Bullous Kaposiform Hemangioendothelioma Masquerading as Aplasia Cutis Congenita.

We present the case of a male infant with violaceous bullae on the scalp that were initially thought to be bullous aplasia cutis but at 3 months of age were diagnosed as a kaposiform hemangioendothelioma. This diagnosis should be considered when evaluating newborns with bullous plaques on the scalp that do not heal in the first 2-3 weeks of life. Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor that typically presents as a violaceous to purpuric plaque at birth or early infancy. It may be associated with Kasabach-Merritt phenomenon (KMP), a potentially life-threatening consumptive coagulopathy.

Racial and socioeconomic disparities in reduction mammoplasty: an analysis of nationwide inpatient sample database.

The American Society of Plastic Surgery recently recorded a decline in numbers of breast reductions, one of the most common procedures performed by plastic surgeons. The purpose of this study is to characterize the reduction mammoplasty patient population which would further assist in planning the future workforce needs. Using the Nationwide Inpatient Sample database for 2007, a χ analysis of female in-patients treated with reduction mammoplasty for breast hypertrophy was performed to identify significant differences in race and payer mix. Of 8394 female in-patients with breast hypertrophy, 61% were treated with reduction mammoplasty. Black and Hispanic patients (P < 0.0001) and patients with private insurance (P < 0.0001) were more likely to undergo reduction mammoplasty. This study demonstrates racial and socioeconomic disparities in breast reduction in the United States in 2007. With the pending institution of universal healthcare, it is predicted that disparities revealed may worsen due to cost containment pressures.

The Effects of Cannabis: Implications for the Surgical Patient.

Cannabis use is increasingly prevalent. Cannabinoid receptors regulate pro-inflammatory cytokines, and compounds in marijuana exert diverse physiologic effects. As more patients use cannabis, clinicians should recognize implications of perioperative cannabis use. Although the role of cannabis use in perioperative pain control has been explored, little is known about its effect on perioperative wound healing or on hematologic, pulmonary, and cardiovascular physiology. METHODS: We searched PubMed for English-language articles related to cannabis (ie, marijuana, cannabidiol oil, and tetrahydrocannabinol) and wound healing, cardiovascular, pulmonary, or hematologic outcomes, and surgery. Titles and abstracts were reviewed, and relevant articles were analyzed. Human, animal, and pathology studies were included. Editorials, case reports, and review articles were excluded. RESULTS: In total, 2549 wound healing articles were identified; 5 human studies and 8 animal/pathology studies were included. Results were conflicting. An estimated 2900 articles related to cardiovascular effects were identified, of which 2 human studies were included, which showed tetrahydrocannabinol and marijuana caused tachycardia. A total of 142 studies regarding pulmonary effects were identified. Three human studies were included, which found no difference in respiratory complications. In total, 114 studies regarding hematologic effects were identified. The 3 included human studies found conflicting venous thromboembolism risks. The overall study quality was poor. Information about dose/duration, administration route, and follow-up was reported with variable completeness. CONCLUSIONS: Surgeons should consider effects of cannabis in the perioperative setting. Little is known about its perioperative effects on wound healing, or on cardiovascular, pulmonary, and hematologic physiology. Further research should elucidate the effects of administration route, dose, and timing of cannabis use among surgical patients.

Associated (parallel) tomographic findings in patients with single-sutural synostosis.

