Cloverleaf Deformity Research Articles

Duodenal Cloverleaf Deformity Associated With Eosinophilic Inflammation.

Duodenal Cloverleaf Deformity Associated With Eosinophilic Inflammation.

Cloverleaf Skull Syndrome: A Rare Case Report

Cloverleaf deformity is an extremely rare skull deformity that occurs when several joints (sutures) between a baby's skull bones begin to fuse too early. Also known as Kleeblattschädel syndrome, the cloverleaf deformity causes bulging at the front and sides of the skull, resembling a cloverleaf shape. Cloverleaf skull syndrome is an abnormal configuration of the calvaria classified as craniosynostosis, consisting of premature ossification of cranial sutures. It is a deformity characterized by a remarkable enlargement of the head, with a trilobed configuration of the frontal view, resembling a three leaved clover(1) The first report about such a syndrome in the literature occurred in 1973 and, over the years only some tens of cases have been documented worldwide(3). The present report describes a case of a severe craniofacial condition that is known as "cloverleaf skull syndrome". At Physical examination multiple malformations were observed, with altered craniofacial configuration, low set ear, hypertelorism, exopthalmos, syndactyly, closed anterior fontanelle, high arched palate, bilateral CTEV. The vast majority of patients with kleeblattschädel have intellectual impairment and hydrocephalus. It is usually seen in thanatophoric dysplasia: classically type II only, severe Apert syndrome, severe Crouzon syndrome, Boston-type craniosynostosis & Carpenter syndrome.(5)Craniosynostosis has an incidence of 1 in 2100 to 1 in 2500 live births with multi-suture craniosynostosis constituting about 5% of all the cases [9]. This condition is associated with a variety of presentations including optic atrophy, hydrocephalus, respiratory problems, cleft palate, and disorders of hearing and speech.[10] These cases are very rare & so shall be reported promptly to deal with management part of this disease. An expert team of maxillofacial, plastic, and neurosurgeons is needed to handle such complex conditions which involve the correction of the bony deformity.

Severe Constricted Head – An Extreme Form Of Crouzon Syndrome Posing Challenging Fronto-Orbital Advancement : Case Reports

Background : Constricted head or cloverleaf deformity in Crouzon syndrome is a severe form of the syndrome involving trilobed coronal and lambdoid synostosis. Crouzon syndrome with acanthosis nigricans is distinct from the classic Crouzon syndrome, characterized by thick and dark skin in body folds. The major problems resulting from constricted head are related to craniostenosis, orbitostenosis developing from abnormalities of the skull base associated with progressive rise in intracranial and intraorbital pressures which could progress to hydrocephalus and cerebellar herniation. Case: Two cases with severe Crouzon syndrome were reported. The first was a five month old girl associated with acanthosis nigricans and the other was a sixteen month old boy, both whose phenotypic expressions were at the extreme severe end of the disease spectrum. On examination there was serious corneal exposure, visual loss, severely narrow head, midface hypoplasia, and beaked nose. CT scan showed typical cloverleaf skull, expanded middle cranial fossa, foreshortened anterior and posterior fossae and honeycomb appearance in the occiput involving pansutural synostosis and extensive copper beaten deformity from the high intracranial pressure . Both were complicated with hydrocephalus requiring ventriculoperitoneal shunt. This required an arduous effort in releasing the brain from the multiply punctured calvaria, avoiding excessive dural tear and bleeding and ultimately preserving the brain. Excessive bleeding was also caused by the raised ICP. Both patients are planned to undergo occipital expansion three months later. Conclusion: Severely constricted head in Crouzon syndrome is an extreme manifestation and late stage of the syndrome which not only will result in irreversible complication but will require meticulous surgical technique.

Novel Suturectomy Using Absorbable Plates in Early Surgery for Craniosynostosis.

In craniosynostosis patients under 3 months of age, suturectomy is a valuable early treatment improving their outcomes. However, conventional suturectomy might not be in severe patients. The efficacy of our developed suturectomy using absorbable plates was examined. Our method was indicated for craniosynostosis patients under 3 months old who had severe intracranial hypertension, scaphocephaly, plagiocephaly, or trigonocephaly between September 2011 and March 2018. All patients underwent suturectomy, and the bone edges on both sides of the cuts were covered with absorbable plates. Evaluation was conducted with 3-dimensional computed tomography and photographs, and cephalic index, distance from dorsum sellae to forehead on computed tomography were analyzed. Twenty-one of the 25 patients were evaluated. The preoperative cranial shapes were 4 brachycephaly, 6 scaphocephaly, 5 oxycephaly, 2 clover-leaf deformity, and 4 plagiocephaly. There were 9 syndromic and 12 nonsyndromic patients. The mean age at the time of surgery was 52.3 days (7-89), and the mean follow-up period was 3.5 years (1-8).The cephalic index and cranial definition improved in 18 patients. The secondary surgery was not required in four syndromic and none of the 12 nonsyndromic patients. There were no major complications. Placement of absorbable plates was able to prevent bone formation during the early postoperative period, and yet also promote bone formation after plate absorption. The authors believe syndromic craniosynostosis patients with severe deformities and nonsyndromic ones with scaphocephaly or plagiocephaly, successfully avoided secondary surgeries. This approach is less invasive for craniosynostosis and is expected to be highly effective.

