Neonatal Presentation Research Articles

Pituitary Stalk Interruption Syndrome in a Newborn with Encephalopathy: A Case Report

Background: Pituitary Stalk Interruption Syndrome is a rare midline brain anomaly involving the pituitary gland that leads to a variable degree of pituitary hypofunction with a classic neuroimaging finding of the ectopic posterior pituitary, thin or absent pituitary stalk, and anterior pituitary hypoplasia. Case Presentation: We present the case of a term newborn who developed encephalopathy and respiratory distress during the neonatal period and was later diagnosed with Pituitary Stalk Interruption Syndrome. Conclusion: Genetic defects have been identified in 5% of cases, and at times there are associated extra pituitary malformations. In spite of being a welldescribed syndrome, a detailed description of its neonatal presentation and course is lacking in the literature.

Meningitis secondary to rectothecal fistula associated with congenital anterior meningocele

Meningitis secondary to rectothecal fistula is an exceptionally rare phenomena which has previously been reported exclusively in the setting of Currarino Syndrome for the pediatric population. We describe the case of a neonate presenting with polymicrobial meningitis due to rectothecal communication via congenital anterior sacral meningocele in the absence of an anorectal malformation. The authors opted for expeditious surgical resection of the fistulous tract and closure of the thecal sac using a posterior sagittal approach to the presacral space. The patient completed a course of intravenous antibiotics postoperatively. This case highlights the first reported neonatal case of rectothecal fistula in the absence of an anorectal anomaly. The surgical approach is described.

COMPARISON OF MEAN PLATELET VOLUME IN NEONATES WITH AND WITHOUT SEPSIS

ABSTRACT
 Objectives
 To compare mean platelet volume (MPV) in neonates with and without sepsis. 
 Study design
 Cross sectional descriptive study.
 Place & duration of study
 Neonatal Unit, Department of Paediatrics, Military Hospital, Rawalpindi from 2nd July 2017 to 22nd January 2018.
 Material and Methods
 140 neonates, of either gender with ages between 0-28 days, suspected of neonatal sepsis presenting with any two of the signs: core body temperature of > 38.5°C or < 36°C at time of presentation, pulse rate beyond the range of 100-200 beats/minute, leukocyte count beyond the range of 4000 to 30,000/mm3 or > 10% immature neutrophils on peripheral smear, tachypnea (>60 breaths/minute) and oxygen saturation (< 90%) on pulse oximeter, were included; while neonates having taken > 2 doses of antibiotics in last 48 hours as per history and clinical record were excluded. 3ml of blood was drawn and sent for culture, while another 3ml of blood sent for peripheral smear and MPV. All the data along with demographic details of the patients were noted. Same laboratory was used for all samples and the peripheral smear was done by the same pathologist each time to eliminate bias.
 Results
 31 neonates were culture positive for neonatal sepsis. Mean MPV(fl) was 7.31+0.92(fl). MPV was 8.52+0.51(fl) in septic neonates and 6.97+0.70(fl) in neonates without sepsis. The p value was significant (0.0001).
 Conclusion
 MPV was significantly raised in patients with sepsis.
 KEYWORDS
 
 Neonates, Sepsis, Mean Platelet Volume 
 

Pro-Inflammatory Profile of Children Exposed to Maternal Chikungunya Virus Infection during the Intrauterine Period: A One-Year Follow-Up Study.

Chikungunya virus (CHIKV) vertical transmission occurs due to maternal viremia in the prepartum. Clinical presentation in neonates can be varied; however, the consequences of intrauterine exposure on the immune response are unclear. Thus, we aimed to analyze inflammatory alterations in children exposed to maternal CHIKV infection. This is a cross-sectional study that included children exposed to maternal CHIKV infection (confirmed by RT-qPCR and/or IgM). Circulant immune mediators were analyzed by a multiplex assay. RESULTS: We included 33 children, with a mean age of 3 ± 2.9 months-old, and 19 (57.6%) were male. Only one child presented neurological alterations. CHIKV-exposed infants showed elevated levels of MIP-1α, MIP-1β, and CCL-2 (p < 0.05). Pro-inflammatory cytokines such as TNFα, IL-6, and IL-7 (p < 0.0001) were also increased. In addition, lower levels of PDGF-BB and GM-CSF were observed in the same group (p < 0.0001). Principal component (PC) analysis highlighted a distinction in the inflammatory profile between groups, where PC explained 56.6% of the alterations. Our findings suggest that maternal exposure to CHIKV can affect the circulating levels of pro-inflammatory cytokines during the infants’ first year of life. The long-term clinical consequences of these findings should be investigated.

