pulmonary hypertension, tracheomalacia, pulmonary emphysema and finally he succumbed to pneumothorax. Genetic analysis revealed his skin condition as autosomal recessive cutis laxa type-1C which is also known as URDS. Exome sequencing revealed a novel frameshift mutation c.426delC (p.Cys143Alafs*41) of the LTBP4 gene in the exon 5 of Chromosome 19. Conclusion: Out of the 28 cases of URDS reported in the world literature 57% had congenital diaphragmatic hernia (CDH) and 53% of them died during infancy. Such a high incidence of CDH is not observed in other subtypes of elastic disorders. Thus, congenital diaphragmatic defects appear to be a characteristic diagnostic feature of URDS in patients with cutis laxa.

In the presence of an appendicular mass, surgery is generally limited to the drainage of abscess. Scientific literature is sparse and controversial as to how the ongoing sepsis despite the drainage of appendicular abscess should be managed. Deliberate appendicectomy in the presence of mass formation is often not recommended as it may cause collateral damage to the adherent bowel loops. The authors describe a useful technique of doing appendicectomy by stripping the mucosa alone, leaving behind the adherent muscular cuff undisturbed. This technique is given an acronym “mucosa-coring salvage (MU-CO-SAL) appendicectomy.” This article is intended to draw the attention of pediatric surgeons to this useful technique, which remains underutilized despite being described almost a decade ago.

Background: Congenital masses of the nasal tip are extremely rare. Differential diagnoses include nasal-tip teratoma, nasal chondromesenchymal hamartoma (NCMH), and heterotopic nasal glioma. Herein, the case of a newborn is reported that does not match with any of these differential diagnoses. Case presentation: A newborn male presented with a congenital pedunculated mass arising from the nasal columella. The mass was symmetrically bilobed which is very unusual for any hamartomatous or neoplastic lesions. Histologically, it was composed of cartilage and ciliated epithelium. Conclusion: Based on the uniqueness of the case it is hypothesized that the lesion could be an embryological error of frontonasal process overshoot defying, hitherto unknown, controlling mechanism. More research is needed to know how the morphometric proportion of the human body is determined by Nature.

Management of ambiguous genitalia is highly controversial. This condition was known previously as intersex and presently as disorders of sex development (DSD). There is no consensus regarding the choice, timing and method of sex assignment in neonates with DSD. Consensus conferences could not unify the views of various stakeholders and third parties. This article philosophically examines the nature and origin of such controversies. Misconception, bias and conflicting priorities are identified as the three cardinal sources of controversies. Conceptual duality of sexes, confused notion of sex and gender, bias towards penetrative intercourse, conflict between utopian ideals and reality, unwillingness to compromise are identified as perpetuators of controversies. Suggestions are made regarding sex assignment in various types of DSD based on the understanding of published literature and the author’s personal experience.

Disseminated intravascular coagulation (DIC) is a contraindication for major surgical operations. However, if surgery is required to correct the underlying cause of DIC, a clinical conflict is created. In such complex scenario, standard resources such as textbooks and journals offer very little guidance. In this communication, we report a 22-month-old boy who developed sepsis-induced DIC following intestinal obstruction. Pre-operative attempts to normalize coagulation parameters failed. Damage control laparotomy was undertaken as it was considered essential to control the underlying cause of DIC. His abnormal coagulation status reverted quickly after surgical relief of intestinal obstruction. Paradoxically intraoperative blood loss was less than anticipated amount. There are a few case reports of adult patients who have successfully undergone major surgery despite the presence of abnormal coagulation. However, this appears to be the first paediatric report of successful surgery in DIC status. Lessons learnt from this case and hypothetical speculations of clinical paradoxes are discussed.

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Retroperitoneal hemorrhage is known to cause bruising of flanks (Turner's sign), umbilicus (Cullen's sign), pubis and groin (Stabler's sign), upper thigh (Fox's sign), or scrotum (Bryant's sign). Spontaneous adrenal hemorrhage is the most common cause of nontraumatic parietal ecchymosis sign in neonates. Only 20 such neonatal cases have been reported in the literature. Because all of them were of benign etiology, conservative management is generally recommended in the presence of this sign. We report the sign for the first time in a case of spontaneously ruptured adrenal neuroblastoma of newborn. Perinatal physicians need to be aware of this unusual presentation of neonatal neuroblastoma. [ABSTRACT FROM AUTHOR]

Competing Interests: Declaration of Competing Interest The authors declare no conflicts of interest.