Kleeblattsch\xe4del skull presenting in concert with Pfeiffer syndrome

BackgroundKleeblattschädel skull, also known as the cloverleaf is a complex synostosis which presents with an enlarged tri-lobar skull resulting temporal bulging and a flat posterior skull, a rare find in the population. This pansynostosis is associated with other congenital syndromes which include Crouzon, Pfeifer, and Carpenter’s. The genetic disorder Pfeiffer syndrome results from skull bones’ premature fusion characterized by deformities of the hand and feet. The author reports a 10-month-old girl who presented with a tri-lobar skull and wide toes which is a significant finding of Pfeiffer syndrome.Case presentationA 10-month-old girl presents with congenital obstructive hydrocephalus due to cloverleaf skull with horizontally enlarged head and large fontanelles. The child also had mid-facial hypoplasia and pre-axial bilateral lower limb polydactyly. Wide toes were also observed, an indicator of Pfeiffer’s Syndrome. Computed tomography (CAT) scans grossly abnormal craniofacial appearances, with premature closure of the sagittal suture giving a cloverleaf skull appearance. There was also a significant thinning of the brain parenchyma.ConclusionPremature closure of sutures leads to a forced growth in a plane perpendicular to the closed suture. Cloverleaf deformity can present with multiple syndromes such as Pfeiffer’s. This leads to gross alteration of the skull with potential underlying morbidity.

Functional Outcomes, Quantitative Morphometry, and Aesthetic Analysis of Articulated Alar Rim Grafts in Septorhinoplasty.

The design, use, and indications for the articulated alar rim graft (AARG) and the functional and aesthetic improvements that can be achieved have not been fully characterized. To analyze the functional and aesthetic outcomes of AARG placement on nasal airway function, nasal base shape change, and appearance. A case series study of patients who underwent septorhinoplasty with placement of AARG at University of California, Irvine Medical Center, from 2015 to 2018 was carried out. Surgical data recorded included stage of rhinoplasty (primary vs revision), use of spreader grafts, rim grafts (and dimensions), caudal septal extension graft (CSEG), lateral crural tensioning (LCT), and turbinate reductions. Preoperative and postoperative Nasal Obstruction Symptom Evaluation Survey (NOSE) surveys were analyzed and correlated with AARG geometry, use of CSEG, and the LCT maneuver. Preoperative and postoperative alar base views were evaluated by fitting base shape to a parametric numerical model to categorize each to 1 of 6 shape categories. Blinded reviewers rated alar furrow severity and the alar ridge presence using a Likert scale for both preoperative and postoperative images to subjectively gauge aesthetic outcomes. Overall, 90 patients with both preoperative and postoperative NOSE scores who underwent septorhinoplasty and placement of an AARG were included. Of the 90 patients, 60 were women (mean age, 38.2 years). Patient NOSE scores (70.4 preoperatively to 25.1 postoperatively) significantly improved from preoperation to postoperation (P < .001), regardless of AARG size, CSEG, or LCT. Alar base shape parametric analysis showed preoperative to postoperative improvements were significant for anterior-to-posterior ratio mass distribution (95% CI, -0.16 to 0.02; P = .05) and vertical projection-to-horizontal base width ratio (95% CI, 0.01-0.32; P = .02) in flat noses and cloverleafing for narrow noses (95% CI, -0.05 to -0.01; P = .001); enhancement approached significance for reduction in lateral scalloping in cloverleaf noses (P = .06). Aesthetic analysis showed that there was a statistically significant improvement for the alar furrow (95% CI, -0.68 to -0.29 for rater 1; -0.54 to -0.27 for rater 2; and -0.59 to -0.27 for rater 3; P < .001) for all raters and for the alar ridge (95% CI, 0.16-0.48; P < .001) for 1 rater. To our knowledge, this is the first study to demonstrate that AARG use is associated with statistically significant improvement in NOSE scores. Placement of AARGs may improve posterior mass ratios in flat noses and lateral cloverleafing in narrow noses as suggested by quantitative shape change parameter analysis. The placement of AARGs was associated with aesthetic and functional enhancement in the cloverleaf deformity, which is associated with a prominent alar furrow, and often external nasal valve collapse. Patient selection is key when placing AARGs. NA.