Primary neonatal sacrococcygeal neuroblastoma masquerading as a teratoma: A case report

Background: Neonatal tumors comprise about 2% of all pediatric malignancies, with neuroblastoma having the highest incidence. Neuroblastoma involving the adrenal medulla and sympathetic ganglia is the most typical scenario in infancy, while the pelvic variant is rare. We report this case because of the unusual and rare presentation of neuroblastoma in a newborn baby mimicking sacrococcygeal teratoma. Case Presentation: A newborn male baby presented with a firm sacral mass, about 5 x 7 cm, with normal overlying skin. MRI revealed an intrapelvic mass extending to the sacral region encasing the coccyx. After preparation, complete tumor excision was performed, and the diagnosis of neuroblastoma was confirmed by histological and immunohistochemical study. Conclusion: Primary neonatal neuroblastoma presenting as a sacrococcygeal mass is a rare and atypical clinical finding of neuroblastoma. It is hard to diagnose this sort of tumor preoperatively unless the mass is subjected to histological and immunohistochemical analysis after tumor excision.

Major Congenital Malformations of Gastrointestinal Tract Among Neonates Presenting to Tertiary Care

Background: Major congenital malformations of gastrointestinal tract (CMGIT), are usually manifested in early neonatal period by signs of intestinal obstruction and may be life threatening. A broad spectrum of Gastrointestinal Tract (GIT) anomalies can be seen including both upper and lower GIT. As compared to other congenital anomalies, GIT anomalies are not well studied and till now very few studies have reported prevalence and pattern of GIT malformations. Objective: To determine different type of common gastrointestinal anomalies among neonates presenting at tertiary care hospital. Methods: This descriptive cross sectional study was done at National Institute of Child Health (NICH) Karachi over period of 6 months, from 1-8-17 to 31-1-18. All neonates presenting to emergency department and got hospitalized with CMGIT were enrolled. Results: There were 35 neonates enrolled with CMGIT. Out of these 16(45.7%) were male and 19(54.3%) were females. Mean age 9.4+3.82 (range 1-20) days. Mean weight was 2.5±0.98 (range 0.5-4.5) kg. Mean duration of symptoms was 5.8+1.72 (range 1-9) days. Among the CMGIT, 12 (34.3%) cases were of Anorectal malformation, persistent Vitelline duct was found in 6 (17.1%), Intestinal atresia in 4(11.4%), Intestinal stenosis in 5(14.3%), Hirshsprung disease in 4(11.4%) and Exomphalos in 4 (11.4%). Conclusion: Anorectal malformations were most common anomaly followed by persistent vitelline duct. Antenatal follow up and anomaly scan of all neonates should be done. Keywords: Gastrointestinal anomaly, Neonates, congenital anomaly.

Case Report-Right atrial myxoma with total anomalous pulmonary venous connection in neonate.

Introduction and importanceMyxoma is the commonest cardiac tumor in adults but is very rare in a newborn. The majority of myxoma arises from the interatrial septum. 75% of myxomas are found in the left atrium and 20% are found in the right atrium. Myxomas can be single or multiple. Multiple myxomas are usually associated with familial syndromes. Myxomas are generally not associated with another congenital heart defect except atrial septal defect. As myxomas are rare in newborns most of the knowledge is through various case reports.Case presentationWe report a rare case of an 8-day-old neonate presenting with cyanosis and respiratory distress. Transthoracic echocardiography was suggestive of right atrial myxoma which was arising from the atrioventricular junction on the right side of an interatrial septum. It was associated with the total anomalous pulmonary venous connection. The patient underwent successful excision of myxoma and rerouting of the common venous chamber to the left atrium with an uneventful immediate postoperative course.Clinical discussionCardiac myxomas are rare in newborns and their association with TAPVC even rare. Our patient had single atrial myxoma with TAPVC. The significance of this association is not clear. This association is mostly by chance. Probably it is the first case reported of its kind.ConclusionAs myxomas are very rare in neonates, it is highly possible to miss other associated structural heart defects. Diagnosis of atrial myxoma can be suggested by echocardiogram but a definite diagnosis can only be confirmed by histology.