INTRODUCTION: Craniosynostosis is the premature fusion of 1 or more of the cranial sutures, which causes an abnormal skull shape. The treatment is complex and requires surgical intervention for correction, between 3 and 12 months of age. The current standard for diagnosing craniosynostosis is to obtain a computed tomographic (CT) scan. However, recent studies have emphasized potential problems associated with ionizing radiation. The aim of this study was to evaluate the use of the preoperative CT scan, and its findings, and to define the value of these data. MATERIALS AND METHODS: This study is a retrospective analysis of 89 patients with craniosynostosis during the period between 1998 and 2007, who had CT using the Yale-New Haven Hospital computer system. Clinical findings such as age, sex, and diagnosis were recorded. All intraoperative abnormalities were noted. Preoperative CT scans were reevaluated for accuracy and completeness of diagnosis, cerebral anomalies, and other head and neck pathologic conditions. RESULTS: Initially, 131 cases of nonsyndromic craniosynostosis were identified. Of these, we had access to CT evaluations in 89 patients. The mean age at the time of the surgery was 13 months, varying from 2 months to 18 years old. Most patients were males. The most common diagnoses were sagittal synostosis in 47 cases; unilateral coronal, 16; and metopic, 15. In most patients, the CT scan demonstrated the same diagnosis as the clinical examination-derived diagnosis. However, 6 patients had an incomplete diagnosis or misdiagnosis. In addition, 3 cases of prominence of the subarachnoid space, 2 cases of cerebral atrophy, 1 case of subarachnoid tumor, and 1 deformational dysplasia of encephalon were identified. Other minor additional diagnoses were 5 cases of fluid in the middle ear and antrum of mastoid, 2 cases of cysts in the maxilla, and 1 case of an associated cervical lymphatic tumor. CONCLUSIONS: The advantage of CT use is greater accuracy in diagnosis, less potential for inappropriate surgery, and, as an ancillary effect, a more complete and timely assessment of congenital anomalies so that more appropriate referrals can be made. Low-dose (pediatric) radiation CT still seems to have a place in the examination of a patient with skull deformity and potential craniosynostosis.

Does the timing of postoperative showering impact infection rates? A systematic review and meta-analysis.

INTRODUCTION: Optimum timing of postoperative showering varies. Earlier showering improves patient satisfaction, but the impact of the timing of showering on postoperative infection is unclear. We conducted a systematic literature review and meta-analysis to investigate the outcomes of various postoperative showering practices. METHODS: We searched PubMed to identify relevant human clinical studies in English, and searched these for additional references. Articles were reviewed for patient demographics, surgical specialty and procedure, wound closure method, placement of drains, showering protocol, and rates of infection and complications. Only randomized controlled trials were analyzed. A random-effects meta-analysis model was used to determine overall infection and complication rates between patients allowed to shower within the first 48 h postoperatively or later. RESULTS: Out of 357 studies, seven and five were included in the infection and complications rate meta-analyses, respectively. A total of 1,881 and 958 patients were included in each analysis; 605 and 477 patients in each analysis were allowed to shower on or before postoperative day 2 ("early"), while the remainder were prohibited from showering until postoperative day 3 to beyond one week ("delayed") postoperatively. There was no difference in infection (p = 0.45, [-0.0052, 2 × 0.007 95% CI]) or complication rate (p = 0.36, [-0.0046, 2 × 0.005 95% CI]) with earlier vs. delayed showering protocols. CONCLUSION: Published literature demonstrates no increase in the overall rate of wound infections or complications when patients showered earlier in the postoperative period. Additional randomized studies are needed to determine the ideal time for postoperative showering. These data should be considered by surgeons while determining when to permit patients to shower after surgery.

Immediate or delayed nasal reconstruction in infant after subtotal amputation? Nasal reconstruction with forehead flap in a 2-year-old child.

Nasal reconstruction using the forehead flap is one of the oldest recorded reconstructive procedures. The forehead flap is considered a standard for reconstruction of large defect. Most of reports concerning the forehead flap in nasal reconstructions deal with adults. Nasal reconstruction in infants raises some controversies, and very few articles report nasal reconstruction in the infants. A 15-month-old boy was presented after subtotal nasal amputation, after a dog bite. Microsurgical replantation was performed, but proved unsuccessful. The wound healed with debridement and local care. At age 2, nasal reconstruction was performed with autogenous ear cartilage and forehead flap. The reconstructed nose currently appears to be of good color, texture match, and functional. To our knowledge, few reconstructions of acquired nasal deformities using a forehead flap have been previously reported in infants. This 2-year-old patient may be among the youngest to have undergone this procedure. The forehead flap can be applied in very young children with reasonable expectations of excellent functional and esthetic outcome. Long-term results remain to be seen regarding nasal function and growth.

Blindness as a complication of monobloc frontofacial advancement with distraction.

The monobloc frontofacial osteotomy provides aesthetic and functional improvement in the treatment of various craniofacial deformities. This procedure, through highly complex, has had some significant associated complication, such as cerebrospinal fluid leakage, hematoma, infection, and bone resorption. Distraction has been successfully used to gradually elongate bone and soft tissue. This method seems to provide improved results over conventional surgery, with less morbidity. We present a case of a patient with Apert syndrome who underwent monobloc advancement using the Rigid External Device (RED) device and who developed a transient bilateral amaurosis on the fourth postoperative day before distraction. A second procedure was performed to push back the frontal bandeau, maintaining the device in position. The blindness was resolved with this procedure as well as treatment with systemic steroids. The distraction was started thereafter, and the desired improvement was acquired. To our knowledge, this is the first case of transient bilateral amaurosis in a patient undergoing monobloc distraction.