S4A-12 SESSION 4A: GENERALITIES IN CRANIOSYNOSTOSIS – PART I THE EFFICACY OF OUR NOVEL SUTURECTOMY TECHNIQUE WITH ABSORBABLE PLATES IN EARLY SURGERY FOR CRANIOSYNOSTOSIS

Introduction: Surgical procedures for craniosynostosis patients with severe intracranial hypertension include suturectomy, cranial distraction and conventional cranial reconstruction. Suturectomy has been performed in children younger than 3 months of age with insufficient bone strength. Conventional suturectomy is generally ineffective, because bone formation occurs rapidly in the grooves, resulting in recurrent elevation of intracranial pressure and severe deformity. Therefore, the surgical effects should be extended by preventing bone formation during the early postoperative period to correct the severe deformity and subsequently achieve sufficient bone formation and strength. This study examined the efficacy of our newly developed suturectomy technique using an absorbable plate. We present our novel method and the postoperative follow-up. Methods: Our new method was indicated for craniosynostosis cases younger than 3 months of age who had severe intracranial hypertension, scaphocephaly, or plagiocephaly between 2011 and 2018. All patients underwent suturectomy. Suture lines that indicate premature fusion were incised at a width of approximately 7–10 mm to insert the absorbable plates. The bone stumps on both sides of the grooves were covered with absorbable sheet-type plates. Evaluation was conducted primarily with three-dimensional computed tomography and clinical photographs, and the preoperative and postoperative cephalic index were analyzed. Results: Twenty-three patients who underwent surgery were evaluated. The cranial shape was brachycephaly in five, scaphocephaly in four, oxycephaly in five, clover-leaf deformity in three, and plagiocephaly in four. There were eleven syndromic (1 Crouzon, 4 Apert, 3 Pfeiffer and 3 Saethre-Chotzen syndromes) and twelve non-syndromic patients. The patients’ mean age at the time of surgery was 43.5 days (12 to 76), and the mean follow-up period was 44.2 months (7 months to 7 years). The postoperative cephalic index improved in all patients. Secondary surgery was not required in five of eleven syndromic and in all non-syndromic patients. There were no major complications. Conclusion: Placement of an absorbable plate during strip craniectomy can prevent bone formation during the early postoperative period and promote bone formation after plate absorption. Even in patients who underwent distraction osteogenesis, the planning of the procedure was relatively easy because improvement in cranial shape had been achieved. In particular, some syndromic craniosynostosis patients were able to avoid additional surgery. Also, good outcomes were obtained without adjunctive therapy, such as helmet therapy. The present approach is less invasive and expected to be highly effective.

The unexpected presence of a huge cystic hygroma with thanatophoric dysplasia type I: a case report

Introduction: The presence of a thin-walled, multicystic structure posterior to the fetal head and neck with an intact vertebral column is known as a cystic hygroma. Thanatophoric dysplasia is the most lethal skeletal dysplasia. Thanatophoric dysplasia (TD) is an autosomal dominant disorder with two phenotypes. TD type I is characterized by a normal shaped skull with bowed femurs and micromelia, while TD type II is characterized by straight femurs with a clover-leaf deformity of the skull and micromelia. We report here a case of thanatophoric dysplasia type I associated with cystic hygroma, this association needs further evaluation. Case history: A 30 year old woman was referred at 26 weeks of gestation because of marked polyhydramnios. Ultrasonographic examination of the fetus revealed a narrow chest, protuberant abdomen, short curved femur, normal shaped skull with accidentally cystic hygroma were present. No other anomalies were detected. We explained to the patient the nature and severity of the anomaly and the patient preferred the termination of pregnancy. Induction of abortion was done successfully. Conclusion: This case report has highlighted the association between cystic hygroma and thanatophoric dysplasia type I. To our knowledge, this is the first report in the literature reporting the association of cystic hygroma and thanatophoric dysplasia type I. We believe that this case is useful for obstetricians and pediatricians. We also highly recommend the genetic evaluation of the fetus and parents.