Necrotizing Enterocolitis as a Possible Manifestaion of Multisystem Inflammatory Syndrome in Neonates Related to SARS-CoV-2

Background: Multisystem inflammatory syndrome in neonates (MIS-N) is hypothesized to be caused either following transplacental transfer of severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) antibodies or antibodies developed in the neonate after infection with SARS-CoV-2. The common clinical manifestations include fever, respiratory distress, shock, and encephalopathy. Gastrointestinal manifestations include vomiting, diarrhea, bloody stools, and gastric residues. Necrotizing enterocolitis (NEC) has not been described before in MIS-N. Clinical Description: We report a term neonate presenting on day 10 of life with 2 days history of fever, abdominal distension, vomiting, reduced feeding, and not passing stool. He had tachycardia, tachypnea, a distended tense and tender abdomen with absent bowel sounds, and excessive irritability. Salient investigation results were anemia, neutrophilic leukocytosis, hyponatremia, metabolic acidosis, and elevated inflammatory markers. Dilated bowel loops on abdominal radiograph and thickening of bowel wall on abdominal ultrasonography were consistent with NEC. In view of the unusual presentation of NEC, the elevated biomarkers and setting of the pandemic, MIS-N was suspected, investigated, and the diagnostic criteria were found to be satisfied. Management: The baby received supportive care. Methylprednisolone, intravenous immunoglobulin, aspirin, and enoxaparin were started resulting in defervescence and decline in inflammatory markers. However, he developed fixed bowel loops and underwent an exploratory laparotomy. This revealed stricture and extensive necrosis of the terminal ileum with adhesive obstruction. The affected segment was excised, and loop ileo-colostomy was done. He gradually improved and was discharged on the 11th postoperative day on enteral feeds. The infant was thriving on follow-up. Conclusion: NEC may be a possible manifestation of MIS-N.

#57 COVID-19 related Multisystem Inflammatory Syndrome in a Neonate Presenting as Supraventricular Tachycardia. A Case Report

BackgroundThe increasing trend in COVID-19-associated multisystem inflammatory syndrome in children MIS-C has been reported as severe acute respiratory syndrome coronavirus 2 continues to spread worldwide. Impact of covid 19 on newborns remains uncertain, while low, but the risk of neonatal infection does exist. A study from China reported 3% of neonates had evidence of SARS-CoV-2 infection. Postnatal infection is considered through horizontal transmission, as SARS-CoV-2 testing on placenta, umbilical cord, amniotic fluid, vaginal secretions, and breast milk samples has been negative. Diagnosis of MIS-C is based on 6 criteria: pediatric age, persistence fever, raised inflammatory markers, signs of organ dysfunction, lack of an alternative diagnosis, and temporal relation to COVID-19 infection or exposureMethodA CASE REPORT 24 days old neonate presented with fever, Reluctance to feed and Respiratory distress for 1 day. He was born to a mother G2P1 + 0 with no Co-morbid at 38 weeks gestation with APGAR scores of 8 at 1 minute and 9 at 5 minutes. Clinically he was febrile with heart rate of 270 beats per minute and respiratory rate of 70 breaths per minute. First and 2nd heart sounds were audible along with gallop rhythm. ECG showed rate of 270 & absent p wave suggestive of supraventricular tachycardia. INVESTIGATIONS His pro BNP 152772 pg/ml and trop I were significantly raised. Echocardiography was consistent with severe biventricular dysfunction and ejection fraction of 20 %. COVID antibodies were sent which were reactive. His inflammatory markers ferritin, LDH and D Dimer were strikingly raised. Two blood cultures and bio fire were negative, fulfilling the MIS-C criteria Adenosine was given twice with no improvement, IV amiodarone was added, which reverted SVT. IVIG was given.ResultsImproved echocardiographic led to extubation on day seven AND DISCHARGEDConclusionMIS-C in a neonate highlights the importance of considering the increasing spectrum of clinical manifestations, associated with SARS-CoV-2 infection. Further research is needed to make neonatal MISC guidelines

The gain of function SCN1A disorder spectrum: novel epilepsy phenotypes and therapeutic implications.