Fetal diagnosis of cleft lip: natural history and outcomes.

Previous studies have suggested that most fetuses diagnosed prenatally with cleft lip have multiple associated anomalies, that fetuses with isolated cleft lip/palate are uncommon, and that their ultimate outcome is poor (Lopoo, 1999). However, our clinical experience failed to make a similar connection. The purpose of this study was to examine the natural history and current outcomes of fetuses diagnosed with cleft lip. A retrospective study was performed on all patients undergoing fetal ultrasonography over a 7-year period (1993-2001) in a tertiary referral center. All patients who had a fetal diagnosis of cleft lip were reviewed (N = 57). Fifty-seven fetuses with cleft lip were identified. Forty-three of 57 patients have complete records. Of these, 18 patients (43%) had terminations for associated severe malformations. Three fetuses (7%) had intrauterine or neonatal demise. The remainder, 22 fetuses (53%) survived to term. Of this group, 18 (82%) of 22 infants were diagnosed postnatally with isolated cleft lip/palate and no associated anomalies. Only 4 of 22 had associated anomalies. All recorded terminations were performed with known karyotyping. We demonstrate that a significant percentage of fetuses diagnosed ultrasonographically with cleft lip/palate in our study were isolated and that this subset of patients has an excellent prognosis and outcome. This may provide further or alter implications for genetic counseling and management.

Surgical correction of Tessier number 0 cleft.

The no. 0-14 cleft involves the midline of the face and cranium. It may include both a true and a false median cleft lip, with or without associated hypotelorism or hypertelorism. The no. 0 cleft is the most common of the craniofacial clefts. The objective of this study was to review the functional outcome and aesthetic results of the different techniques applied for each case. We have conducted a retrospective analysis of our series consisting of 32 cases of Tessier no. 0 cleft, in the period between 1997 and 2007. The patients were divided into 2 groups: those with the true median cleft and those with the false median cleft. The clinical findings, lip malformation, alveolar cleft, nasal appearance, septal involvement, associated deformities, and surgical procedures, were all reviewed. Holoprosencephaly was present in 9 cases, with a false median cleft upper lip and an absence of the premaxilla, septum, and columella (only 1 patient underwent lip and columella reconstruction at 2 years of age). Nine patients had an incomplete median cleft lip. Seven of these cases had associated median alveolar cleft, and 1 had an intranasal tumor, associated with lipoma of corpus callosum, characteristic of the Pai syndrome. Six cases of a bifid nose were seen, 2 of which were associated with an alveolar median cleft and hypertelorism. An isolated median alveolar cleft was present in 7 cases, 2 of them associated with a no. 30 cleft. This article presents a large series of Tessier no. 0 cleft, describing the differences between the false and the true median cleft. The surgical procedures may vary in relation to the type of involvement.

Use of the external pectoralis myocutaneous major flap in anterior skull base reconstruction.

Despite the emergence of free tissue transfer, the pectoralis major myocutaneous flap (PMMF) still has a role in anterior base skull reconstruction (when free tissue transfer is not feasible). The aim of this study is to evaluate the results of external PMMF in anterior skull base reconstruction. A retrospective study from 1977 to 2006 was conducted at Yale New Haven Hospital. 16 patients (mean age 64 years), presenting with a malignant tumour of the anterior base skull, were included. The primary pathology was recurrent squamous carcinoma. Tumour resection resulted in orbital exenteration in 60%, and bone resection of the anterior skull base in 81% of patients. The initial skin defect was 49 cm(2) (range 16-100 cm(2)). The PMMF was the primary reconstructive choice in 87%, and utilized after free flap failure in two cases. Three minor complications were noted. Orbital exenteration and anterior base skull resection is a surgical procedure that leads to significant reconstructive challenges. The PMMF remains a safe and versatile reconstructive tool in anterior skull base tumour resection. The externalized pedicle allows this flap to reach periorbital and anterior skull base.

The Tessier number 5 facial cleft: surgical strategies and outcomes in six patients.