Molecular Analysis of a Case of Thanatophoric Dysplasia Reveals Two de Novo FGFR3 Missense Mutations located in Cis

Thanatophoric dysplasia (TD) is the most common form of lethal skeletal dysplasia. It is primarily an autosomal dominant disorder and is characterised by macrocephaly, a narrow thorax, short ribs, brachydactyly, and hypotonia. In addition to these core phenotypic features, TD type I involves micromelia with bowed femurs, while TD type II is characterised by micromelia with straight femurs and a moderate to severe clover-leaf deformity of the skull. Mutations in the FGFR3 gene are responsible for all cases of TD reported to date. The objective of the study here was to delineate further the mutational spectrum responsible for TD. Conventional polymerase chain reaction (PCR), allele-specific PCR, and sequence analysis were used to identify FGFR3 gene mutations in a fetus with a lethal skeletal dysplasia consistent with TD, which was detected during a routine antenatal ultrasound examination. In this report we describe the identification of two de novo missense mutations in cis in the FGFR3 gene (p.Asn540Lys and p.Val555Met) in a fetus displaying phenotypic features consistent with TD. This is the second description of a case of TD occurring as a result of double missense FGFR3 gene mutations, suggesting that the spectrum of mutations involved in the pathogenesis of TD may be broader than previously recognised.

History of the Kleeblattschädel deformity: origin of concepts and evolution of management in the past 50 years

The history and evolution of surgical strategies for the treatment of Kleeblattschädel deformity are not well described in the medical literature. Kleeblattschädel anomaly is one of the most formidable of the craniosynostoses, requiring a multidisciplinary team for surgical treatment. The initial descriptions of this cloverleaf deformity and the evolution of surgical treatment are detailed in the present report. Two illustrative cases of Kleeblattschädel deformity, syndromic and nonsyndromic craniosynostoses treated by the senior authors, are also described along with insights into operative strategies.

Clinical experiences on the treatment of congenital cutis aplasia and craniosynostosis

The congenital cutis aplasia mainly occurs in head, sometimes involving the skull and dura mater. It's cause and the rate of falling ill are not known yet, it is the disease that rate of death is high by the infection, such as, the injured vascular hemorrhange of meningitis. Craniosynostosis is the disease the appears the skull as well as the facial deformity with growing, has from the developmental difficiency, visual distibance, motor disturbance, convulsion to the neurologic impairment of mental retardation, and accompanies the each characteristic deformity following the suture fused. Satisfactory results was achieved by local flap surgery and conservative treatment on the infant, diagnosed as the congenital cutis aplasia, case 1 Also successful treatment experiences of craniosynostosis(oxycephaly, brachycephaly, trigonocephaly, cloverleaf deformity) through the frontal bone advancement and the barrel stave asteotomy, were reviewed & pursues and investigates the intracranial volume of before and after of surgery, and then reports with the literature investigation.

Base line tenting: a sign of duodenal ulcer disease.

Indirect signs such as clover-leaf deformity, pseudodiverticulum formation, eccentric location of pyloric channel, and flattening of the fornix are of considerable value in the radiologic detection of an ulcer crater. We have found another indirect sign, base line tenting (BLT) to be very useful in the detection and diagnosis of duodenal ulcer disease. This is characterized by interruption of the base line of the bulb, with 2 associated perpendicular lines extending from the base and converging to a point in the duodenal bulb. After we observed this sign in various patients with peptic ulcer disease, a prospective investigation was performed involving 62 patients with duodenal ulcer disease over a period of 2 years. We found the BLT sign in 52 (83.8%). To date, no false-positive incidence has been identified. We concluded that BLT is a most valuable sign in the radiologic identification of duodenal ulcer disease.

Cloverleaf skull and bone dysplasias (report of four cases).

SUMMARYFour newborns with cloverleaf skull – one with elbow synostoses, one with thanatophoric dysplasia and two with a generalised bone dysplasia are reported.The syndromic relationship of cloverleaf skull deformity is discussed, specifically that between cloverleaf deformity with thanatophoric dysplasia and cloverleaf deformity with generalised bone dysplasia which is different from both achondroplasia and thanatophoric dysplasia.The diagnosis “thanatophoric dysplasia with cloverleaf deformity” should be strictly applied to the rare cases of cloverleaf skull which show generalised bone changes characteristic for this syndrome.CLOVERLEAF SKULL (CS) is an easily recognised anomaly. Its associations with other skeletal anomalies is well known. There is however confusion between cases of CS with generalised bone dysplasias.The purpose of this paper is to clarify this latter problem as well as to report four new cases of this rare anomaly.

The so-calle "Cloverleaf Skull Syndrome". A report of three cases with a discussion of its relationships with thanatophoric dwarfism and the craniostenoses.

Three cases of cloverleaf skull are reported, one in a newborn with generalized bone changes suggesting thanatophoric dwarfism, one in an infant with acrocephalosyndactyly, and the other in an infant with several features of Crouzon's disease. The relationships between cloverleaf skull, on one side, and thanatophoric dwarfism and the severe craniostenoses, on the other, are discussed. The cloverleaf deformity should be considered a purely morphologic feature in common to several diseases, and should no longer be considered a separate “syndrome”.