Brain voltage-gated sodium channel NaV1.1 (SCN1A) loss-of-function variants cause the severe epilepsy Dravet syndrome, as well as milder phenotypes associated with genetic epilepsy with febrile seizures plus. Gain of function SCN1A variants are associated with familial hemiplegic migraine type 3. Novel SCN1A-related phenotypes have been described including early infantile developmental and epileptic encephalopathy with movement disorder, and more recently neonatal presentations with arthrogryposis. Here we describe the clinical, genetic and functional evaluation of affected individuals. Thirty-five patients were ascertained via an international collaborative network using a structured clinical questionnaire and from the literature. We performed whole-cell voltage-clamp electrophysiological recordings comparing sodium channels containing wild-type versus variant NaV1.1 subunits. Findings were related to Dravet syndrome and familial hemiplegic migraine type 3 variants. We identified three distinct clinical presentations differing by age at onset and presence of arthrogryposis and/or movement disorder. The most severely affected infants (n = 13) presented with congenital arthrogryposis, neonatal onset epilepsy in the first 3 days of life, tonic seizures and apnoeas, accompanied by a significant movement disorder and profound intellectual disability. Twenty-one patients presented later, between 2 weeks and 3 months of age, with a severe early infantile developmental and epileptic encephalopathy and a movement disorder. One patient presented after 3 months with developmental and epileptic encephalopathy only. Associated SCN1A variants cluster in regions of channel inactivation associated with gain of function, different to Dravet syndrome variants (odds ratio = 17.8; confidence interval = 5.4-69.3; P = 1.3 × 10-7). Functional studies of both epilepsy and familial hemiplegic migraine type 3 variants reveal alterations of gating properties in keeping with neuronal hyperexcitability. While epilepsy variants result in a moderate increase in action current amplitude consistent with mild gain of function, familial hemiplegic migraine type 3 variants induce a larger effect on gating properties, in particular the increase of persistent current, resulting in a large increase of action current amplitude, consistent with stronger gain of function. Clinically, 13 out of 16 (81%) gain of function variants were associated with a reduction in seizures in response to sodium channel blocker treatment (carbamazepine, oxcarbazepine, phenytoin, lamotrigine or lacosamide) without evidence of symptom exacerbation. Our study expands the spectrum of gain of function SCN1A-related epilepsy phenotypes, defines key clinical features, provides novel insights into the underlying disease mechanisms between SCN1A-related epilepsy and familial hemiplegic migraine type 3, and identifies sodium channel blockers as potentially efficacious therapies. Gain of function disease should be considered in early onset epilepsies with a pathogenic SCN1A variant and non-Dravet syndrome phenotype.

Neonatal presentation of Loeys-Dietz syndrome: two case reports and review of the literature

BackgroundLoeys-Dietz syndrome (LDS) is a rare connective tissue disorder characterized by cardiovascular manifestations, especially aortic dilatations and arterial tortuosity, craniofacial and skeletal features, joint laxity or contractures, skin abnormalities, hypotonia and motor delay. Its diagnosis is established by the identification of a pathogenic variant in TGFBR1, TGFBR2, SMAD2, SMAD3, TGFB2 or TGFB3 genes. In newborns and toddlers, vascular complications such as aneurism rupture, aortic dissection, and intracerebral incidents, can occur already in the weeks of life. To avoid these events, it is crucial to precociously identify this condition and to start an apunderwent a surgical procedurepropriate treatment which, depending on the severity of the vascular involvement, might be medical or surgical.Case presentationWe report two cases of Loeys-Dietz syndrome precociously diagnosed. The first describes a male, born at 38 + 1 weeks of gestation, with hypotonia, joint hypermobility, arachnodactyly, and fingers joint contractures, as well as senile appearance and facial dysmorphisms. In the suspect of a connective tissue disorder, an echocardiography was performed and revealed an aortic root dilatation of 13 mm (Z score + 3). A trio based Whole Exome Sequencing found a novel de novo variant in the TGFBR2 gene. Despite the onset of a low-dose angiotensin receptor blocker therapy, the aneurysm progressed. The second case describes a female, born at 41 + 3 weeks of gestation. During the neonatal examination a cleft palate was noticed, as well as minor dysmorphisms. Since the family history was suspicious for connective tissue disorders, a genetic panel was performed and identified a pathogenetic variant in TGFB3 gene. In this case, the echocardiography revealed no abnormalities.ConclusionsIn addition to our cases, we identified 14 subjects with neonatal LDS in the medical literature. All of them had aortic involvement. Skeletal and face abnormalities, including eyes and palate malformations, were also highly frequent. Overall, 10 subjects required medical therapy to avoid aneurysm progression, and 8 patients underwent surgical procedures.Benefits of an early diagnosis of LDS are various and imply a potential modification of the natural history of the disease with early interventions on its complications.