The Tessier no. 5 facial cleft is an extremely rare congenital malformation. Only 26 cases have been described in the English-language literature. The cleft begins in the upper lip just medial to the oral commissure, extending across the cheek as a groove ending at the junction of the middle and lateral thirds of the lower eyelid. The bone involvement usually includes an alveolar cleft in the premolar region, extends across the maxilla lateral to the infraorbital nerve, up to the infraorbital rim and orbital floor. The goals of the surgical procedure include reconstructing the lower eyelid, repositioning the lateral canthus, closure of the labiomaxillary cleft, and restoration of the skeletal continuity (including the orbital floor defect) with bone grafts. We present six patients with the Tessier no. 5 facial cleft who have been treated in our combined centers and discuss the surgical options and difficulties faced in the reconstruction of this rare and challenging craniofacial malformation. To date, we have treated six patients (two with bilateral and four with unilateral clefts). Three of the patients with unilateral clefting had an associated no. 4 cleft and one patient with a bilateral cleft had an associated no. 3 cleft. This paper represents the largest series to date documenting surgery for patients with the Tessier no. 5 facial cleft.

The misdiagnosis of craniosynostosis as deformational plagiocephaly.

Deformational plagiocephaly, unilateral coronal craniosynostosis, and lambdoid craniosynostosis all cause oblique deformities of the skull. They are separate entities with different etiologies resulting in clear differences of physical examination, radiologic findings, and clinical course. Treatment for each condition is markedly different. Surgical intervention is recommended for unilateral coronal and lambdoid craniosynostosis using different techniques. Deformational plagiocephaly, on the other hand, is often satisfactorily managed with conservative treatment. We present 4 clinical reports on patients in whom unilateral coronal craniosynostosis and lambdoid craniosynostosis were originally misdiagnosed as occipital deformational plagiocephaly and highlight individual structural distinctions.

Familial lambdoid craniosynostosis between father and son.

Lambdoid craniosynostosis is an uncommon condition, with an incidence of 1 per 33,000 live births. Its etiopathology remains controversial. Although many forms of syndromic and nonsyndromic craniosynostosis demonstrate an inherited pattern, few articles have reported lambdoid craniosynostosis in the same family. In this article, we report lambdoid synostosis in a father and son. A case review is performed. A full-term male infant was delivered by cesarean delivery because of failure to progress. He presented at 3 years of age with right unilateral lambdoid craniosynostosis with facial asymmetry and lateral deviation of his jaw, with occlusal abnormality. At presentation, the father reported skull surgery during his infancy for unilateral lambdoid craniosynostosis. Review of the computed tomographic scan of the child demonstrated a plagiocephalic appearance of the calvarium with frontal bossing and a fusion of the lambdoid suture on the right sides. The brain parenchyma showed no abnormality. The review of his father's surgical record from 33 years ago and of his computed tomographic scan ordered by our team confirmed the diagnosis of previous lambdoid craniosynostosis. Only 2 previous cases of familial isolated lambdoid craniosynostosis have been previously described in literature. Although the genetic basis has been established for many types of craniosynostosis, the etiopathogenesis of isolated lambdoid synostosis has not yet been established. We report the third case of inherited unilateral lambdoid craniosynostosis. The genetic pathogenesis of lambdoid craniosynostosis will be discussed.

Asymmetric skull shapes: diagnostic and therapeutic consideration.

The asymmetric skull shape, or plagiocephaly has become of particular concern since the advent of the "Back to Sleep Campaign," as increasing numbers of children present with this deformity within the first year of life. In this article we review the diagnosis and treatment options for the management of various forms of plagiocephaly. Of particular concern are the various forms of craniosynostosis which may be mistaken for deformational plagiocephaly.

Orthotic treatement improves cranial base abnormality in patients with craniofacial microsomia and deformational plagiocephaly

Craniofacial microsomia is assciated with hypoplasia of the facial skeleton and musculature. These primary defects cause a secondy alteration of the craniofacial skeleton. Current therapies do not attempt to correct the cranial base deformity in childhoold. Another cause of oblique deformities of the skull is deformational plagiocephaly. This common disorder is secundary to external deformational forces and tends to improve with time and may require only conservative treatment. We present two cases of deformational plagiocephaly superimposed upon hemifacial microsomia. Orthotic treatment was utilized to improve both the deformational plagiocephaly and the cranial base deformity. This novel therapy has the potential to correct the cranial base deformity in craniofacial microsomia.