Mature ruptured sacrococcygeal teratoma in a 1-day old neonate

Sacrococcygeal teratomas (SCTs) are rare congenital tumors that are derived from embryonic germ cell layers [1]. They are the most common extragonadal germ cell tumors (GCTs) in the neonate, with a reported incidence of 1 in 35 000 births [2]. Teratomas are made of tissues that derive from three germinal layers (ectoderm, endoderm, and mesoderm). Sacrococcygeal teratomas are more common in girls, with a sex ratio of 1:4 [3]. The most frequent location of teratoma in children is the sacrococcygeal area [4]. Many sacrococcygeal teratoma cases are diagnosed prenatally with routine ultrasonography between the 22nd and the 34th week of gestation. However, access to routine prenatal ultrasound in low and middle income countries is limited [5]. Antenatal diagnosis is essential to the pregnancy management and to prevent neonatal death by identifying fetuses that are at a higher risk of complications and planning an adequate intervention when needed [6]. This case is reported to describe a North African neonate presenting with a sacrococcygeal teratoma at birth without prior antenatal diagnosis, and highlight the need for prenatal routine care in low and middle income countries.

COVID-19 AMONG NEONATES: EXPERIENCE FROM AN SNCU OF A TERTIARY CARE HOSPITAL IN EASTERN INDIA

Background: Coronavirus disease 2019 (COVID-19) pandemic has spread to every corner of the globe with immense rapidity. The clinical presentation of neonates due to COVID19 is poorly understood. Aim and Objectives: The study assessed the clinical profile of neonates with COVID-19 from the Special Newborn Care Unit (SNCU) of a tertiary care hospital in eastern India. Materials and Methods: Data from the hospital record section was retrieved and bed head tickets (BHT) were collected. A thorough review of the BHTs was done. Retrieved data was presented in tabulated form and analyzed. Results: Out of the 285 neonates tested for COVID19 by reverse transcriptase-polymerase chain reaction (RT-PCR) from the nasopharyngeal swab, 23 tested positive. Fever was the most common presenting complaint in 9 cases (39.13%). Respiratory distress was the clinical presentation in 5 cases (21.73%), while 4 cases (17.39%) presented with diarrhea. The other symptoms included fever and cough with nasal congestion, found in 2 cases (8.69%). Mother being positive for COVID-19 was found to be a risk factor for COVID-19 in 3 (13.04%) cases. Conclusions: It is essential to screen neonates not only with the history of COVID-19 positive mothers but also other neonates presenting with symptoms suggestive of COVID-19.

Frequency of small bowel perforation in neonates with pneumopeirtoneum.

Objective: To determine the frequency of small bowel perforation in neonates with pneumoperitoneum. Study Design: Descriptive Cross-sectional study. Setting: Department of Pediatrics Surgery, National Institute of Child Health Karachi. Period: November 2018 to April 2019. Material &amp; Methods: Study conducted to identify clinical presentation, risk factors, site of perforation and outcome of pneumoperitoneum in neonates. Data collected was entered and analyzed in the SPSS version 21. Mean with standard deviation was calculated for quantitative variables like age, gestational age, weight and duration of symptoms. Results: A total of 87 neonate presenting with abdominal distension and bilious vomiting presenting within 24 hours diagnosed as pneumoperitoneum with chest X-ray erect shows gas under diaphragm were included in this study. The average age of the neonate was 11.02±8.52 days. There were 48 (55.17%) male and 39(44.83%) female. Frequency of small bowel perforation in neonates with pneumoperitoneum was observed in 31.03 %( 27/87). Conclusion: It is to be concluded rate of small bowel perforation was significantly high in those neonate who had below and equal to 2 kg weight and also high in those neonates who presented with in 12-24 hours duration of symptoms.

Ectrodactyly: Rare Presentation in Pre-Term Twin Neonates

Ectrodactyly: Rare Presentation in Pre-Term Twin Neonates

A neonate presenting with lactic acidosis, hypotonia, encephalopathy and tubulopathy: Possible RRM2Brelated encephalomyopathic mitochondrial DNA depletion syndrome

The Sri Lanka Journal of Perinatal Medicine (SLJPM) was launched in 2019 as the official journal of the Perinatal Society of Sri Lanka. This journal is unique in the fact that it brings together three core specialties of perinatal medicine - obstetrics, neonatology and community medicine. It is an open access peer reviewed journal containing original research articles, expert reviews, current practices and other articles of interest to all involved in perinatal medicine.

Multiple Sulfatase Deficiency: A Clinical Report with Neonatal Presentation

Multiple Sulfatase Deficiency: A Clinical Report with Neonatal Presentation

Neonatal presentations of neuromuscular disorders.

The term 'neuromuscular diseases' defines disorders of the extended motor unit. Newborns with disorders of peripheral nervous system (PNS) (motor neurons, nerve roots, plexuses, peripheral nerves, neuromuscular junction, and skeletal muscles) present most frequently with hypotonia, weakness, contractures, respiratory and feeding difficulties. Challenge in the newborn period is, hypotonia may also occur with more common central causes such as; electrolyte disturbances, sepsis, hypoxic-ischemic encephalopathy (HIE), congestive heart failure, and inborn errors of metabolism. Moreover, newborns with PNS involvement and/or genetic neuromuscular disorders (NMD) can also be prone to intrapartum asphyxia which further masks an underlying condition. This is why, NMD is also described as one of the 'HIE-mimics'. Genetic counseling, individualized treatment strategies, standards of care require accurate diagnosis. Neuromuscular field is moving to screening programs and pre-symptomatic diagnosis with promising disease-modifying treatments. The aim of this paper is to discuss approach to NMD within the newborn period in line with clinical history, examination findings, and diagnostic tests.

Outcome of Neonates Presenting With Severe Cardiac Failure due to Cerebral Arteriovenous Fistula

BackgroundCongenital cerebral arteriovenous fistulas (AVFs), including vein of Galen malformations, presenting in infancy carry variable mortality and morbidity. This study aimed to describe the outcome of neonates with cerebral AVFs who present with refractory cardiac failure. MethodsRetrospective chart review of neonates with refractory cardiac failure due to cerebral AVFs presenting before 28 days of age in a single-center neuro-intensive care nursery over a 12-year period (2008-2020) was conducted. ResultsSeventeen neonates were included. Twelve had a vein of Galen malformation, four a non-galenic pial AVF, and one a dural AVF. Seven neonates (41%) died without receiving an embolization procedure. The remaining ten were critically ill. Seven (70%) were mechanically ventilated and on nitric oxide, 5 (50%) were on pressors, and 6 (60%) had renal and/or hepatic dysfunction. Seven (70%) had pre-existing brain injury on imaging. The first embolization procedure occurred at a median age of 4 days (range: 0-8 d). Complications included intracranial hemorrhage in 8 of 10 (80%) and seizures in 5 of 8 (62%). Five (50%) neonates who underwent embolization died. Among the 5 neonates who survived, all have motor impairment. Four (80%) developed hydrocephalus requiring a ventriculoperitoneal shunt, and 2 (40%) developed epilepsy and are nonverbal. ConclusionIn this cohort of critically ill neonates with cerebral AVF, all seven who did not receive embolization and half of ten who were treated died. The five survivors all have neurodevelopmental impairment. This information may be helpful to parents and providers who make decisions regarding life-sustaining treatments for neonates with cerebral AVFs and refractory cardiac failure.

Factor XIII Deficiency in a Mother-Baby Dyad

Background: Factor XIII deficiency is a rare autosomal recessive coagulation disorder with varied presentations including prolonged bleeding from the umbilical cord stump, defective wound healing, recurrent miscarriages, or life-threatening intracranial hemorrhage. Clinical Description: A male baby was born at term gestation to a fourth gravida mother with a history of two abortions in the past. He was born out of a third-degree consanguineous marriage, with smooth perinatal transition, but developed multiple episodes of seizures associated with poor feeding and lethargy after 24 h of life. Management: On evaluation, septic screen, metabolic screen (serum electrolytes, calcium, and blood sugar), and coagulation assays were normal. Ultrasonogram revealed a hyperechoic lesion restricted to the left cerebral hemisphere, suggestive of an intraparenchymal hemorrhage. Magnetic resonance imaging brain showed left intraparenchymal hemorrhage with significant mass effect and midline shift. In view of intracranial bleed with normal coagulation assay and other causes being ruled out, factor XIII clot solubility assay was sent and found to have undetectable levels. Factor XIII levels of the mother were also found low (5.5%) though the levels in the father were normal. The baby was managed conservatively with supportive measures in the form of anticonvulsant and anti-edema measures. He recovered successfully and is under close follow-up. Conclusion: A high index of suspicion of factor XIII deficiency should be kept in any neonate presenting with intraparenchymal hemorrhage and recurrent abortions in the mother. This case is being reported to highlight factor XIII deficiency in recurrent pregnancy loss and neonatal intracranial bleeding. Prenatal screening for factor XIII deficiency in these circumstances will help in effective management of future pregnancies.